SitesBLAST

SitesBLAST – Find functional sites

SitesBLAST

Comparing H281DRAFT_04625 H281DRAFT_04625 3-hydroxyacyl-CoA dehydrogenase 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)

P00348 Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; HCDH; L-3-hydroxyacyl CoA dehydrogenase; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase; EC 1.1.1.35 from Sus scrofa (Pig) (see paper)
33% identity, 36% coverage: 4:293/811 of query aligns to 25:312/314 of P00348

query
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P00348

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GGGLMG 34:39 (≠ GAGVMG 13:18) binding
D57 (= D36) binding
E122 (= E96) binding
K127 (= K101) binding
S149 (= S123) binding

Q16836 Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; HCDH; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase; EC 1.1.1.35 from Homo sapiens (Human) (see 7 papers)
32% identity, 36% coverage: 4:293/811 of query aligns to 25:312/314 of Q16836

query
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Q16836

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GGGLMG 34:39 (≠ GAGVMG 13:18) binding
A40 (= A19) to T: in HADH deficiency; dbSNP:rs137853101
D57 (= D36) binding ; to E: in HADH deficiency; dbSNP:rs137853102; to G: found in a patient with Reye-like syndrome. Does not affect 3-hydroxyacyl-CoA dehydrogenase activity. Increases KM value for NADH. Does not affect dimerization
S73 (≠ N54) binding
K80 (≠ A61) binding
L86 (≠ V65) to P: in dbSNP:rs4956145
E122 (= E96) binding
K127 (= K101) binding ; modified: N6-(2-hydroxyisobutyryl)lysine
S149 (= S123) binding ; binding
Q152 (≠ S126) to H: in dbSNP:rs1051519
N173 (= N149) binding
Y226 (≠ V207) to H: found in a patient with Reye-like syndrome; loss of 3-hydroxyacyl-CoA dehydrogenase activity. Does not affect dimerization; dbSNP:rs146036912
P258 (≠ T236) to L: in HHF4; loss of 3-hydroxyacyl-CoA dehydrogenase activity; dbSNP:rs137853103
K305 (= K286) binding

1f0yA L-3-hydroxyacyl-coa dehydrogenase complexed with acetoacetyl-coa and NAD+ (see paper)
32% identity, 36% coverage: 4:293/811 of query aligns to 2:289/291 of 1f0yA

query
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1f0yA

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active site: S126 (= S123), H147 (= H146), E159 (= E158), N197 (= N197)
binding acetoacetyl-coenzyme a: S50 (≠ N54), V54 (≠ L58), K57 (≠ A61), S126 (= S123), H147 (= H146), F148 (= F147), F149 (= F148), N150 (= N149), P151 (= P150), P153 (≠ R152), V154 (≠ Y153), M155 (= M154), N197 (= N197), L200 (≠ I204), G228 (= G230), A229 (≠ R231), P232 (≠ S234), M233 (vs. gap), L238 (≠ T239), Y241 (≠ V242), V242 (= V243)
binding nicotinamide-adenine-dinucleotide: I10 (≠ L12), G11 (= G13), G12 (≠ A14), G13 (= G15), L14 (≠ V16), M15 (= M17), D34 (= D36), Q35 (≠ L37), I39 (≠ E41), A96 (= A93), I97 (= I94), V98 (≠ A95), E99 (= E96), K104 (= K101), N124 (= N121), S126 (= S123), H147 (= H146), F148 (= F147), N150 (= N149), V242 (= V243), T246 (= T247), K282 (= K286)

1f17A L-3-hydroxyacyl-coa dehydrogenase complexed with nadh (see paper)
32% identity, 36% coverage: 4:293/811 of query aligns to 2:289/293 of 1f17A

query
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1f17A

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V

I

D

D

D

-

L

-

T

T

G

A

S

M

R

K

L

L

G

G

R

A

A

G

K

Y

S

P

-

M

A

G

T

P

F

F

R

E

T

L

A

L

D

D

V

Y

V

V

G

G

L

L

D

D

T

T

M

T

A

K

H

F

V

I

I

V

K

D

T

G

M

W

Q

H

D

E

N

M

L

-

K

-

D

-

D

D

P

A

F

E

F

N

P

P

V

L

Y

H

E

Q

T

P

P

S

A

P

V

S

L

L

A

N

E

K

L

L

V

V

K

A

K

E

G

N

A

K

L

F

G

G

Q

K

K

K

T

T

G

G

G

E

G

G

F

F

Y

Y

R

K

active site: S126 (= S123), H147 (= H146), E159 (= E158), N197 (= N197)
binding 1,4-dihydronicotinamide adenine dinucleotide: G11 (= G13), G12 (≠ A14), G13 (= G15), L14 (≠ V16), M15 (= M17), D34 (= D36), Q35 (≠ L37), I39 (≠ E41), A96 (= A93), I97 (= I94), V98 (≠ A95), E99 (= E96), K104 (= K101), N124 (= N121), S126 (= S123), H147 (= H146), F148 (= F147)

1f12A L-3-hydroxyacyl-coa dehydrogenase complexed with 3-hydroxybutyryl-coa (see paper)
32% identity, 36% coverage: 4:293/811 of query aligns to 2:289/293 of 1f12A

query
sites
1f12A

L

I

I

I

I

V

R

K

K

H

V

V

A

T

V

V

L

I

G

G

A

G

G

G

V

L

M

M

G

G

A

A

Q

G

I

I

A

A

A

Q

H

V

L

A

I

A

N

A

A

T

K

G

V

H

P

T

V

V

L

V

L

L

F

V

D

D

L

Q

P

T

A

E

K

D

E

I

G

L

P

A

K

K

N

S

A

K

I

K

A

G

L

I

K

E

A

-

I

-

E

E

N
x
S

L

L

K

R

K

K

L

V

S

A

P

K

A
x
K

P

K

F

F

G

A

V

E

K

N

D

P

D

K

A

A

-

G

-

D

Q

E

Y

C

I

V

Q

E

P

K

-

T

-

L

-

S

-

T

-

I

A

A

N

T

Y

S

D

T

D

D

D

A

I

A

E

S

K

V

L

V

A

H

E

S

C

T

D

D

L

L

V

V

I

V

E

E

A

A

I

I

A

V

E

E

R

N

M

L

D

K

W

V

K

K

H

N

D

E

L

L

Y

F

K

K

K

R

V

L

S

D

P

K

H

F

I

A

A

A

P

E

N

H

A

T

I

I

F

F

A

A

T

S

N

N

T

T

S
|
S

G

S

L

L

S

Q

I

I

T

T

E

S

L

I

S

A

Q

N

G

-

F

-

A

A

D

T

E

T

L

R

K

Q

A

D

R

R

F

F

C

A

G

G

V

L

H
|
H

F

F

F
|
F

N
|
N

P
|
P

P

V

R

P

Y
x
V

M
|
M

H

K

L

L

V

V

E
|
E

L

V

I

I

P

K

T

T

A

P

T

M

T

T

R

S

P

Q

E

K

I

T

L

F

D

E

Q

S

L

L

E

V

T

D

F

F

L

-

T

S

S

K

V

A

V

L

G

G

K

K

G

H

V

P

V

V

R

S

A

C

K

K

D

D

T

T

P

P

N

G

F

F

I

I

A

V

N
|
N

R

R

V

L

G
x
L

I

V

F

P

S

Y

I

L

L

M

A

E

V

A

I

I

T

R

E

L

A

Y

A

E

K

R

F

G

G

D

L

A

R

S

F

K

D

E

E

D

V

I

D

D

D

-

L

-

T

T

G

A

S

M

R

K

L

L

G

G

R

A

A

G

K

Y

S
x
P

-
x
M

A

G

T

P

F

F

R

E

T
x
L

A

L

D

D

V

Y

V

V

G

G

L

L

D

D

T

T

M

T

A

K

H

F

V

I

I

V

K

D

T

G

M

W

Q

H

D

E

N

M

L

-

K

-

D

-

D

D

P

A

F

E

F

N

P

P

V

L

Y

H

E

Q

T

P

P

S

A

P

V

S

L

L

A

N

E

K

L

L

V

V

K

A

K

E

G

N

A

K

L

F

G

G

Q

K

K

K

T

T

G

G

G

E

G

G

F

F

Y

Y

R

K

active site: S126 (= S123), H147 (= H146), E159 (= E158), N197 (= N197)
binding 3-hydroxybutanoyl-coenzyme a: S50 (≠ N54), K57 (≠ A61), S126 (= S123), H147 (= H146), F149 (= F148), N150 (= N149), P151 (= P150), V154 (≠ Y153), M155 (= M154), N197 (= N197), L200 (≠ G200), P232 (≠ S234), M233 (vs. gap), L238 (≠ T239)

P9WNP7 3-hydroxybutyryl-CoA dehydrogenase; Beta-hydroxybutyryl-CoA dehydrogenase; BHBD; EC 1.1.1.157 from Mycobacterium tuberculosis (strain ATCC 25618 / H37Rv) (see paper)
31% identity, 35% coverage: 6:292/811 of query aligns to 5:284/286 of P9WNP7

query
sites
P9WNP7

I

I

R

Q

K

R

V

V

A

G

V

V

L

V

G

G

A

A

G

G

V

Q

M

M

G

G

A

S

Q

G

I

I

A

A

A

E

H

V

L

S

I

A

N

R

A

A

K

G

V

V

P

E

V

V

L

T

L

V

F

F

D

E

L

-

P

P

A

A

K

E

-

A

-

L

-

I

E

T

G

A

P

G

K

R

N

N

A

R

I

I

A

-

L

V

K

K

A

S

I

L

E

E

N

-

L

-

K

R

K

A

L

V

S

S

P

A

A

G

P

K

F

V

G

T

V

E

K

R

D

E

D

R

A

D

Q

R

Y

A

I

L

Q

G

P

L

A

L

N

T

Y

F

D

T

D

D

I

L

E

N

K

D

L

L

A

S

E

D

C

R

D

Q

L

L

V

V

I

I

E

E

A

A

I

V

A

V

E

E

R

D

M

E

D

A

W

V

K

K

H

S

D

E

L

I

Y

F

K

A

K

E

V

L

S

D

P

R

H

V

I

V

A

T

-

D

P

P

N

D

A

A

I

V

F

L

A

A

T

S

N

N

T

T

S
|
S

G

S

L

I

S

P

I

I

T

M

E

K

L

V

S

A

Q

A

G

-

F

-

A

A

D

T

E

K

L

Q

K

P

A

Q

R

R

F

V

C

L

G

G

V

L

H

H

F

F

N

N

P

P

P

V

R

P

Y

V

M

L

H

P

L

L

V

V

E

E

L

L

I

V

P

R

T

T

A

L

T

V

T

T

R

D

P

E

E

A

I

A

L

A

D

A

Q

R

L

T

E

E

T

E

F

F

L

A

T

S

S

T

V

V

V

L

G

G

K

K

G

Q

V

V

R

R

A

C

K

S

D

D

T

R

P

S

N

G

F

F

I

V

A

V

N

N

R

A

V

L

G

L

I

V

F

P

S

Y

I

L

L

L

A

S

V

A

I

I

T

R

E

M

A

V

A

E

K

A

F

G

G

F

L

A

R

T

F

V

D

E

E

D

V

V

D

D

D

K

L

A

T

V

G

V

S

A

R

G

L

L

G

S

R

H

A

P

K

M

S

-

A

G

T

P

F

L

R

R

T

L

A

S

D

D

V

L

V

V

G

G

L

L

D

D

T

T

M

L

A

K

H

L

V

I

I

A

K

D

T

K

M

M

Q

F

D

E

N

E

L

F

K

K

D

E

D

-

P

-

F

-

P

P

V

H

Y

Y

E

G

T

P

P

P

A

P

V

L

L

L

A

L

E

R

L

M

V

V

K

E

K

A

G

G

A

Q

L

L

G

G

Q

K

K

K

T

S

G

G

G

R

G

G

F

F

Y

Y

S122 (= S123) mutation to A: Loss of fatty acyl dehydrogenase activity.

4kuhA Crystal structure of 3-hydroxybutylryl-coa dehydrogenase with acetoacetyl-coa from clostridium butyricum
30% identity, 35% coverage: 6:292/811 of query aligns to 1:278/280 of 4kuhA

query
sites
4kuhA

I

M

R

K

K

K

V

V

A

F

V

V

L

L

G

G

A

A

G

G

V

T

M

M

G

G

A

A

Q

G

I

I

A

V

A

Q

H

A

L

F

I

A

N

A

A

K

K

G

V

C

P

E

V

V

L

I

L

V

F

R

D

D

L

I

P

K

A

-

K

E

E

E

G

F

P

V

K

D

N

R

A

G

I

I

A

A

L

T

K

I

A

T

I

K

E

S

N

L

L

S

K
|
K

K
x
L

L

V

S

A

P
x
K

A

E

P
x
K

F

I

G

T

V

E

K

A

D

D

D

K

A

E

Q

E

Y

I

I

L

Q

S

P

R

A

I

N

S

Y

G

D

T

D

D

I

M

E

K

K

L

L

A

A

A

E

D

C

C

D

D

L

L

V

V

I

V

E

E

A

A

I

A

A

I

E

E

R

N

M

M

D

K

W

I

K

K

H

K

D

E

L

I

Y

F

K

A

K

E

V

L

S

D

P

G

H

I

I

C

A

K

P

P

N

E

A

T

I

I

F

L

A

A

T

S

N

N

T

T

S
|
S

G

S

L

L

S

S

I

I

T

T

E

E

L

V

S

A

Q

S

G

-

F

-

A

A

D

T

E

K

L

R

K

A

A

D

R

K

F

V

C

I

G

G

V

M

H
|
H

F

F

F
|
F

N
|
N

P

P

P

A

R

P

Y
x
V

M

M

H

K

L

L

V

V

E
|
E

L

V

I

I

P

R

T

G

A

A

T

A

T

T

R

S

P

Q

E

E

I

T

L

F

D

D

Q

A

L

V

E

K

T

E

F

-

L

M

T

S

S

E

V

S

V

I

G

G

K

K

G

T

V

P

V

V

R

E

A

V

K

A

D

E

T

A

P

P

N

G

F

F

I

V

A

V

N
|
N

R

R

V

I

G

-

I

-

F

-

S

-

I

L

L

I

A

P

V

M

I

I

T

N

E

E

A

A

A

T

-

F

-

I

-

L

K

Q

F

E

G

G

L

V

R

A

F

K

D

E

E

E

V

D

D

I

D

D

L

A

T

A

G

M

S

K

R

L

L

G

G

A

R

N

A

H

K
x
P

S
x
M

A

G

T

P

F

L

R

A

T

L

A

G

D

D

V

L

V
x
I

G

G

L

L

D

D

T

V

M

C

A

L

H

A

V

I

I

M

K

D

T

V

M

L

Q

Y

D

N

N

E

L

T

K

G

D

D

D

T

P

K

F

-

P

-

V

-

Y

Y

E

R

T

A

P

S

A

S

V

L

L

L

A

R

E

K

L

Y

V

V

K

R

K

A

G

G

A

W

L

L

G

G

Q

R

K

K

T

T

G

G

G

K

G

G

F

F

Y

Y

active site: S117 (= S123), H138 (= H146), E150 (= E158), N188 (= N197)
binding acetoacetyl-coenzyme a: K50 (= K56), L51 (≠ K57), K54 (≠ P60), K56 (≠ P62), S117 (= S123), H138 (= H146), F140 (= F148), N141 (= N149), V145 (≠ Y153), N188 (= N197), P223 (≠ K233), M224 (≠ S234), I233 (≠ V243)

4kugA Crystal structure of 3-hydroxybutylryl-coa dehydrogenase with NAD from clostridium butyricum
30% identity, 35% coverage: 6:292/811 of query aligns to 1:278/282 of 4kugA

query
sites
4kugA

I

M

R

K

K

K

V

V

A

F

V

V

L
|
L

G
|
G

A

A

G
|
G

V
x
T

M
|
M

G

G

A

A

Q

G

I

I

A

V

A

Q

H

A

L

F

I

A

N

A

A

K

K

G

V

C

P

E

V

V

L

I

L

V

F
x
R

D
|
D

L
x
I

P

K

A

-

K

E

E

E

G

F

P

V

K

D

N

R

A

G

I

I

A

A

L

T

K

I

A

T

I

K

E

S

N

L

L

S

K

K

K

L

L

V

S

A

P

K

A

E

P

K

F

I

G

T

V

E

K

A

D

D

D

K

A

E

Q

E

Y

I

I

L

Q

S

P

R

A

I

N

S

Y

G

D

T

D

D

I

M

E

K

K

L

L

A

A

A

E

D

C

C

D

D

L

L

V

V

I

V

E

E

A
|
A

I
x
A

A
x
I

E
|
E

R

N

M

M

D

K

W

I

K
|
K

H

K

D

E

L
x
I

Y

F

K

A

K

E

V

L

S

D

P

G

H

I

I

C

A

K

P

P

N

E

A

T

I

I

F

L

A

A

T

S

N
|
N

T
|
T

S
|
S

G

S

L

L

S

S

I

I

T

T

E

E

L

V

S

A

Q

S

G

-

F

-

A

A

D

T

E

K

L

R

K

A

A

D

R

K

F

V

C

I

G

G

V

M

H
|
H

F
|
F

F

F

N
|
N

P

P

P

A

R

P

Y

V

M

M

H

K

L

L

V

V

E
|
E

L

V

I

I

P

R

T

G

A

A

T

A

T

T

R

S

P

Q

E

E

I

T

L

F

D

D

Q

A

L

V

E

K

T

E

F

-

L

M

T

S

S

E

V

S

V

I

G

G

K

K

G

T

V

P

V

V

R

E

A

V

K

A

D

E

T

A

P

P

N

G

F

F

I

V

A

V

N
|
N

R

R

V

I

G

-

I

-

F

-

S

-

I

L

L

I

A

P

V

M

I

I

T

N

E

E

A

A

A

T

-

F

-

I

-

L

K

Q

F

E

G

G

L

V

R

A

F

K

D

E

E

E

V

D

D

I

D

D

L

A

T

A

G

M

S

K

R

L

L

G

G

A

R

N

A

H

K

P

S

M

A

G

T

P

F

L

R

A

T

L

A

G

D

D

V

L

V

I

G

G

L

L

D

D

T

V

M

C

A

L

H

A

V

I

I

M

K

D

T

V

M

L

Q

Y

D

N

N

E

L

T

K

G

D

D

D

T

P

K

F

-

P

-

V

-

Y

Y

E

R

T

A

P

S

A

S

V

L

L

L

A

R

E

K

L

Y

V

V

K

R

K

A

G

G

A

W

L

L

G

G

Q

R

K

K

T

T

G

G

G

K

G

G

F

F

Y

Y

active site: S117 (= S123), H138 (= H146), E150 (= E158), N188 (= N197)
binding nicotinamide-adenine-dinucleotide: L7 (= L12), G8 (= G13), G10 (= G15), T11 (≠ V16), M12 (= M17), R30 (≠ F35), D31 (= D36), I32 (≠ L37), A87 (= A93), A88 (≠ I94), I89 (≠ A95), E90 (= E96), K95 (= K101), I98 (≠ L104), N115 (= N121), T116 (= T122), S117 (= S123), H138 (= H146), F139 (= F147), N141 (= N149)

4pzeA Crystal structure of (s)-3-hydroxybutyryl-coa dehydrogenase paah1 in complex with acetoacetyl-coa (see paper)
33% identity, 35% coverage: 6:292/811 of query aligns to 2:279/283 of 4pzeA

query
sites
4pzeA

I

I

R

R

K

T

V

V

A

G

V

I

L

V

G

G

A

A

G

G

V

T

M

M

G

G

A

N

Q

G

I

I

A

A

A

Q

H

A

L

C

I

A

N

V

A

V

K

G

V

L

P

N

V

V

L

V

L

M

F

V

D

D

L

I

P

S

A

D

K

A

E

A

G

V

P

Q

K

K

N

G

-

V

-

A

-

T

-

V

A

A

I

S

A

S

L

L

K

D

A

R

I

L

E

I

N

K

L

K

K

E

K

K

L

L

S

T

P

E

A

A

P

-

F

-

G

-

V

-

K

-

D

D

D

K

A

A

Q

S

Y

A

I

L

Q

A

-

R

-

I

-

K

-

G

P

S

A

T

N

S

Y

Y

D

D

D

-

I

-

E

-

K

-

L

L

A

K

E

A

C

T

D

D

L

I

V

V

I

I

E

E

A

A

I

A

A

T

E

E

R

N

M

Y

D

D

W

L

K

K

H

V

D

K

L

I

Y

L

K

K

K

Q

V

I

S

D

P

G

H

I

I

V

A

G

P

E

N

N

A

V

I

I

F

I

A

A

T

S

N

N

T

T

S
|
S

G

S

L

I

S

S

I

I

T

T

E

K

L

L

S

A

-

A

-

V

Q

T

G

S

F

R

A

A

D

D

E

-

L

-

K

-

A

-

R

R

F

F

C

I

G

G

V

M

H
|
H

F

F

F
|
F

N

N

P

P

P

V

R

P

Y

V

M

M

H

A

L

L

V

V

E
|
E

L

L

I

I

P

R

T

G

A

L

T

Q

T

T

R

S

P

D

E

T

I

T

L

H

D

A

Q

A

L

V

E

E

T

A

F

-

L

L

T

S

S

K

V

Q

V

L

G

G

K

K

G

Y

V

P

V

I

R

T

A

V

K

K

D

N

T

S

P

P

N

G

F

F

I

V

A

V

N
|
N

R

R

V

I

G

-

I

-

F

-

S

-

I

L

L

C

A

P

V

M

I

I

T

N

E

E

A

A

A

F

K

C

F

V

-

L

-

G

-

E

G

G

L

L

R

A

F

S

-

P

D

E

E

E

V

I

D

D

D

E

L

-

T

-

G

G

S

M

R

K

L

L

G

G

R

C

A

N

K

H

S
x
P

-
x
I

A

G

T

P

F

L

R

A

T
x
L

A

A

D

D

V

M

V
x
I

G

G

L

L

D

D

T
|
T

M

M

A

L

H

A

V

V

I

M

K

E

T

V

M

L

Q

Y

D

T

N

E

L

F

K

A

D

D

D

-

P

-

F

-

P

P

V

K

Y

Y

E

R

T

P

P

A

A

M

V

L

L

M

A

R

E

E

L

M

V

V

K

A

G

G

A

Y

L

L

G

G

Q

R

K

K

T

T

G

G

G

R

G

G

F

V

Y

Y

active site: S118 (= S123), H139 (= H146), E151 (= E158), N189 (= N197)
binding acetoacetyl-coenzyme a: S118 (= S123), F141 (= F148), N189 (= N197), P224 (≠ S234), I225 (vs. gap), L230 (≠ T239), I234 (≠ V243), T238 (= T247)

4pzdA Crystal structure of (s)-3-hydroxybutyryl-coa dehydrogenase paah1 in complex with NAD+ (see paper)
33% identity, 35% coverage: 6:292/811 of query aligns to 2:279/283 of 4pzdA

query
sites
4pzdA

I

I

R

R

K

T

V

V

A

G

V

I

L
x
V

G
|
G

A
|
A

G
|
G

V
x
T

M
|
M

G

G

A

N

Q

G

I

I

A

A

A

Q

H

A

L

C

I

A

N

V

A

V

K

G

V

L

P

N

V

V

L

V

L

M

F

V

D
|
D

L
x
I

P

S

A

D

K

A

E
x
A

G

V

P

Q

K

K

N

G

-

V

-

A

-

T

-

V

A

A

I

S

A

S

L

L

K

D

A

R

I

L

E

I

N

K

L

K

K

E

K

K

L

L

S

T

P

E

A

A

P

-

F

-

G

-

V

-

K

-

D

D

D

K

A

A

Q

S

Y

A

I

L

Q

A

-

R

-

I

-

K

-

G

P

S

A

T

N

S

Y

Y

D

D

D

-

I

-

E

-

K

-

L

L

A

K

E

A

C

T

D

D

L

I

V

V

I

I

E

E

A
|
A

I
x
A

A
x
T

E
|
E

R

N

M

Y

D

D

W

L

K

K

H

V

D

K

L
x
I

Y

L

K

K

K

Q

V

I

S

D

P

G

H

I

I

V

A

G

P

E

N

N

A

V

I

I

F

I

A

A

T

S

N
|
N

T

T

S
|
S

G

S

L

I

S

S

I

I

T

T

E

K

L

L

S

A

-

A

-

V

Q

T

G

S

F

R

A

A

D

D

E

-

L

-

K

-

A

-

R

R

F

F

C

I

G

G

V

M

H
|
H

F

F

N
|
N

P

P

P

V

R

P

Y

V

M

M

H

A

L

L

V

V

E
|
E

L

L

I

I

P

R

T

G

A

L

T

Q

T

T

R

S

P

D

E

T

I

T

L

H

D

A

Q

A

L

V

E

E

T

A

F

-

L

L

T

S

S

K

V

Q

V

L

G

G

K

K

G

Y

V

P

V

I

R

T

A

V

K

K

D

N

T

S

P

P

N

G

F

F

I

V

A

V

N
|
N

R

R

V

I

G

-

I

-

F

-

S

-

I

L

L

C

A

P

V

M

I

I

T

N

E

E

A

A

A

F

K

C

F

V

-

L

-

G

-

E

G

G

L

L

R

A

F

S

-

P

D

E

E

E

V

I

D

D

D

E

L

-

T

-

G

G

S

M

R

K

L

L

G

G

R

C

A

N

K

H

S

P

-

I

A

G

T

P

F

L

R

A

T

L

A

A

D

D

V

M

V

I

G

G

L

L

D

D

T

T

M

M

A

L

H

A

V

V

I

M

K

E

T

V

M

L

Q

Y

D

T

N

E

L

F

K

A

D

D

D

-

P

-

F

-

P

P

V

K

Y

Y

E

R

T

P

P

A

A

M

V

L

L

M

A

R

E

E

L

M

V

V

K

A

G

G

A

Y

L

L

G

G

Q

R

K

K

T

T

G

G

G

R

G

G

F

V

Y

Y

active site: S118 (= S123), H139 (= H146), E151 (= E158), N189 (= N197)
binding nicotinamide-adenine-dinucleotide: V8 (≠ L12), G9 (= G13), A10 (= A14), G11 (= G15), T12 (≠ V16), M13 (= M17), D32 (= D36), I33 (≠ L37), A37 (≠ E41), A88 (= A93), A89 (≠ I94), T90 (≠ A95), E91 (= E96), I99 (≠ L104), N116 (= N121), S118 (= S123), H139 (= H146), N142 (= N149)

6aa8E Crystal structure of (s)-3-hydroxybutyryl-coenzymea dehydrogenase from clostridium acetobutylicum complexed with NAD+ (see paper)
31% identity, 35% coverage: 7:292/811 of query aligns to 1:277/281 of 6aa8E

query
sites
6aa8E

R

K

K

K

V

V

A

C

V

V

L
x
I

G
|
G

A

A

G
|
G

V
x
T

M
|
M

G

G

A

S

Q

G

I

I

A

A

A

Q

H

A

L

F

I

A

N

A

A

K

K

G

V

F

P

E

V

V

L

V

L

L

F
x
R

D
|
D

L
x
I

P

-

A

-

K

-

E

-

G

-

P

-

K

K

N

D

A

E

I
x
F

A

V

L

D

K
x
R

A

G

I

L

E

D

-

F

-

I

-

N

-

K

N

N

L

L

K

S

K

K

L

L

S

V

P

K

A

K

P

-

F

-

G

G

V

K

K

I

D

E

A

A

Q

T

Y

K

I

V

Q

E

P

I

A

L

N

T

Y

R

D

I

D

S

-

G

-

T

-

V

D

D

I

L

E

N

K

M

L

A

A

A

E

D

C

C

D

D

L

L

V

V

I

I

E

E

A
|
A

I
x
A

A

V

E
|
E

R

R

M

M

D

D

W

I

K
|
K

H

K

D

Q

L
x
I

Y

F

K

A

K

D

V

L

S

D

P

N

H

I

I

C

A

K

P

P

N

E

A

T

I

I

F

L

A

A

T

S

N
|
N

T
|
T

S
|
S

G

S

L

L

S

S

I

I

T

T

E

E

L

V

S

A

Q

S

G

-

F

-

A

A

D

T

E

K

L

R

K

P

A

D

R

K

F

V

C

I

G

G

V

M

H
|
H

F

F

N
|
N

P

P

P

A

R

P

Y

V

M

M

H

K

L

L

V

V

E
|
E

L

V

I

I

P

R

T

G

A

I

T

A

T

T

R

S

P

Q

E

E

I

T

L

F

D

D

Q

A

L

V

E

K

T

E

F

-

L

-

T

T

S

S

V

I

-

A

V

I

G

G

K

K

G

D

V

P

V

V

R

E

A

V

K

A

D

E

T

A

P

P

N

G

F

F

I

V

A

V

N
|
N

R

R

V

I

G

L

I

I

F

P

S

M

I

I

L

N

A

E

V

A

I

V

T

G

E

I

A

L

A

A

K

E

F

G

G

I

L

A

R

S

F

V

D

E

E

D

V

I

D

D

D

-

L

-

T

K

G

A

S

M

R

K

L

L

G

G

-

A

R

N

A

H

K

P

S

M

A

G

T

P

F

L

R

E

T

L

A

G

D

D

V

F

V

I

G

G

L

L

D

D

T

I

M

C

A

L

H

A

V

I

I

M

K

D

T

V

M

L

Q

Y

D

S

N

E

L

T

K

G

D

D

D

S

P

K

F

Y

F

R

P

P

V

-

Y

-

E

-

T

-

P

H

A

T

V

L

L

L

A

K

E

K

L

Y

V

V

K

R

K

A

G

G

A

W

L

L

G

G

Q

R

K

K

T

S

G

G

G

K

G

G

F

F

Y

Y

active site: S116 (= S123), H137 (= H146), E149 (= E158), N187 (= N197)
binding nicotinamide-adenine-dinucleotide: I6 (≠ L12), G7 (= G13), G9 (= G15), T10 (≠ V16), M11 (= M17), R29 (≠ F35), D30 (= D36), I31 (≠ L37), F35 (≠ I47), R38 (≠ K50), A86 (= A93), A87 (≠ I94), E89 (= E96), K94 (= K101), I97 (≠ L104), N114 (= N121), T115 (= T122), S116 (= S123), H137 (= H146), N140 (= N149)

Q08426 Peroxisomal bifunctional enzyme; PBE; PBFE; L-bifunctional protein; LBP; Multifunctional enzyme 1; MFE1; EC 4.2.1.17; EC 5.3.3.8; EC 1.1.1.35 from Homo sapiens (Human) (see 5 papers)
25% identity, 49% coverage: 6:403/811 of query aligns to 297:681/723 of Q08426

query
sites
Q08426

I

V

R

S

K

S

V

V

A

G

V

V

L

V

G

G

A

L

G

G

V

T

M

M

G

G

A

R

Q

G

I

I

A

V

A

I

H

S

L

F

I

A

N

R

A

A

K

R

V

I

P

P

V

V

L

I

L
x
A

F

V

D

D

L

-

P

-

A

-

K

S

E

D

G

K

P

N

K

Q

N

L

A

A

I

T

A

A

L

N

K

K

A

M

I

I

E

T

N

S

L

V

K

-

L

-

S

-

P

-

A

-

P

-

F

-

G

-

V

L

K

E

D
x
K

D

E

A

A

Q

S

Y

K

I

M

Q

Q

P

Q

A

S

N

G

Y

H

-

P

-

W

-

S

-

G

-

P

-

K

-

P

-

R

-

L

D

T

D

S

I

V

E

K

K

E

L

L

A

G

E

G

C

V

D

D

L

L

V

V

I

I

E

E

A

A

I

V

A

F

E

E

R

E

M

M

D

S

W

L

K

K

H

K

D

Q

L

V

Y

F

K

A

K

E

V

L

S

S

P

A

H

V

I

C

A

K

P

P

N

E

A

A

I

F

F

L

A

C

T

T

N

N

T

T

S

S

G

A

L

L

S

D

I

V

T

D

E

E

L

I

S

A

Q

S

G

-

F

-

A

S

D

T

E

D

L

R

K

P

A

H

R

L

F

V

C

I

G

G

V

T

H

H

F

F

N

S

P

P

P

A

R

H

Y

V

M

M

H

K

L

L

V

L

E

E

L

V

I

I

P

P

T

S

A

Q

T

Y

T

S

R

S

P

P

E

T

I

T

L

I

D

A

Q

T

L

V

E

M

T

N

F

L

L

S

T

K

S

K

V

I

V

K

G

K

K

I

G

G

V

V

R

-

A

V

K

G

D

N

T

C

P

F

N

G

F

F

I

V

A

G

N

N

R

R

V

M

-

L

-

N

G

P

I

Y

F

Y

S

N

I

Q

L

A

A

Y

V

F

I

L

T

L

E

E

A

E

A

G

K

S

F

K

G

P

L

E

R

E

F

V

D

D

E

Q

V

V

D

L

D

E

L

E

T

F

G

G

S

F

R

K

L

M

G

G

R

-

A

-

K

-

S

-

A

-

T

P

F

F

R

R

T

V

A

S

D

D

V

L

V

A

G

G

L

L

D

D

T

V

M

G

A

W

H

K

V

S

I

R

K

K

-

G

-

Q

-

G

-

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x
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x
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N

P

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R

-

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-

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Q

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A325 (≠ L34) to G: in dbSNP:rs1062555
K346 (≠ D67) modified: N6-acetyllysine; mutation to Q: Greatly reduced acetylation and insensitive to treatment with TSA and NAM; when associated with Q-165; Q-171 and Q-584.
K584 (≠ E318) modified: N6-acetyllysine; alternate; mutation to Q: Greatly reduced acetylation and insensitive to treatment with TSA and NAM; when associated with Q-165; Q-171 and Q-346.
K598 (≠ E330) to T: in dbSNP:rs1042437
T606 (≠ S338) to P: in dbSNP:rs1042438

Sites not aligning to the query:

3 E → K: in FRTS3; the mutant is mistargeted to mitochondria; results in impaired mitochondrial oxidative phosphorylation and defects in the transport of fluids across the epithelium of renal proximal tubular cells; dbSNP:rs398124646
40 V → G: in dbSNP:rs1062551
41 I → R: in dbSNP:rs1062552
75 T → I: in dbSNP:rs1062553
165 modified: N6-acetyllysine; alternate; K→Q: Greatly reduced acetylation and insensitive to treatment with TSA and NAM; when associated with Q-171; Q-346 and Q-584.
171 modified: N6-acetyllysine; K→Q: Greatly reduced acetylation and insensitive to treatment with TSA and NAM; when associated with Q-165; Q-346 and Q-584.
274 A → T: in dbSNP:rs2302819

P40939 Trifunctional enzyme subunit alpha, mitochondrial; 78 kDa gastrin-binding protein; Monolysocardiolipin acyltransferase; TP-alpha; EC 2.3.1.-; EC 4.2.1.17; EC 1.1.1.211 from Homo sapiens (Human) (see 5 papers)
26% identity, 51% coverage: 6:416/811 of query aligns to 361:749/763 of P40939

query
sites
P40939

I

V

R

K

K

H

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A

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A

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E510 (= E158) active site, For hydroxyacyl-coenzyme A dehydrogenase activity; to Q: in AFLP and LCHAD deficiency; loss of long-chain-3-hydroxyacyl-CoA dehydrogenase activity; dbSNP:rs137852769

Sites not aligning to the query:

282 V → D: in MTPD; mild phenotype with slowly progressive myopathy and sensorimotor polyneuropathy; dbSNP:rs137852773
305 I → N: in MTPD; mild phenotype with slowly progressive myopathy and sensorimotor polyneuropathy; dbSNP:rs137852774
342 L → P: in LCHAD deficiency; dbSNP:rs137852772

4b3iA Crystal structure of mycobacterium tuberculosis fatty acid beta-oxidation complex with coenzymea bound at the hydratase active sites (see paper)
29% identity, 35% coverage: 6:292/811 of query aligns to 336:616/731 of 4b3iA

query
sites
4b3iA

I

I

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S

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P

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H

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E

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D

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N

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x
S

R

R

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V

-

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F

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-

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S

E

I

A

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