SitesBLAST

SitesBLAST – Find functional sites

SitesBLAST

Comparing WP_078717588.1 NCBI__GCF_900167125.1:WP_078717588.1 to proteins with known functional sites using BLASTp with E ≤ 0.001.

Or try Sites on a Tree, PaperBLAST, Conserved Domains, or compare to all protein structures

Found 20 (the maximum) hits to proteins with known functional sites (download)

P29803 Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial; PDHE1-A type II; EC 1.2.4.1 from Homo sapiens (Human) (see 4 papers)
40% identity, 97% coverage: 9:319/319 of query aligns to 60:368/388 of P29803

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P29803

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M227 (≠ I178) to V: in SPGF70; uncertain significance; dbSNP:rs200969445
S230 (≠ P181) mutation to A: Slightly reduces enzyme activity.
S291 (≠ F244) modified: Phosphoserine; by PDK1, PDK2, PDK3 and PDK4; mutation to A: Strongly reduces enzyme activity. Increases enzyme activity in stem cells.; mutation S->E,D: Abolishes enzyme activity. Increases neuronal cell death in response to glutamate excitotoxicity.
S293 (≠ G246) modified: Phosphoserine; mutation to A: Increases enzyme activity in stem cells.; mutation to D: Abolishes enzyme activity. Increases neuronal cell death in response to glutamate excitotoxicity.
S298 (≠ T250) modified: Phosphoserine; by PDK3; mutation to A: Slightly reduces enzyme activity.

P35486 Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; PDHE1-A type I; EC 1.2.4.1 from Mus musculus (Mouse) (see 2 papers)
39% identity, 97% coverage: 9:319/319 of query aligns to 62:370/390 of P35486

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P35486

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S232 (≠ P181) modified: Phosphoserine; by PDK1
S293 (≠ F244) modified: Phosphoserine; by PDK1, PDK2, PDK3 and PDK4
S300 (≠ T250) modified: Phosphoserine; by PDK1, PDK2, PDK3 and PDK4
K336 (= K285) modified: N6-acetyllysine; mutation K->Q,R: Decreases phosphorylation at S-232 and S-300 but does not affect activity or substrate metabolism.

P26284 Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; PDHE1-A type I; EC 1.2.4.1 from Rattus norvegicus (Rat) (see paper)
39% identity, 97% coverage: 9:319/319 of query aligns to 62:370/390 of P26284

query
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P26284

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S232 (≠ P181) modified: Phosphoserine; by PDK1
S293 (≠ F244) modified: Phosphoserine; by PDK1, PDK2, PDK3 and PDK4
S300 (≠ T250) modified: Phosphoserine; by PDK1, PDK2, PDK3 and PDK4

Q10489 Pyruvate dehydrogenase E1 component subunit alpha, mitochondrial; PDHE1-A; EC 1.2.4.1 from Schizosaccharomyces pombe (strain 972 / ATCC 24843) (Fission yeast) (see paper)
41% identity, 98% coverage: 5:318/319 of query aligns to 75:388/409 of Q10489

query
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Q10489

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R

I

I

E

R

K

G

E

M

V

V

E

D

E

E

A

E

V

V

A

R

F

I

A

A

E

E

Q

E

S

S

P

P

L

F

P

P

D

D

P

P

-

I

-

E

S

E

V

S

L

L

M

F

D

S

D

D

V

V

Y

Y

Y306 (≠ W240) modified: Phosphotyrosine
S310 (≠ F244) modified: Phosphoserine
S312 (≠ G246) modified: Phosphoserine

Sites not aligning to the query:

6 modified: Phosphothreonine

P26267 Pyruvate dehydrogenase E1 component subunit alpha type I, mitochondrial; PDHA1; PDHE1-A; EC 1.2.4.1 from Ascaris suum (Pig roundworm) (Ascaris lumbricoides) (see paper)
39% identity, 98% coverage: 5:318/319 of query aligns to 54:365/396 of P26267

query
sites
P26267

K

K

K

E

Q

D

M

A

V

V

S

H

M

Y

Y

Y

E

T

T

Q

M

M

N

L

R

T

I

I

R

R

L

R

F

M

E

E

T

S

K

A

L

A

Q

G

E

N

F

L

F

Y

A

K

A

E

G

K

Q

K

I

V

P

R

G

G

F

F

V

C

H

H

L

L

Y

Y

L

S

G

G

E

Q

E

E

A

A

V

C

A

A

T

V

G

G

A

T

C

K

A

A

G

A

L

M

T

D

N

A

N

G

D

D

K

A

I

A

T

V

S

T

T

A

H

Y

R

R

G

C

H

H

G

G

H

W

L

T

L

Y

A

L

K

S

G

G

G

S

D

S

L

V

K

A

L

K

M

V

M

L

A

C

E

E

I

L

F

T

G

G

R

R

K

I

D

T

G

G

Y

N

C

V

K

Y

G

G

K

K

G

G

S

S

M

M

H

H

I

M

A

-

D

-

F

Y

D

G

L

E

G

N

I

F

L

Y

G

G

A

G

N

N

G

G

I

I

V

V

G

G

G

A

G

Q

P

P

L

L

A

G

L

T

G

G

A

I

A

A

L

F

A

A

N

M

K

K

Y

Y

E

R

K

K

T

E

D

K

D

N

V

V

V

C

I

I

C

T

F

M

F

F

G

G

D

D

G

G

A

A

S

T

N

N

Q

Q

G

G

T

Q

T

L

Q

F

E

E

S

S

L

M

N

N

M

M

A

A

S

K

A

L

W

W

K

D

L

L

P

P

L

V

V

L

F

Y

V

V

N

C

E

E

N

N

G

G

Y

Y

G

G

I

M

S

G

C

T

P

A

Q

A

C

A

K

R

S

S

M

S

A

A

V

S

T

T

D

D

I

Y

A

Y

D

T

R

R

A

G

A

D

A

Y

Y

-

D

-

M

V

P

P

G

G

V

I

V

W

V

V

D

D

G

G

N

M

D

D

V

V

L

L

A

A

V

V

H

R

E

Q

A

A

V

V

S

R

E

W

A

A

V

K

K

E

R

W

A

C

R

N

N

A

G

G

E

K

G

G

P

P

S

L

L

M

V

I

E

E

C

M

K

A

T

T

Y

Y

R

R

W

Y

R

S

G

G

H

H

F
x
S

E

M

G

S

D

D

A

P

C

G

T

T

-
x
S

Y

Y

R

R

C

T

D

R

E

E

L

V

K

Q

E

E

W

V

M

R

-

K

A

T

K

R

D

D

P

P

I

I

P

T

R

G

F

F

E

K

E

D

K

K

L

I

V

V

E

T

S

A

G

G

V

L

L

V

K

T

K

E

K

D

D

E

L

I

K

K

E

E

I

I

K

D

A

K

R

Q

I

V

E

R

K

K

E

E

V

I

E

D

E

A

A

A

V

V

A

K

F

Q

A

A

E

H

Q

T

S

D

P

K

L

E

P

S

D

P

P

V

S

E

V

L

L

M

M

L

D

T

D

D

V

I

Y

Y

S289 (≠ F244) modified: Phosphoserine
S296 (vs. gap) modified: Phosphoserine

P16387 Pyruvate dehydrogenase E1 component subunit alpha, mitochondrial; Pyruvate dehydrogenase complex component E1 alpha; PDHE1-A; EC 1.2.4.1 from Saccharomyces cerevisiae (strain ATCC 204508 / S288c) (Baker's yeast) (see 2 papers)
38% identity, 98% coverage: 5:318/319 of query aligns to 78:391/420 of P16387

query
sites
P16387

K

K

K

A

Q

T

M

L

V

L

S

Q

M

M

Y

Y

E

K

T

D

M

M

N

V

R

I

I

I

R

R

L

R

F

M

E

E

T

M

K

A

L

C

Q

D

E

A

F

L

F

Y

A

K

A

A

G

K

Q

K

I

I

P

R

G

G

F

F

V

C

H

H

L

L

Y

S

L

V

G

G

E

Q

E

E

A

A

V

I

A

A

T

V

G

G

A

I

C

E

A

N

G

A

L

I

T

T

N

K

N

L

D

D

K

S

I

I

T

I

S

T

T

S

H

Y

R

R

G

C

H

H

G

G

H

F

L

T

L

F

A

M

K

R

G

G

G

A

D

S

L

V

K

K

L

A

M

V

M

L

A

A

E

E

I

L

F

M

G

G

R

R

K

R

D

A

G

G

Y

V

C

S

K

Y

G

G

K

K

G

G

S

S

M

M

H

H

I

L

A

-

D

-

F

Y

D

A

L

P

G

G

I

F

L

Y

G

G

A

G

N

N

G

G

I

I

V

V

G

G

G

A

G

Q

G

V

P

P

L

L

A

G

L

A

G

G

A

L

A

A

L

F

A

A

N

H

K

Q

Y

Y

E

K

K

N

T

E

D

D

D

A

V

C

V

S

I

F

C

T

F

L

F

Y

G

G

D

D

G

G

A

A

S

S

N

N

Q

Q

G

G

T

Q

T

V

Q

F

E

E

S

S

L

F

N

N

M

M

A

A

S

K

A

L

W

W

K

N

L

L

P

P

L

V

V

V

F

F

V

C

N

C

E

E

N

N

G

K

Y

Y

G

G

I

M

S

G

C

T

P

A

Q

A

C

S

K

R

S

S

M

S

A

A

V

M

T

T

D

E

I

Y

A

F

D

K

R

R

A

G

A

Q

A

Y

Y

-

D

-

M

I

P

P

G

G

V

L

V

K

V

V

D

N

G

G

N

M

D

D

V

I

L

L

A

A

V

V

H

Y

E

Q

A

A

V

S

S

K

E

F

A

A

V

K

K

D

R

W

A

C

R

L

N

S

G

G

E

K

G

G

P

P

S

L

L

V

V

L

E

E

C

Y

K

E

T

T

Y

Y

R

R

W

Y

R

G

G

G

H

H

F
x
S

E

M

G

S

D

D

-

P

A

G

C

T

T

T

Y

Y

R

R

C

T

D

R

E

D

E

E

L

I

K

Q

E

H

W

M

M

R

A

S

K

K

-

N

D

D

P

P

I

I

P

A

R

G

F

L

E

K

E

M

K

H

L

L

V

I

E

D

S

L

G

G

V

I

L

A

K

T

K

E

K

A

D

E

L

V

K

K

E

A

I

Y

K

D

A

K

R

S

I

A

E

R

K

K

E

Y

V

V

E

D

E

E

A

Q

V

V

A

E

F

L

A

A

E

D

Q

A

S

A

P

P

L

P

P

P

D

E

P

A

-

K

-

L

S

S

V

I

L

L

M

F

D

E

D

D

V

V

Y

Y

S313 (≠ F244) modified: Phosphoserine; by PDK1 and PDK2

Sites not aligning to the query:

1:33 modified: transit peptide, Mitochondrion

Q8H1Y0 Pyruvate dehydrogenase E1 component subunit alpha-2, mitochondrial; PDHE1-A; Protein IAA-CONJUGATE-RESISTANT 4; EC 1.2.4.1 from Arabidopsis thaliana (Mouse-ear cress) (see paper)
38% identity, 98% coverage: 6:318/319 of query aligns to 61:372/393 of Q8H1Y0

query
sites
Q8H1Y0

K

E

Q

E

M

I

V

L

S

A

M

F

Y

F

E

R

T

D

M

M

N

A

R

R

I

M

R

R

L

R

F

M

E

E

T

I

K

A

L

A

Q

D

E

S

F

L

F

Y

A

K

A

A

G

K

Q

L

I

I

P

R

G

G

F

F

V

C

H

H

L

L

Y

Y

L

D

G

G

E

Q

E

E

A

A

V

L

A

A

T

V

G

G

A

M

C

E

A

A

G

A

L

I

T

T

N

K

D

D

K

A

I

I

T

I

S

T

T

S

H

Y

R
|
R

G

D

H

H

G

C

H

T

L

F

L

I

A

G

K

R

G

G

D

K

L

L

K

V

L

D

M

A

M

F

A

S

E

E

I

L

F

M

G

G

R

R

K

K

D

T

G

G

Y

C

C

S

K

H

G

G

K

K

G

G

S

S

M

M

H

H

I

F

A

Y

D

K

F

K

D

D

L

A

G

S

I

F

L

Y

G

G

A

G

N

H

G

G

I

I

V

V

G

G

G

A

G

Q

G

I

P

P

L

L

A

G

L

C

G

G

A

L

A

A

L

F

A

A

N

Q

K

K

Y

Y

E

N

K

K

T

D

D

E

D

A

V

V

V

T

I

F

C

A

F

L

F

Y

G

G

D

D

G

G

A

A

S

A

N

N

Q

Q

G

G

T

Q

T

L

Q

F

E

E

S

A

L

L

N

N

M

I

A

S

S

A

A

L

W

W

K

D

L

L

P

P

L

A

V

I

F

L

V

V

N

C

E

E

N

N

G

H

Y

Y

G

G

I

M

S

G

C

T

P

A

Q

T

C

W

K

R

S

S

M

A

A

K

V

S

T

P

D

A

I

Y

A

F

D

K

R

R

A

G

A

D

A

Y

Y

-

D

-

M

V

P

P

G

G

V

L

V

K

V

V

D

D

G

G

N

M

D

D

V

A

L

L

A

A

V

V

H

K

E

Q

A

A

V

C

S

K

E

F

A

A

V

K

K

E

R

H

A

A

R

L

N

K

G

N

E

-

G

G

P

P

S

I

L

I

V

L

E

E

C

M

K

D

T

T

Y

Y

R

R

W

Y

R

H

G

G

H

H

F

S

E

M

G

S

D

D

-

P

A

G

C

S

T

T

Y

Y

R

R

C

T

D

R

E

D

E

E

L

I

K

S

-

G

E

V

W

R

M

Q

A

V

K

R

D

D

P

P

I

I

P

E

R

R

F

V

E

R

E

K

K

L

L

L

V

L

E

T

S

H

G

D

V

I

L

A

K

T

K

E

K

K

D

E

L

L

K

K

E

D

I

M

K

E

A

K

R

E

I

I

E

R

K

K

E

E

V

V

E

D

A

A

V

V

A

A

F

Q

A

A

E

K

Q

E

S

S

P

P

L

I

P

P

D

D

P

A

S

S

V

E

L

L

M

F

D

T

D

N

V

M

Y

Y

R121 (= R66) mutation to C: In iar4-1; reduced sensitivity to several IAA-amino acid conjugates.

P08559 Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; PDHE1-A type I; EC 1.2.4.1 from Homo sapiens (Human) (see 16 papers)
40% identity, 97% coverage: 9:319/319 of query aligns to 62:370/390 of P08559

query
sites
P08559

V

L

S

K

M

Y

Y

Y

E

R

T

M

M

M

N

Q

R

T

I

V

R

R

L

R

F

M

E

E

T

L

K

K

L

A

Q

D

E

Q

F

L

F

Y

A

K

A

Q

G

K

Q

I

I

I

P

R

G

G

F

F

V

C

H

H

L

L

Y

C

L

D

G

G

E

Q

E

E

A

A

V

C

A

C

T

V

G

G

A

L

C

E

A

A

G

G

L

I

T

N

N

P

N

T

D

D

K

H

I

L

T

I

S

T

T

A

H
x
Y

R
|
R

G

A

H

H

G

G

H

F

L

T

L

F

A

T

K

R

G

G

G

L

D

S

L

V

K

R

L

E

M

I

M

L

A
|
A

E

E

I

L

F

T

G

G

R

R

K

K

D

G

G

G

Y

C

C

A

K

K

G

G

K

K

G

G

S

S

M

M

H

H

I

M

A

-

D

-

F

Y

D

A

L

K

G

N

I

F

L

Y

G

G

A

G

N

N

G
|
G

I

I

V
|
V

G

G

G

A

G

Q

G

V

P

P

L

L

A

G

L

A

G

G

A

I

A

A

L

L

A

A

N

C

K

K

Y

Y

E

N

K

G

T

K

D

D

D

E

V

V

V

C

I

L

C

T

F

L

F

Y

G

G

D
|
D

G
|
G

A
|
A

S

A

N

N

Q

Q

G

G

T

Q

T

I

Q

F

E

E

S

A

L

Y

N

N

M

M

A

A

S

A

A

L

W

W

K

K

L

L

P

P

L

C

V

I

F

F

V

I

N

C

E

E

N

N

N
|
N

G

R

Y
|
Y

G

G

I

M

S

G

C

T

P
x
S

Q

V

C

E

K

R

S

A

M

A

A

A

V

S

T

T

D

D

I

Y

A

Y

D

K

R

R

A

G

A

D

A

F

Y

-

D

-

M

I

P

P

G

G

V

L

V

R

V

V

D

D

G

G

N

M

D

D

V

I

L

L

A

C

V

V

H

R

E

E

A

A

V

T

S

R

E

F

A

A

V

A

K

A

R

Y

A

C

R

R

N

S

G

G

E

K

G

G

P

P

S

I

L

L

V
x
M

E

E

C

L

K

Q

T

T

Y

Y

R

R

W

Y

R

H

G

G

H
|
H

F
x
S

E

M

G

S

D

D

-

P

A

G

C

V

T
x
S

Y

Y

R
|
R

C

T

D

R

E

E

L

I

K

Q

E

E

W

V

M

R

A

S

K

K

-

S

D

D

P

P

I

I

P

M

R

L

F

L

E

K

E

D

K

R

L

M

V

V

E

N

S

S

G

N

V

L

L

A

K

S

K

V

K

E

D

E

L

L

K

K

E

E

I

I

K

D

A

V

R

E

I

V

E

R

K

K

E

E

V

I

E

E

E

D

A

A

V

A

A

Q

F

F

A

A

E

T

Q

A

S

D

P

P

L

E

P

P

D

P

P

L

S

E

V

E

L

L

M

G

D

Y

D

H

V

I

Y

Y

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S

Y118 (≠ H65) binding thiamine diphosphate
R119 (= R66) binding thiamine diphosphate
A136 (= A83) to T: found in a patient with moderate developmental delay, mild dysmorphism and mildly elevated serum lactate; uncertain significance; dbSNP:rs138727886
G165 (= G114) binding thiamine diphosphate
V167 (= V116) binding thiamine diphosphate; to M: in PDHAD; disrupts magnesium binding and results in deficient activity of the pyruvate dehydrogenase complex; dbSNP:rs2063174067
D196 (= D145) binding Mg(2+); binding thiamine diphosphate
G197 (= G146) binding thiamine diphosphate
A198 (= A147) binding thiamine diphosphate
N225 (= N174) binding Mg(2+); binding thiamine diphosphate
Y227 (= Y176) binding Mg(2+)
S232 (≠ P181) modified: Phosphoserine; by PDK1; mutation to A: Abolishes inactivation by phosphorylation; when associated with A-293 and A-300.
M282 (≠ V233) to L: in dbSNP:rs2229137
H292 (= H243) binding thiamine diphosphate
S293 (≠ F244) modified: Phosphoserine; by PDK1, PDK2, PDK3 and PDK4; mutation to A: Reduces enzyme activity. Abolishes inactivation by phosphorylation; when associated with A-232 and A-300.; mutation to E: Interferes with substrate binding.
S300 (≠ T250) modified: Phosphoserine; by PDK1, PDK2, PDK3 and PDK4; mutation to A: Abolishes inactivation by phosphorylation; when associated with A-232 and A-293.
R302 (= R252) to C: in PDHAD; loss of activity; common mutation; dbSNP:rs137853252

Sites not aligning to the query:

1:29 modified: transit peptide, Mitochondrion
10 R → P: in PDHAD; affects mitochondrial import of precursor protein; dbSNP:rs137853257

6cfoA Human pyruvate dehydrogenase e1 component complex with covalent tdp adduct acetyl phosphinate (see paper)
40% identity, 97% coverage: 9:319/319 of query aligns to 34:342/362 of 6cfoA

query
sites
6cfoA

V

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Y

Y

Y

E

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M

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N

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x
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A

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A

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A

C

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G

A

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E

A

A

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G

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N

P

N

T

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D

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T

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x
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R

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A

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S

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M

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H

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-

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-

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L

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G

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A

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N

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Q

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G

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A

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A

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K

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A

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-

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-

S

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P

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I

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M

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L

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K

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K

R

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K

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E

L

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K

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E

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E

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V

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R

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K

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A

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active site: Q52 (≠ E27), G137 (= G114), R260 (= R239), H264 (= H243), S265 (≠ F244), Y273 (= Y251)
binding 3-[(4-amino-2-methylpyrimidin-5-yl)methyl]-2-{(1S)-1-hydroxy-1-[(R)-hydroxy(oxo)-lambda~5~-phosphanyl]ethyl}-5-(2-{[(S)-hydroxy(phosphonooxy)phosphoryl]oxy}ethyl)-4-methyl-1,3-thiazol-3-ium: F62 (= F37), Y90 (≠ H65), R91 (= R66), G137 (= G114), V139 (= V116), G167 (= G144), D168 (= D145), G169 (= G146), N197 (= N174), Y199 (= Y176), G200 (= G177), H264 (= H243)
binding magnesium ion: D168 (= D145), N197 (= N174), Y199 (= Y176)

1ni4A Human pyruvate dehydrogenase (see paper)
40% identity, 97% coverage: 9:319/319 of query aligns to 34:342/362 of 1ni4A

query
sites
1ni4A

V

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Y

Y

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x
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A

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A

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A

A

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G

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N

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M

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S

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D

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P

I

I

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M

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F

L

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K

E

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K

R

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N

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S

K

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K

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E

L

L

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K

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E

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V

E

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K

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E

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A

A

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A

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F

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P

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P

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E

L

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D

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I

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Y

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active site: Q52 (≠ E27), G137 (= G114), R260 (= R239), H264 (= H243), S265 (≠ F244), Y273 (= Y251)
binding magnesium ion: D168 (= D145), N197 (= N174), Y199 (= Y176)
binding thiamine diphosphate: Y90 (≠ H65), R91 (= R66), V139 (= V116), G167 (= G144), D168 (= D145), G169 (= G146), A170 (= A147), N197 (= N174), G200 (= G177), H264 (= H243)

3exeA Crystal structure of the pyruvate dehydrogenase (e1p) component of human pyruvate dehydrogenase complex (see paper)
40% identity, 97% coverage: 9:319/319 of query aligns to 35:343/363 of 3exeA

query
sites
3exeA

V

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x
Q

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A

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C

A

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G

A

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A

A

G

G

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N

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x
Y

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R

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A

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M

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G

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K

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K

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H

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-

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D

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G

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V

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G

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L

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A

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A

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A

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N

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Q

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W

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K

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Y

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G

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D

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A

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M

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x
S

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K

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S

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P

I

I

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M

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F

L

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K

E

D

K

R

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N

V

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L

A

K

S

K

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K

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D

E

L

L

K

K

E

E

I

I

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D

A

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R

E

I

V

E

R

K

K

E

E

V

I

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E

E

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A

A

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A

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F

A

A

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A

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E

L

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D

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D

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I

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Y

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active site: Q53 (≠ E27), G138 (= G114), R261 (= R239), H265 (= H243), S266 (≠ F244), Y274 (= Y251)
binding manganese (ii) ion: D169 (= D145), N198 (= N174), Y200 (= Y176)
binding thiamine diphosphate: Y91 (≠ H65), R92 (= R66), V140 (= V116), G168 (= G144), D169 (= D145), G170 (= G146), A171 (= A147), N198 (= N174), Y200 (= Y176), G201 (= G177), H265 (= H243)

6cerA Human pyruvate dehydrogenase complex e1 component v138m mutation (see paper)
38% identity, 97% coverage: 9:319/319 of query aligns to 35:322/342 of 6cerA

query
sites
6cerA

V

L

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Y

Y

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x
Q

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A

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A

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G

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A

A

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G

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N

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x
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R

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K

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G

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M

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P

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L

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A

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L

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K

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D

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A

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N

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Q

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G

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T

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Q

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A

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N

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A

A

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W

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K

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P

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N

G

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G

-

I

-

S

-

C

-

P

-

Q

-

C

-

K

R

S

A

M

A

A

A

V

S

T

T

D

D

I

Y

A

Y

D

K

R

R

A

G

A

D

A

F

Y

-

D

-

M

I

P

P

G

G

V

L

V

R

V

V

D

D

G

G

N

M

D

D

V

I

L

L

A

C

V

V

H

R

E

E

A

A

V

T

S

R

E

F

A

A

V

A

K

A

R

Y

A

C

R

R

N

S

G

G

E

K

G

G

P

P

S

I

L

L

V

M

E

E

C

L

K

Q

T

T

Y

Y

R
|
R

W

-

R

-

G

-

H

-

F

-

E

-

G

-

D

-

A

-

C

-

T

-

Y

Y

R

H

C

T

D

R

E

E

L

I

K

Q

E

E

W

V

M

R

A

S

K

K

-

S

D

D

P

P

I

I

P

M

R

L

F

L

E

K

E

D

K

R

L

M

V

V

E

N

S

S

G

N

V

L

L

A

K

S

K

V

K

E

D

E

L

L

K

K

E

E

I

I

K

D

A

V

R

E

I

V

E

R

K

K

E

E

V

I

E

E

E

D

A

A

V

A

A

Q

F

F

A

A

E

T

Q

A

S

D

P

P

L

E

P

P

D

P

P

L

S

E

V

E

L

L

M

G

D

Y

D

H

V

I

Y

Y

A

S

active site: Q53 (≠ E27), G138 (= G114), R252 (= R239)
binding thiamine diphosphate: Y91 (≠ H65), R92 (= R66), M140 (≠ V116), R252 (= R239)

6cerE Human pyruvate dehydrogenase complex e1 component v138m mutation (see paper)
37% identity, 97% coverage: 9:319/319 of query aligns to 34:320/340 of 6cerE

query
sites
6cerE

V

L

S

K

M

Y

Y

Y

E

R

T

M

M

M

N

Q

R

T

I

V

R

R

L

R

F

M

E

E

T

L

K

K

L

A

Q

D

E
x
Q

F

L

F

Y

A

K

A

Q

G

K

Q

I

I

I

P

R

G

G

F

F

V

C

H

H

L

L

Y

C

L

D

G

G

E

Q

E

E

A

A

V

C

A

C

T

V

G

G

A

L

C

E

A

A

G

G

L

I

T

N

N

P

N

T

D

D

K

H

I

L

T

I

S

T

T

A

H
x
Y

R
|
R

G

A

H

H

G

G

H

F

L

T

L

F

A

T

K

R

G

G

G

L

D

S

L

V

K

R

L

E

M

I

M

L

A

A

E

E

I

L

F

T

G

G

R

R

K

K

D

G

G

G

Y

C

C

A

K

K

G

G

K

K

G

G

S

S

M

M

H

H

I

M

A

-

D

-

F

Y

D

A

L

K

G

N

I

F

L

Y

G

G

A

G

N

N

G
|
G

I

I

V
x
M

G

G

G

A

G

Q

G

V

P

P

L

L

A

G

L

A

G

G

A

I

A

A

L

L

A

A

N

C

K

K

Y

Y

E

N

K

G

T

K

D

D

D

E

V

V

V

C

I

L

C

T

F

L

F

Y

G

G

D

D

G

G

A

A

S

A

N

N

Q

Q

G

G

T

Q

T

I

Q

F

E

E

S

A

L

Y

N

N

M

M

A

A

S

A

A

L

W

W

K

K

L

L

P

P

L

C

V

I

F

F

V

I

N

C

E

E

N

N

G

R

Y

-

G

-

I

-

S

-

C

-

P

-

Q

-

C

-

K

R

S

A

M

A

A

A

V

S

T

T

D

D

I

Y

A

Y

D

K

R

R

A

G

A

D

A

F

Y

-

D

-

M

I

P

P

G

G

V

L

V

R

V

V

D

D

G

G

N

M

D

D

V

I

L

L

A

C

V

V

H

R

E

E

A

A

V

T

S

R

E

F

A

A

V

A

K

A

R

Y

A

C

R

R

N

S

G

G

E

K

G

G

P

P

S

I

L

L

V

M

E

E

C

L

K

Q

T

T

Y

Y

R
|
R

W

Y

R
x
H

G

-

H

-

F

-

E

-

G

-

D

-

A

-

C

-

T

-

Y

-

R

-

C

-

D

-

E

E

L

I

K

Q

E

E

W

V

M

R

A

S

K

K

-

S

D

D

P

P

I

I

P

M

R

L

F

L

E

K

E

D

K

R

L

M

V

V

E

N

S

S

G

N

V

L

L

A

K

S

K

V

K

E

D

E

L

L

K

K

E

E

I

I

K

D

A

V

R

E

I

V

E

R

K

K

E

E

V

I

E

E

E

D

A

A

V

A

A

Q

F

F

A

A

E

T

Q

A

S

D

P

P

L

E

P

P

D

P

P

L

S

E

V

E

L

L

M

G

D

Y

D

H

V

I

Y

Y

A

S

active site: Q52 (≠ E27), G137 (= G114), R252 (= R239)
binding magnesium ion: R252 (= R239), H254 (≠ R241)
binding thiamine diphosphate: Y90 (≠ H65), R91 (= R66), M139 (≠ V116), R252 (= R239)

3exhE Crystal structure of the pyruvate dehydrogenase (e1p) component of human pyruvate dehydrogenase complex (see paper)
36% identity, 97% coverage: 9:319/319 of query aligns to 34:311/331 of 3exhE

query
sites
3exhE

V

L

S

K

M

Y

Y

Y

E

R

T

M

M

M

N

Q

R

T

I

V

R

R

L

R

F

M

E

E

T

L

K

K

L

A

Q

D

E
x
Q

F

L

F

Y

A

K

A

Q

G

K

Q

I

I

I

P

R

G

G

F

F

V

C

H

H

L

L

Y

C

L

D

G

G

E

Q

E

E

A

A

V

C

A

C

T

V

G

G

A

L

C

E

A

A

G

G

L

I

T

N

N

P

N

T

D

D

K

H

I

L

T

I

S

T

T

A

H
x
Y

R
|
R

G

A

H

H

G

G

H

F

L

T

L

F

A

T

K

R

G

G

G

L

D

S

L

V

K

R

L

E

M

I

M

L

A

A

E

E

I

L

F

T

G

G

R

R

K

K

D

G

G

G

Y

C

C

A

K

K

G

G

K

K

G

G

S

S

M

M

H

H

I

M

A

-

D

-

F

Y

D

A

L

K

G

N

I

F

L

Y

G

G

A

G

N

N

G
|
G

I

I

V
|
V

G

G

G

A

G

Q

G

V

P

P

L

L

A

G

L

A

G

G

A

I

A

A

L

L

A

A

N

C

K

K

Y

Y

E

N

K

G

T

K

D

D

D

E

V

V

V

C

I

L

C

T

F

L

F

Y

G
|
G

D
|
D

G
|
G

A

A

S

A

N

N

Q

Q

G

G

T

Q

T

I

Q

F

E

E

S

A

L

Y

N

N

M

M

A

A

S

A

A

L

W

W

K

K

L

L

P

P

L

C

V

I

F

F

V

I

N

C

E

E

N

N

N
|
N

G

-

Y

-

G

-

I

-

S

-

C

-

P

-

Q

R

C

E

K

R

S

A

M

A

A

A

V

S

T

T

D

D

I

Y

A

Y

D

K

R

R

A

G

A

D

A

F

Y

-

D

-

M

I

P

P

G

G

V

L

V

R

V

V

D

D

G

G

N

M

D

D

V

I

L

L

A

C

V

V

H

R

E

E

A

A

V

T

S

R

E

F

A

A

V

A

K

A

R

Y

A

C

R

R

N

S

G

G

E

K

G

G

P

P

S

I

L

L

V

M

E

E

C

L

K

Q

T

T

Y

Y

R

S

W

-

R

-

G

-

H

-

F

-

E

-

G

-

D

-

A

-

C

-

T

-

Y

-

R

-

C

-

D

-

E

-

L

-

K

-

E

-

W

-

M

-

A

K

K

S

D

D

P

P

I

I

P

M

R

L

F

L

E

K

E

D

K

R

L

M

V

V

E

N

S

S

G

N

V

L

L

A

K

S

K

V

K

E

D

E

L

L

K

K

E

E

I

I

K

D

A

V

R

E

I

V

E

R

K

K

E

E

V

I

E

E

E

D

A

A

V

A

A

Q

F

F

A

A

E

T

Q

A

S

D

P

P

L

E

P

P

D

P

P

L

S

E

V

E

L

L

M

G

D

Y

D

H

V

I

Y

Y

A

S

active site: Q52 (≠ E27), G137 (= G114)
binding manganese (ii) ion: D168 (= D145), N197 (= N174)
binding thiamine diphosphate: Y90 (≠ H65), R91 (= R66), V139 (= V116), G167 (= G144), D168 (= D145), G169 (= G146), N197 (= N174)

1umdA Branched-chain 2-oxo acid dehydrogenase (e1) from thermus thermophilus hb8 with 4-methyl-2-oxopentanoate as an intermediate (see paper)
33% identity, 98% coverage: 6:319/319 of query aligns to 31:345/362 of 1umdA

query
sites
1umdA

K

E

Q

K

M

L

V

R

S

R

M

L

Y

Y

E

R

T

D

M

M

N

L

R

A

I

A

R

R

L

M

F

L

E

D

T

E

K

R

L

Y

Q

T

E
x
I

F

L

F

I

A

R

A

T

G

G

Q

K

I

T

P

-

G

S

F
|
F

V

I

H

A

L

P

Y

A

L

A

G

G

E

H

E

E

A

A

V

A

A

Q

T

V

G

A

A

I

C

A

A

H

G

A

L

I

T

R

N

P

N

G

-

F

D

D

K

W

I

V

T

F

S

P

T
x
Y

H
x
Y

R
|
R

G

D

H

H

G

G

H

L

L

A

L

L

A

A

K

L

G

G

G

I

D

P

L

L

K

K

L

E

M

L

A

G

E

Q

I

M

F

L

G

A

R

T

K

K

D

A

G

D

Y

P

C

N

K

K

G

G

K

R

G

Q

G

M

S

P

M

E

H

H

I

P

A

G

D

S

F

K

D

A

L

L

G

N

I

F

L

F

G

T

A

V

N

A

G
x
S

I
x
P

V
x
I

G

A

G

S

G

H

G

V

P

P

L

P

A

A

L

A

G

G

A

A

L

I

A

S

N

M

K

K

Y

L

E

L

K

R

T

T

D

G

D

Q

V

V

V

A

I

V

C

C

F

T

F

F

G
|
G

D
|
D

G
|
G

A

A

S

T

N

S

Q

E

G

G

T

D

T

W

Q

Y

E

A

S

G

L

I

N

N

M

F

A

A

S

A

A

V

W

Q

K

G

L

A

P

P

L

A

V

V

F

F

V

I

N

A

E

E

N

N

N
|
N

G

F

Y
|
Y

G
x
A

I
|
I

S

S

C

V

P

D

Q

Y

C

R

K

H

S

Q

M

T

A

H

V

S

T

P

D

T

I

I

A

A

D

D

R

K

A

A

A

H

A

A

Y

F

D

G

M

I

P

P

G

G

V

Y

V

L

V

V

D

D

G

G

N

M

D

D

V

V

L

L

A

A

V

S

H

Y

E

Y

A

V

V

V

S

K

E

E

A

A

V

V

K

E

R

R

A

A

R

R

N

R

G

G

E

E

G

G

P

P

S

S

L

L

V

V

E

E

C

L

K

R

T

V

Y

Y

R
|
R

W

Y

R

G

G

P

H
|
H

F
x
S

E

S

G

A

-

D

D

D

A

D

C

S

T

R

Y
|
Y

R

R

C

P

D

K

E

E

L

V

K

A

E

F

W

W

M

R

A

K

K

K

D

D

P

P

I

I

P

P

R

R

F

F

E

R

K

F

L

L

V

E

E

A

S

R

G

G

V

L

L

W

K

N

K

E

D

W

L

E

K

E

E

D

I

V

K

R

A

E

R

E

I

I

E

R

K

A

E

E

V

L

E

E

E

R

A

G

V

L

A

K

F

E

A

A

E

E

Q

E

S

A

-

G

P

P

L

V

P

P

D

-

P

P

S

E

V

W

L

M

M

F

D

E

D

D

V

V

Y

F

A

A

active site: I52 (≠ E27), S139 (≠ G114), R264 (= R239), H268 (= H243), S269 (≠ F244), Y277 (= Y251)
binding 2-oxo-4-methylpentanoic acid: F61 (= F37), Y90 (≠ H65), S139 (≠ G114)
binding magnesium ion: D170 (= D145), N199 (= N174), Y201 (= Y176)
binding thiamine diphosphate: Y89 (≠ T64), Y90 (≠ H65), R91 (= R66), P140 (≠ I115), I141 (≠ V116), G169 (= G144), D170 (= D145), G171 (= G146), N199 (= N174), Y201 (= Y176), A202 (≠ G177), I203 (= I178), H268 (= H243)

1umcA Branched-chain 2-oxo acid dehydrogenase (e1) from thermus thermophilus hb8 with 4-methylpentanoate (see paper)
33% identity, 98% coverage: 6:319/319 of query aligns to 31:345/362 of 1umcA

query
sites
1umcA

K

E

Q

K

M

L

V

R

S

R

M

L

Y

Y

E

R

T

D

M

M

N

L

R

A

I

A

R

R

L

M

F

L

E

D

T

E

K

R

L

Y

Q

T

E
x
I

F

L

F

I

A

R

A

T

G

G

Q

K

I

T

P

-

G

S

F

F

V

I

H

A

L

P

Y

A

L

A

G

G

E

H

E

E

A

A

V

A

A

Q

T

V

G

A

A

I

C

A

A

H

G

A

L

I

T

R

N

P

N

G

-

F

D

D

K

W

I

V

T

F

S

P

T
x
Y

H
x
Y

R
|
R

G

D

H

H

G

G

H

L

L

A

L

L

A

A

K

L

G

G

G

I

D

P

L

L

K

K

L

E

M

L

A

G

E

Q

I

M

F

L

G

A

R

T

K

K

D

A

G

D

Y

P

C

N

K

K

G

G

K

R

G

Q

G

M

S

P

M

E

H
|
H

I

P

A

G

D

S

F

K

D

A

L

L

G

N

I

F

L

F

G

T

A

V

N

A

G
x
S

I

P

V
x
I

G

A

G

S

G

H

G

V

P

P

L

P

A

A

L

A

G

G

A

A

L

I

A

S

N

M

K

K

Y

L

E

L

K

R

T

T

D

G

D

Q

V

V

V

A

I

V

C

C

F

T

F

F

G
|
G

D
|
D

G
|
G

A

A

S

T

N

S

Q

E

G

G

T

D

T

W

Q

Y

E

A

S

G

L

I

N

N

M

F

A

A

S

A

A

V

W

Q

K

G

L

A

P

P

L

A

V

V

F

F

V

I

N

A

E

E

N

N

N
|
N

G

F

Y
|
Y

G

A

I
|
I

S

S

C

V

P

D

Q

Y

C

R

K

H

S

Q

M

T

A

H

V

S

T

P

D

T

I

I

A

A

D

D

R

K

A

A

A

H

A

A

Y

F

D

G

M

I

P

P

G

G

V

Y

V

L

V

V

D

D

G

G

N

M

D

D

V

V

L

L

A

A

V

S

H

Y

E

Y

A

V

V

V

S

K

E

E

A

A

V

V

K

E

R

R

A

A

R

R

N

R

G

G

E

E

G

G

P

P

S

S

L

L

V

V

E

E

C

L

K

R

T

V

Y

Y

R
|
R

W

Y

R

G

G

P

H
|
H

F
x
S

E

S

G

A

-

D

D

D

A

D

C

S

T

R

Y
|
Y

R

R

C

P

D

K

E

E

L

V

K

A

E

F

W

W

M

R

A

K

K

K

D

D

P

P

I

I

P

P

R

R

F

F

E

R

K

F

L

L

V

E

E

A

S

R

G

G

V

L

L

W

K

N

K

E

D

W

L

E

K

E

E

D

I

V

K

R

A

E

R

E

I

I

E

R

K

A

E

E

V

L

E

E

E

R

A

G

V

L

A

K

F

E

A

A

E

E

Q

E

S

A

-

G

P

P

L

V

P

P

D

-

P

P

S

E

V

W

L

M

M

F

D

E

D

D

V

V

Y

F

A

A

active site: I52 (≠ E27), S139 (≠ G114), R264 (= R239), H268 (= H243), S269 (≠ F244), Y277 (= Y251)
binding 4-methyl valeric acid: Y90 (≠ H65), H126 (= H101)
binding magnesium ion: D170 (= D145), N199 (= N174), Y201 (= Y176)
binding thiamine diphosphate: Y89 (≠ T64), Y90 (≠ H65), R91 (= R66), I141 (≠ V116), G169 (= G144), D170 (= D145), G171 (= G146), N199 (= N174), Y201 (= Y176), I203 (= I178), H268 (= H243)

1umbA Branched-chain 2-oxo acid dehydrogenase (e1) from thermus thermophilus hb8 in holo-form (see paper)
33% identity, 98% coverage: 6:319/319 of query aligns to 31:345/362 of 1umbA

query
sites
1umbA

K

E

Q

K

M

L

V

R

S

R

M

L

Y

Y

E

R

T

D

M

M

N

L

R

A

I

A

R

R

L

M

F

L

E

D

T

E

K

R

L

Y

Q

T

E
x
I

F

L

F

I

A

R

A

T

G

G

Q

K

I

T

P

-

G

S

F

F

V

I

H

A

L

P

Y

A

L

A

G

G

E

H

E

E

A

A

V

A

A

Q

T

V

G

A

A

I

C

A

A

H

G

A

L

I

T

R

N

P

N

G

-

F

D

D

K

W

I

V

T

F

S

P

T
x
Y

H
x
Y

R
|
R

G

D

H

H

G

G

H

L

L

A

L

L

A

A

K

L

G

G

G

I

D

P

L

L

K

K

L

E

M

L

A

G

E

Q

I

M

F

L

G

A

R

T

K

K

D

A

G

D

Y

P

C

N

K

K

G

G

K

R

G

Q

G

M

S

P

M

E

H

H

I

P

A

G

D

S

F

K

D

A

L

L

G

N

I

F

L

F

G

T

A

V

N

A

G
x
S

I
x
P

V
x
I

G

A

G

S

G

H

G

V

P

P

L

P

A

A

L

A

G

G

A

A

L

I

A

S

N

M

K

K

Y

L

E

L

K

R

T

T

D

G

D

Q

V

V

V

A

I

V

C

C

F

T

F

F

G
|
G

D
|
D

G
|
G

A

A

S

T

N

S

Q

E

G

G

T

D

T

W

Q

Y

E

A

S

G

L

I

N

N

M

F

A

A

S

A

A

V

W

Q

K

G

L

A

P

P

L

A

V

V

F

F

V

I

N

A

E

E

N

N

N
|
N

G

F

Y
|
Y

G
x
A

I
|
I

S

S

C

V

P

D

Q

Y

C

R

K

H

S

Q

M

T

A

H

V

S

T

P

D

T

I

I

A

A

D

D

R

K

A

A

A

H

A

A

Y

F

D

G

M

I

P

P

G

G

V

Y

V

L

V

V

D

D

G

G

N

M

D

D

V

V

L

L

A

A

V

S

H

Y

E

Y

A

V

V

V

S

K

E

E

A

A

V

V

K

E

R

R

A

A

R

R

N

R

G

G

E

E

G

G

P

P

S

S

L

L

V

V

E

E

C

L

K

R

T

V

Y

Y

R
|
R

W

Y

R

G

G

P

H
|
H

F
x
S

E

S

G

A

-

D

D

D

A

D

C

S

T

R

Y
|
Y

R

R

C

P

D

K

E

E

L

V

K

A

E

F

W

W

M

R

A

K

K

K

D

D

P

P

I

I

P

P

R

R

F

F

E

R

K

F

L

L

V

E

E

A

S

R

G

G

V

L

L

W

K

N

K

E

D

W

L

E

K

E

E

D

I

V

K

R

A

E

R

E

I

I

E

R

K

A

E

E

V

L

E

E

E

R

A

G

V

L

A

K

F

E

A

A

E

E

Q

E

S

A

-

G

P

P

L

V

P

P

D

-

P

P

S

E

V

W

L

M

M

F

D

E

D

D

V

V

Y

F

A

A

active site: I52 (≠ E27), S139 (≠ G114), R264 (= R239), H268 (= H243), S269 (≠ F244), Y277 (= Y251)
binding magnesium ion: D170 (= D145), N199 (= N174), Y201 (= Y176)
binding thiamine diphosphate: Y89 (≠ T64), Y90 (≠ H65), R91 (= R66), P140 (≠ I115), I141 (≠ V116), G169 (= G144), D170 (= D145), G171 (= G146), N199 (= N174), Y201 (= Y176), A202 (≠ G177), I203 (= I178), H268 (= H243)

Q5SLR4 2-oxoisovalerate dehydrogenase subunit alpha; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDH E1-alpha; EC 1.2.4.4 from Thermus thermophilus (strain ATCC 27634 / DSM 579 / HB8) (see paper)
33% identity, 98% coverage: 6:319/319 of query aligns to 36:350/367 of Q5SLR4

query
sites
Q5SLR4

K

E

Q

K

M

L

V

R

S

R

M

L

Y

Y

E

R

T

D

M

M

N

L

R

A

I

A

R

R

L

M

F

L

E

D

T

E

K

R

L

Y

Q

T

E

I

F

L

F

I

A

R

A

T

G

G

Q

K

I

T

P

-

G

S

F
|
F

V

I

H

A

L

P

Y

A

L

A

G

G

E

H

E

E

A

A

V

A

A

Q

T

V

G

A

A

I

C

A

A

H

G

A

L

I

T

R

N

P

N

G

-

F

D

D

K

W

I

V

T

F

S

P

T
x
Y

H
x
Y

R
|
R

G

D

H

H

G

G

H

L

L

A

L

L

A

A

K

L

G

G

G

I

D

P

L

L

K

K

L

E

M

L

A

G

E

Q

I

M

F

L

G

A

R

T

K

K

D

A

G

D

Y

P

C

N

K

K

G

G

K

R

G

Q

G
x
M

S
x
P

M
x
E

H
|
H

I

P

A

G

D

S

F

K

D

A

L

L

G

N

I

F

L

F

G

T

A

V

N

A

G
x
S

I
x
P

V
x
I

G

A

G

S

G

H

G

V

P

P

L

P

A

A

L

A

G

G

A

A

L

I

A

S

N

M

K

K

Y

L

E

L

K

R

T

T

D

G

D

Q

V

V

V

A

I

V

C

C

F

T

F

F

G
|
G

D
|
D

G
|
G

A
|
A

S
x
T

N
x
S

Q
x
E

G

G

T

D

T

W

Q

Y

E

A

S

G

L

I

N

N

M

F

A

A

S

A

A

V

W

Q

K

G

L

A

P

P

L

A

V

V

F

F

V

I

N

A

E

E

N

N

N
|
N

G
x
F

Y
|
Y

G
x
A

I
|
I

S

S

C

V

P

D

Q

Y

C

R

K

H

S

Q

M

T

A

H

V

S

T

P

D

T

I

I

A

A

D

D

R

K

A

A

A

H

A

A

Y

F

D

G

M

I

P

P

G

G

V

Y

V

L

V

V

D

D

G

G

N

M

D

D

V

V

L

L

A

A

V

S

H

Y

E

Y

A

V

V

V

S

K

E

E

A

A

V

V

K

E

R

R

A

A

R

R

N

R

G

G

E

E

G

G

P

P

S

S

L

L

V

V

E

E

C

L

K

R

T

V

Y

Y

R

R

W

Y

R

G

G

P

H
|
H

F

S

E

S

G

A

-

D

D

D

A

D

C

S

T

R

Y

Y

R

R

C

P

D

K

E

E

L

V

K

A

E

F

W

W

M

R

A

K

K

K

D

D

P

P

I

I

P

P

R

R

F

F

E

R

K

F

L

L

V

E

E

A

S

R

G

G

V

L

L

W

K

N

K

E

D

W

L

E

K

E

E

D

I

V

K

R

A

E

R

E

I

I

E

R

K

A

E

E

V

L

E

E

E

R

A

G

V

L

A

K

F

E

A

A

E

E

Q

E

S

A

-

G

P

P

L

V

P

P

D

-

P

P

S

E

V

W

L

M

M

F

D

E

D

D

V

V

Y

F

A

A

F66 (= F37) binding substrate
YYR 94:96 (≠ THR 64:66) binding thiamine diphosphate
Y95 (≠ H65) binding substrate
MPEH 128:131 (≠ GSMH 98:101) binding substrate
S144 (≠ G114) binding substrate
SPI 144:146 (≠ GIV 114:116) binding thiamine diphosphate
174:180 (vs. 144:150, 57% identical) binding thiamine diphosphate
D175 (= D145) binding Mg(2+)
N204 (= N174) binding Mg(2+)
NFYAI 204:208 (≠ NGYGI 174:178) binding thiamine diphosphate
Y206 (= Y176) binding Mg(2+)
H273 (= H243) binding thiamine diphosphate

P12694 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha; EC 1.2.4.4 from Homo sapiens (Human) (see 14 papers)
32% identity, 98% coverage: 5:318/319 of query aligns to 99:413/445 of P12694

query
sites
P12694

K

K

K

E

Q

K

M

V

V

L

S

K

M

L

Y

Y

E

K

T

S

M

M

N

T

R

L

I

L

R

N

L

T

F

M

E

D

T

R

K

I

L

L

Q

Y

E

E

F

S

F

Q

A

R

A

Q

G

G

Q

R

I

I

P

S

G

F

F

Y

V

M

H

T

L

N

Y

Y

L

-

G

G

E

E

A

G

V

T

A

H

T

V

G

G

A

S

C

A

A

A

G

A

L

L

T

D

N

N

N

T

D

D

K

L

I

V

T

F

S

G

T

Q

H
x
Y

R
|
R

G

E

H

A

G

G

H

V

L

L

L

M

A

Y

K

R

G

D

G

Y

D

P

L

L

K

E

L

L

M

F

M

M

A

A

E

Q

I

C

F

Y

G

G

R

N

K

I

D

S

G

D

Y

L

C

G

K

K

G

G

K

R

G
x
Q

G

M

S

P

M

V

H

H

I

Y

A

G

D

C

F

K

D

E

L

R

G

H

I

F

L

V

G

T

A

I

N
x
S

G
x
S

I
x
P

V

L

G

A

G
x
T

G
x
Q

G

I

P

P

L

Q

A

A

L

V

G

G

A

A

L

Y

A

A

N

A

K

K

Y

R

E

A

K

N

T

A

D

N

D

R

V

V

I

I

C

C

F

Y

F

F

G

G

D
x
E

G
|
G

A
|
A

S

A

N

S

Q

E

G

G

T

D

T

A

Q

H

E

A

S
x
G

L

F

N

N

M

F

A
|
A

S
x
A

A

T

W

L

K

E

L

C

P

P

L

I

V

I

F

F

V

F

N

C

E
x
R

N

N

N
|
N

G

G

Y
|
Y

G

A

I

I

S

S

C

T

P

P

Q

T

C

S

K

E

S

Q

M

Y

A

R

V

G

T

D

D

G

I

I

A
|
A

D

A

R

R

A

G

A

P

A
x
G

Y

Y

D

G

M

I

P

M

G

S

V

I

V
x
R

V

V

D

D

G

G

N

N

D

D

V

V

L

F

A

A

V

V

H

Y

E

N

A

A

V
x
T

S

K

E

E

A

A

V

R

K

R

R

R

A

A

R

V

N

A

G

E

E

N

G

Q

P

P

S

F

L

L

V
x
I

E

E

C

A

K

M

T

T

Y

Y

R

R

W

I

R

G

G

H

H
|
H

F
x
S

E

T

G

S

-

D

D

D

A

S

C

S

T

A

Y

Y

R

R

C
x
S

D

V

E

D

E

E

L

V

K

N

E

Y

W

W

M

D

A

K

K

Q

D

D

-

H

P

P

I

I

P

S

R

R

F

L

E

R

E

H

K

Y

L

L

V

L

E

S

S

Q

G

G

V

W

L

W

K

D

K

E

D

Q

L

E

K

K

E

A

I

W

K

R

A

K

R

Q

I

S

E

R

K

R

E

K

V

V

E

M

E

E

A

A

V

F

A

E

F

Q

A

A

E

E

Q

R

S

K

P

P

L

K

P

P

D

N

P

P

S

N

V

L

L

L

M
x
F

D

S

D

D

V

V

Y
|
Y

Y158 (≠ H65) binding thiamine diphosphate
R159 (= R66) binding thiamine diphosphate; to W: in MSUD1A; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs769688327
Q190 (≠ G97) to K: in MSUD1A; decreased 3-methyl-2-oxobutanoate dehydrogenase activity
S206 (≠ N113) binding K(+)
S207 (≠ G114) binding thiamine diphosphate
P208 (≠ I115) binding K(+)
T211 (≠ G118) binding K(+); to M: in MSUD1A; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs398123503
Q212 (≠ G119) binding K(+)
E238 (≠ D145) binding Mg(2+)
G239 (= G146) binding thiamine diphosphate
A240 (= A147) binding thiamine diphosphate
G249 (≠ S156) to S: in MSUD1A; no effect on solubility; no effect on mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs137852874
A253 (= A160) to T: in MSUD1A; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs199599175
A254 (≠ S161) to D: in MSUD1A; uncertain significance; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs373713279
R265 (≠ E172) binding thiamine diphosphate; to W: in MSUD1A; uncertain significance; no effect on solubility; no effect on mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs137852873
N267 (= N174) binding Mg(2+); to S: in MSUD1A; uncertain significance; no effect on solubility; no effect on mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; decreased affinity for the cofactor thiamine diphosphate; decreased 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs1568508047
Y269 (= Y176) binding Mg(2+)
A285 (= A192) to P: in MSUD1A; no effect on solubility; no effect on mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs398123508
G290 (≠ A197) to R: in MSUD1A; no effect on solubility; no effect on mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; decreased 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs137852871
R297 (≠ V204) to H: in MSUD1A; uncertain significance; decreased affinity for the cofactor thiamine diphosphate; decreased 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs200137189
T310 (≠ V217) to R: in MSUD1A; decreased solubility; changed mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs137852875
I326 (≠ V233) to T: in MSUD1A; decreased 3-methyl-2-oxobutanoate dehydrogenase activity
H336 (= H243) binding thiamine diphosphate
S337 (≠ F244) modified: Phosphoserine; by BCKDK; mutation to A: Substantially decreases the stability of the BCKD complex.
S347 (≠ C253) mutation to A: Does not affect the stability of the BCKD complex.
F409 (≠ M314) to C: in MSUD1A; decreased solubility; loss of mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs137852872
Y413 (= Y318) to C: in MSUD1A; uncertain significance; decreased solubility; changed mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs398123508

Sites not aligning to the query:

1:45 modified: transit peptide, Mitochondrion
438 Y → N: in MSUD1A; decreased solubility; loss of mitochondrial alpha-ketoglutarate dehydrogenase complex assembly; loss of 3-methyl-2-oxobutanoate dehydrogenase activity; dbSNP:rs137852870

P11960 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha; EC 1.2.4.4 from Rattus norvegicus (Rat) (see paper)
33% identity, 97% coverage: 9:318/319 of query aligns to 105:409/441 of P11960

query
sites
P11960

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S333 (≠ F244) modified: Phosphoserine; by BCKDK

Query Sequence

>WP_078717588.1 NCBI__GCF_900167125.1:WP_078717588.1
MSPDKKQMVSMYETMNRIRLFETKLQEFFAAGQIPGFVHLYLGEEAVATGACAGLTNNDK
ITSTHRGHGHLLAKGGDLKLMMAEIFGRKDGYCKGKGGSMHIADFDLGILGANGIVGGGG
PLALGAALANKYEKTDDVVICFFGDGASNQGTTQESLNMASAWKLPLVFVNENNGYGISC
PQCKSMAVTDIADRAAAYDMPGVVVDGNDVLAVHEAVSEAVKRARNGEGPSLVECKTYRW
RGHFEGDACTYRCDEELKEWMAKDPIPRFEEKLVESGVLKKKDLKEIKARIEKEVEEAVA
FAEQSPLPDPSVLMDDVYA

Or try a new SitesBLAST search

SitesBLAST's Database

SitesBLAST's database includes (1) SwissProt entries with experimentally-supported functional features; and (2) protein structures with bound ligands, from the BioLip database.

by Morgan Price, Arkin group
Lawrence Berkeley National Laboratory