SitesBLAST

K318 (= K265) modified: N6-acetyllysine; mutation to R: Reduces activity by 37%; reduces activity by 80% when associated with R-322.
K322 (≠ V269) modified: N6-acetyllysine; alternate; mutation to R: Reduces activity by 23%; reduces activity by 80% when associated with R-318.

Sites not aligning to the query:

42 modified: N6-acetyllysine; K→R: Reduces activity by 90% when associated with R-318 and R-322.

P45954 Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; SBCAD; 2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenase; EC 1.3.8.5 from Homo sapiens (Human) (see 6 papers)
29% identity, 92% coverage: 6:349/375 of query aligns to 58:403/432 of P45954

query
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P45954

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V137 (= V84) mutation to L: Decreased acyl-CoA dehydrogenase activity.
F138 (≠ E85) mutation to L: Increased acyl-CoA dehydrogenase activity. No effect on substrate specificity.
174:183 (vs. 121:130, 10% identical) binding in other chain
S183 (≠ Y130) binding
WIS 207:209 (≠ VVQ 154:156) binding in other chain
S210 (≠ H157) mutation to N: Increased acyl-CoA dehydrogenase activity. Changed substrate specificity.
Y229 (≠ D177) binding
L255 (≠ G203) to F: in SBCADD; loss of protein expression; loss of 2-methylbutyryl-CoA dehydrogenase activity; dbSNP:rs137852649
Y283 (≠ F228) binding
NEGR 291:294 (≠ --GV 238:239) binding
I316 (≠ L261) to V: in dbSNP:rs1131430
R319 (= R264) binding
Q330 (= Q275) binding
EWMGG 387:391 (≠ QLHGG 333:337) binding

Sites not aligning to the query:

1:33 modified: transit peptide, Mitochondrion
13 R → K: in dbSNP:rs12263012
416 A→T: Increased acyl-CoA dehydrogenase activity. No effect on substrate specificity.
416:418 binding in other chain

2jifA Structure of human short-branched chain acyl-coa dehydrogenase (acadsb)
29% identity, 92% coverage: 6:349/375 of query aligns to 7:352/381 of 2jifA

query
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2jifA

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active site: L125 (≠ H123), S126 (≠ G124), G242 (= G238)
binding coenzyme a persulfide: S132 (≠ Y130), S134 (≠ L132), F135 (≠ S133), Y178 (≠ D177), Y232 (≠ F228), I236 (= I235), L239 (vs. gap), N240 (vs. gap), R243 (≠ V239), I313 (≠ R309)
binding flavin-adenine dinucleotide: F123 (≠ L121), C124 (≠ A122), L125 (≠ H123), S126 (≠ G124), G131 (≠ H129), S132 (≠ Y130), W156 (≠ V154), I157 (≠ V155), S158 (≠ Q156), K201 (≠ I200), T209 (vs. gap), R268 (= R264), Q270 (= Q266), F271 (= F267), L275 (≠ I271), F278 (= F274), L281 (= L277), E336 (≠ Q333), W337 (≠ L334), G339 (= G336), G340 (= G337)

Sites not aligning to the query:

active site: 363, 375
binding coenzyme a persulfide: 362, 363, 364, 368
binding flavin-adenine dinucleotide: 358, 361, 362, 365, 367, 368, 371

P28330 Long-chain specific acyl-CoA dehydrogenase, mitochondrial; LCAD; EC 1.3.8.8 from Homo sapiens (Human) (see 2 papers)
30% identity, 82% coverage: 40:347/375 of query aligns to 86:399/430 of P28330

query
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P28330

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E291 (≠ G238) active site, Proton acceptor; mutation to Q: Loss of long-chain-acyl-CoA dehydrogenase activity. No effect on protein abundance. No effect on solubility. No effect on substrate binding.
S303 (≠ M250) to T: in dbSNP:rs1801204
K333 (≠ R280) to Q: in dbSNP:rs2286963

1ivhA Structure of human isovaleryl-coa dehydrogenase at 2.6 angstroms resolution: structural basis for substrate specificity (see paper)
28% identity, 92% coverage: 6:351/375 of query aligns to 9:361/387 of 1ivhA

query
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1ivhA

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I

I

D

S

G

G

S

E

R

Y

I

I

V

G

A

A

L
|
L

A
|
A

H
x
M

G
x
S

E

E

P

P

D

N

S

A

H
x
G

Y
x
S

E

D

L
x
V

S
x
V

R

S

V

M

S

K

A

L

R

K

A

A

E

E

R

K

S

K

G

G

S

N

T

H

W

Y

Q

I

L

L

N

N

G

G

A

N

K

K

A

F

V
x
W

V
x
I

Q
x
T

H

N

G

G

E

P

H

D

A

A

S

D

L

V

F

L

L

I

V

V

S

Y

A

A

R

K

T

T

G

D

-

L

G

A

D

A

D

V

D

P

S

A

D
x
S

A
x
R

G

G

I

I

T

T

L

A

F

F

L

I

V

V

P

E

R

K

D

G

A

M

A

P

G

G

V

F

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V

T

R

S

G

-

Y

-

R

K

K

K

I

L

D

D

-

K

-

L

-

G

-

M

-

R

G

G

G

S

R

N

A
x
T

A

C

E

E

V

L

T

I

L

F

D

E

N

D

V

C

T

K

L

I

S

P

D

A

A

N

L

I

L

L

G

G

T

H

P

E

D

N

A

K

G

G

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x
V

A
x
Y

T

V

L

L

E
x
M

Y

S

A

G

I
x
L

G
x
D

C

L

G
x
E

V
x
R

F

L

A

V

L

L

C

A

S

G

E

G

A

P

V

L

G

G

A

L

M

M

D

Q

V

A

A

V

K

L

D

D

Y

H

T

T

L

I

E

P

Y

Y

L

L

R

H

T

V

R
|
R

K

E

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x
A

F
|
F

G

G

V

Q

P

K

I
|
I

G

G

S

H

F
|
F

Q

Q

A
x
L

L
x
M

Q

Q

H

G

R

K

M

M

A

A

D

D

L

M

L

Y

L

T

E

R

I

L

E

M

Q

A

A

C

R

R

S

Q

A

Y

V

V

I

Y

N

N

A

V

A

A

A

K

A

A

L

C

G

D

A

E

E

G

R

H

K

C

V

T

R

A

E

K

R

D

A

C

V

A

S

G

A

V

A

I

K

L

Y

Y

S

S

I

-

G

A

R

E

I

C

G

A

A

T

L

Q

V

V

A

A

E

L

E

D

C

G

I

I

Q
|
Q

L
x
C

H

F

G
|
G

I

N

G

G

M

Y

T

I

W

N

E

D

L

F

P

P

L

M

A

G

H

R

Y

F

A

L

K

R

active site: M130 (≠ H123), S131 (≠ G124), E249 (≠ G238)
binding coenzyme a persulfide: M130 (≠ H123), G136 (≠ H129), S137 (≠ Y130), V139 (≠ L132), V140 (≠ S133), S185 (≠ D177), R186 (≠ A178), V239 (≠ F228), Y240 (≠ A229), M243 (≠ E232), L246 (≠ I235), D247 (≠ G236), E249 (≠ G238), R250 (≠ V239)
binding flavin-adenine dinucleotide: L128 (= L121), A129 (= A122), M130 (≠ H123), S131 (≠ G124), G136 (≠ H129), S137 (≠ Y130), W161 (≠ V154), I162 (≠ V155), T163 (≠ Q156), T216 (≠ A205), R275 (= R264), A277 (≠ Q266), F278 (= F267), I282 (= I271), F285 (= F274), L287 (≠ A276), M288 (≠ L277), Q343 (= Q333), C344 (≠ L334), G346 (= G336), G347 (= G337)

Sites not aligning to the query:

active site: 370, 382
binding coenzyme a persulfide: 369, 370, 371, 375, 382
binding flavin-adenine dinucleotide: 365, 368, 372, 374, 378

P26440 Isovaleryl-CoA dehydrogenase, mitochondrial; IVD; Butyryl-CoA dehydrogenase; EC 1.3.8.4; EC 1.3.8.1 from Homo sapiens (Human) (see 5 papers)
28% identity, 92% coverage: 6:351/375 of query aligns to 46:398/426 of P26440

query
sites
P26440

T

S

E

E

D

E

R

Q

R

R

M

Q

L

L

A

R

D

Q

T

T

L

M

N

A

R

K

F

F

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L

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Q

E

E

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L

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A

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T

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D

E

R

I

I

D

A

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Q

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S

N

D

E

Q

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G

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Y

N

S

L

G

R

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E

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F

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W

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R

Q

F

L

A

G

E

N

L

L

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I

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G

G

A

I

L

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F

A

D

P

E

V

A

Q

D

Y

G

G

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S

G

G

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L

G

G

G

Y

F

L

D

E

I

H

A

V

V

L

V

V

F

M

E

E

S

E

L

I

G

S

R

R

-

A

-

S

-

G

-

A

-

V

G

G

L

L

V

S

V

Y

E

G

P

A

F

H

L

S

D

N

T

L

L

C

I

I

V

-

G

-

R

N

A

Q

I

L

A

V

R

R

S

N

G

G

N

N

E

E

A

A

Q

Q

K

K

-

E

A

K

V

Y

L

L

G

P

E

K

L

L

I

I

D

S

G

G

S

E

R

Y

I

I

V

G

A

A

L
|
L

A
|
A

H
x
M

G
x
S

E
|
E

P
|
P

D
x
N

S
x
A

H
x
G

Y
x
S

E

D

L

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S

V

R

S

V

M

S

K

A

L

R

K

A

A

E

E

R

K

S

K

G

G

S

N

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H

W

Y

Q

I

L

L

N

N

G

G

A

N

K

K

A

F

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x
W

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x
I

Q
x
T

H

N

G

G

E

P

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A

A

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D

L

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L

L

I

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Y

A

A

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G

D

-

L

G

A

D

A

D

V

D

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A

D
x
S

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R

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G

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L

A

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G

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T

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S

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-

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-

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K

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L

D

D

-

K

-

L

-

G

-

M

-

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G
|
G

G

S

R

N

A

T

A

C

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L

T

I

L

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D

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N

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x
Y

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G
x
D

C
x
L

G
x
E

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R

F

L

A

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L

L

C
x
A

S

G

E

G

A

P

V

L

G

G

A

L

M

M

D

Q

V

A

A

V

K

L

D

D

Y

H

T

T

L

I

E

P

Y

Y

L

L

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H

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R

K

E

Q

A

F

F

G

G

V

Q

P

K

I

I

G

G

S

H

F

F

Q
|
Q

A

L

L

M

Q

Q

H

G

R

K

M

M

A

A

D

D

L

M

L

Y

L

T

E

R

I

L

E

M

Q

A

A

C

R

R

S

Q

A

Y

V

V

I

Y

N

N

A

V

A

A

A

K

A

A

L

C

G

D

A

E

E

G

R

H

K

C

V

T

R

A

E

K

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D

A

C

V

A

S

G

A

V

A

I

K

L

Y

Y

S

S

I

-

G

A

R

E

I

C

G

A

A

T

L

Q

V

V

A

A

E

L

E

D

C

G

I
|
I

Q
|
Q

L
x
C

H
x
F

G
|
G

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N

G

G

M

Y

T

I

W

N

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D

L

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P

P

L

M

A

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Y

F

A

L

K
x
R

165:174 (vs. 121:130, 40% identical) binding
S174 (≠ Y130) binding
WIT 198:200 (≠ VVQ 154:156) binding
SR 222:223 (≠ DA 177:178) binding
G250 (= G202) to A: in IVA; unknown pathological significance
Y277 (≠ A229) binding
DLER 284:287 (≠ GCGV 236:239) binding
E286 (≠ G238) active site, Proton acceptor; mutation to D: Residual isovaleryl-CoA dehydrogenase activity.; mutation to G: Loss of isovaleryl-CoA dehydrogenase activity. Does not affect isovaleryl-CoA dehydrogenase activity; when associated with 407-E.; mutation to Q: Loss of isovaleryl-CoA dehydrogenase activity.
A291 (≠ C243) to V: in IVA; unknown pathological significance; dbSNP:rs886042098
R312 (= R264) binding
Q323 (= Q275) binding
I379 (= I332) to T: in IVA; unknown pathological significance
QCFGG 380:384 (≠ QLHGG 333:337) binding
R398 (≠ K351) to Q: in IVA; unknown pathological significance; dbSNP:rs1477527791

Sites not aligning to the query:

1:32 modified: transit peptide, Mitochondrion
403 Y → N: in IVA; unknown pathological significance
407 A→E: Does not affect isovaleryl-CoA dehydrogenase activity; when associated with 286-D.
407:408 binding
409:411 binding

6wy8C Tcur3481-tcur3483 steroid acad (see paper)
33% identity, 99% coverage: 1:370/375 of query aligns to 1:354/364 of 6wy8C

query
sites
6wy8C

M

M

N

D

F

F

Q

T

H

L

T

G

E

E

D

E

R

L

R

T

M

E

L

L

A

-

D

Q

T

G

L

L

N

A

R

R

F

Q

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F

E

T

Q

D

Y

H

A

A

F

T

S

H

T

Q

R

R

D

L

R

R

I

A

A

V

Q

E

S

T

D

S

Q

E

G

S

Y

R

S

I

G

D

E

E

-

T

L

L

W

W

S

R

R

E

F

L

A

A

E

G

L

A

G

G

I

L

G

G

A

V

L

A

F

L

D

P

E

E

A

A

D

A

G

G

F

A

G

G

G

L

G

G

G

L

F

G

D

A

I

L

A

C

V

V

V

L

F

L

E

E

S

E

L

Q

G

G

R

R

G

H

L

V

V

A

V

P

E

V

P

P

F

L

L

W

D

P

T

T

L

L

I

V

V

A

G

A

R

L

A

A

I

I

A

A

R

E

S

H

G

G

N

T

E

A

A

E

Q

Q

K

R

A

D

V

L

L

L

G

P

E

G

L

V

I

V

D

D

G

G

S

S

R

R

I

R

V

L

A

T

L

V

A

A

H
x
L

G
x
E

E

E

P

-

D

-

S

-

H

-

Y

F

E

G

L

V

S

G

R

D

V

V

S

A

A

A

-

P

-

G

-

C

R

T

A

A

E

V

R

P

S

D

G

G

S

D

T

G

W

W

Q

R

L

L

N

S

G

G

A

T

K

K

A

A

V

V

Q

P

H

S

G

I

E

T

H

G

A

A

S

A

L

H

F

L

L

L

V

V

S

S

A

A

R

-

T

T

G

G

G

P

D

D

D

-

S

-

D

-

A

-

G

G

I

P

T

G

L

L

F

F

L

L

V

V

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D

R

A

D

D

A

A

A

P

G

G

V

L

S

S

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W

R

E

G

R

Y

T

R

E

K

T

I

T

D

S

G

R

G

D

R

M

A

A

A

G

E

N

V

L

T

T

L

L

D

D

N

A

V

V

T

P

L

A

S

R

D

-

A

A

L

L

L

-

G

-

T

-

P

-

D

-

A

-

G

G

F

P

A

A

T

A

L

L

E

P

Y

W

A

T

I

L

G

D

C

V

G
x
A

V

R

F

T

A

A

L

L

C

A

S

A

E

V

A

Q

V

L

G

G

A

V

M

A

D

S

V

G

A

A

K

L

D

H

Y

I

T

T

L

A

E

S

Y

Y

L

L

R

K

T

E

R
|
R

K

E

Q
|
Q

F
|
F

G

G

V

R

P

P

I
x
L

G

G

S

T

F
|
F

Q

Q

A

A

L
x
V

Q

Q

H

H

R

Q

M

L

A

A

D

D

L

C

L

Y

L

I

E

E

I

I

E

E

Q

A

A

M

R

R

S

V

A

C

V

L

I

W

N

Q

A

A

A

V

A

C

A

A

L

-

G

-

A

A

E

E

R

D

K

G

V

A

R

T

E

D

-

G

R

K

A

A

V

A

S

L

A

V

A

A

K

K

Y

W

S

W

I

A

G

D

R

E

I

G

G

G

A

L

L

N

V

V

A

V

E

H

E

R

C

T

I

Q

Q
x
H

L
|
L

H
|
H

G
|
G

I

I

G

G

M

V

T

D

W

V

E

D

L

Y

P

P

L

I

A

H

H

R

Y

Y

A

F

K

L

R

W

L

G

V

K

M

Q

I

I

D

S

H

G

Q

T

L

L

G

G

D

G

E

A

D

S

H

A

H

D

L

L

E

Q

R

R

F

L

active site: L123 (≠ H123), E124 (≠ G124), A223 (≠ G238)
binding flavin-adenine dinucleotide: R249 (= R264), Q251 (= Q266), F252 (= F267), L256 (≠ I271), F259 (= F274), V262 (≠ L277), H317 (≠ Q333), L318 (= L334), H319 (= H335), G320 (= G336), G321 (= G337)

Sites not aligning to the query:

active site: 356

6wy9B Tcur3481-tcur3483 steroid acad g363a variant (see paper)
33% identity, 95% coverage: 16:370/375 of query aligns to 12:351/361 of 6wy9B

query
sites
6wy9B

L

L

N

A

R

R

F

Q

V

I

S

F

E

T

Q

D

Y

H

A

A

F

T

S

H

T

Q

R

R

D

L

R

R

I

A

A

V

Q

E

S

T

D

S

Q

E

G

S

Y

R

S

I

G

D

E

E

-

T

L

L

W

W

S

R

R

E

F

L

A

A

E

G

L

A

G

G

I

L

G

G

A

V

L

A

F

L

D

P

E

E

A

A

D

A

G

G

F

A

G

G

G

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G

G

G

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F

G

D

A

I

L

A

C

V

V

V

L

F

L

E

E

S

E

L

Q

G

G

R

R

G

H

L

V

V

A

V

P

E

V

P

P

F

L

L

W

D

P

T

T

L

L

I

V

V

A

G

A

R

L

A

A

I

I

A

A

R

E

S

H

G

G

N

T

E

A

A

E

Q

Q

K

R

A

D

V

L

L

L

G

P

E

G

L

V

I

V

D

D

G

G

S

S

R

R

I

R

V

L

A

T

L

V

A

A

H
x
L

G
x
E

E

E

P

-

D

-

S

-

H

-

Y

F

E

G

L

V

S

G

R

D

V

V

S

A

A

A

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P

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G

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C

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A

A

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D

G

G

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W

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G

G

A

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K

A

A

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H

G

A

A

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A

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H

F

L

L

L

V

V

S

S

A

A

R

-

T

T

G

G

G

P

D

D

D

-

S

-

D

-

A

-

G

G

I

P

T

G

L

L

F

F

L

L

V

V

P

D

R

A

D

D

A

A

A

P

G

G

V

L

S

S

V

W

R

E

G

R

Y

T

R

E

K

T

I

T

D

S

G

R

G

D

R

M

A

A

A

G

E

N

V

L

T

T

L

L

D

D

N

A

V

V

T

P

L

A

S

R

D

-

A

A

L

L

L

-

G

-

T

-

P

-

D

-

A

-

G

G

F

P

A

A

T

A

L

L

E

P

Y

W

A

T

I

L

G

D

C

V

G
x
A

V

R

F

T

A

A

L

L

C

A

S

A

E

V

A

Q

V

L

G

G

A

V

M

A

D

S

V

G

A

A

K

L

D

H

Y

I

T

T

L

A

E

S

Y

Y

L

L

R

K

T

E

R
|
R

K

E

Q
|
Q

F
|
F

G

G

V

R

P

P

I
x
L

G

G

S

T

F
|
F

Q

Q

A

A

L
x
V

Q

Q

H

H

R

Q

M

L

A

A

D

D

L

C

L

Y

L

I

E

E

I

I

E

E

Q

A

A

M

R

R

S

V

A

C

V

L

I

W

N

Q

A

A

A

V

A

C

A

A

L

-

G

-

A

A

E

E

R

D

K

G

V

A

R

T

E

D

-

G

R

K

A

A

V

A

S

L

A

V

A

A

K

K

Y

W

S

W

I

A

G

D

R

E

I

G

G

G

A

L

L

N

V

V

A

V

E

H

E

R

C

T

I

Q

Q
x
H

L
|
L

H
|
H

G
|
G

I

I

G

G

M

V

T

D

W

V

E

D

L

Y

P

P

L

I

A

H

H

R

Y

Y

A

F

K

L

R

W

L

G

V

K

M

Q

I

I

D

S

H

G

Q

T

L

L

G

G

D

G

E

A

D

S

H

A

H

D

L

L

E

Q

R

R

F

L

active site: L120 (≠ H123), E121 (≠ G124), A220 (≠ G238)
binding dihydroflavine-adenine dinucleotide: R246 (= R264), Q248 (= Q266), F249 (= F267), L253 (≠ I271), F256 (= F274), V259 (≠ L277), H314 (≠ Q333), L315 (= L334), H316 (= H335), G317 (= G336), G318 (= G337)

Sites not aligning to the query:

active site: 353

4ktoA Crystal structure of a putative isovaleryl-coa dehydrogenase (psi- nysgrc-012251) from sinorhizobium meliloti 1021
31% identity, 92% coverage: 1:345/375 of query aligns to 6:345/377 of 4ktoA

query
sites
4ktoA

M

L

N

N

F

F

Q

A

H

L

T

G

E

E

D

E

R

I

R

D

M

A

L

L

A

R

D

A

T

S

L

V

N

R

R

R

F

F

V

A

S

S

E

E

Q

R

Y

I

A

A

F

P

S

L

T

A

R

D

D

D

R

-

I

-

A

A

Q

D

S

R

D

S

Q

N

G

A

Y

F

S

P

G

M

E

S

L

L

W

W

S

R

R

E

F

M

A

G

E

E

L

L

G

G

I

L

G

G

A

I

L

T

F

A

D

D

E

E

A

A

D

H

G

G

F

A

G

G

G

L

G

G

G

Y

F

L

D

A

I

H

A

C

V

V

V

A

F

M

E

E

S

E

L

I

G

S

R

R

-

A

-

S

-

A

-

S

-

V

G

G

L

L

V

S

V

Y

E

G

P

A

F

H

L

S

D

N

T

L

L

C

I

V

V

-

G

-

R

N

A

Q

I

I

A

N

R

R

S

N

G

G

N

K

E

P

A

A

Q

Q

K

K

A

S

-

R

V

Y

L

L

G

P

E

K

L

L

I

I

D

S

G

G

S

E

R

H

I

V

V

G

A

A

L
|
L

A
|
A

H
x
M

G
x
S

E

E

P

P

D

G

S

V

H

S

Y

M

E

K

L

L

S

-

R

-

V

-

S

-

A

-

R

K

A

A

E

D

R

K

S

R

G

G

S

D

T

R

W

Y

Q

V

L

L

N

N

G

G

A

S

K

K

A
x
M

V
x
W

V
x
I

Q
x
T

H

N

G

G

E

P

H

D

A

A

S

D

L

V

F

L

L

V

V

V

S

Y

A

A

R

K

T

T

G

-

D

-

D

D

S

P

D

P

A

R

G

G

I

I

T

T

L

A

F

F

L

L

V

V

P

E

R

K

D

A

A

F

A

P

G

G

V

F

S

S

V

A

R

G

G

-

Y

-

R

Q

K

K

I

L

D

D

-

K

-

L

-

G

-

M

-

R

G

G

G

S

R

N

A

T

A

S

E

E

V

L

T

I

L

F

D

T

N

D

V

C

T

E

L

V

S

P

D

E

A

N

L

V

L

L

G

G

T

G

P

V

D

G

A

E

G

G

F

V

A

K

T

V

L

L

E

M

Y

S

A

G

I

L

G

D

C

Y

G
x
E

V

R

F

V

A

V

L

L

C

S

S

A

E

G

A

P

V

L

G

G

A

I

M

M

D

A

V

A

A

C

K

L

D

D

Y

V

T

V

L

V

E

P

Y

Y

L

L

R

H

T

E

R
|
R

K

K

Q
|
Q

F
|
F

G

G

V

Q

P

P

I
|
I

G

G

S

E

F
|
F

Q

Q

A

L

L
x
M

Q

Q

H

G

R

K

M

L

A

A

D

D

L

M

L

Y

L

V

E

T

I

M

E

N

Q

A

A

A

R

R

S

A

A

Y

V

V

I

Y

N

A

A

V

A

A

L

C

G

D

A

R

E

G

R

E

K

T

V

A

R

R

E

K

R

D

A

A

V

A

S

G

A

C

A

I

K

L

Y

Y

S

A

I

A

G

E

R

K

I

A

G

T

A

A

L

M

V

-

A

A

E

L

E

E

C

A

I

I

Q
|
Q

L
x
A

H

L

G

G

G
|
G

I

N

G

G

M

Y

T

T

W

N

E

D

L

Y

P

P

active site: M130 (≠ H123), S131 (≠ G124), E239 (≠ G238)
binding flavin-adenine dinucleotide: L128 (= L121), A129 (= A122), M130 (≠ H123), S131 (≠ G124), M155 (≠ A153), W156 (≠ V154), I157 (≠ V155), T158 (≠ Q156), R265 (= R264), Q267 (= Q266), F268 (= F267), I272 (= I271), F275 (= F274), M278 (≠ L277), Q333 (= Q333), A334 (≠ L334), G337 (= G337)

Sites not aligning to the query:

active site: 360, 372
binding flavin-adenine dinucleotide: 355, 358, 359, 360, 361, 362, 364, 365

P16219 Short-chain specific acyl-CoA dehydrogenase, mitochondrial; SCAD; Butyryl-CoA dehydrogenase; EC 1.3.8.1 from Homo sapiens (Human) (see 3 papers)
27% identity, 81% coverage: 45:349/375 of query aligns to 69:382/412 of P16219

query
sites
P16219

S

A

R

Q

F

V

A

K

E

K

L

M

G

G

I

G

I

L

G

G

A

L

L

L

F

A

D

M

E

D

A

V

D

P

G

E

F

L

G

G

G
|
G

G

A

G

G

F

L

D

D

I

Y

-

L

-

A

-

Y

A

A

V

I

V

A

F

M

E

E

S
x
E

L

I

G

S

R

R

G

G

L

C

-

A

-

S

-

T

-

G

V

V

V

I

E

M

P

S

F

V

L

N

D

N

T

S

L

L

I

Y

V

L

G

G

R

P

A

I

I

L

A

K

R

F

S

G

G

S

N

K

E

E

A

Q

Q

K

K

Q

A

A

V

W

L

V

G

T

E

P

L

F

I

T

D

S

G

G

S

D

R

K

I

I

V

G

A

C

L
x
F

A
|
A

H
x
L

G
x
S

E
|
E

P
|
P

D
x
G

S
x
N

H
x
G

Y
x
S

E

D

L

A

S

G

R

A

V

A

S

S

A

T

R

T

A

A

E
x
R

R

A

S

E

G

G

S

D

T

S

W

W

Q

V

L

L

N

N

G

G

A

T

K

K

A

A

V
x
W

V
x
I

Q
x
T

H

N

G

A

E

W

H

E

A
|
A

S

S

L

A

F

A

L

V

V

V

S

F

A

A

R

S

T

T

G

D

G

-

D

R

D

A

D

L

S

Q

D

N

A

K

G
|
G

I

I

T

S

L

A

F

F

L

L

V

V

P

P

R

M

D

P

A

T

A

P

G

G

V

L

S

T

V

L

R

-

G

G

Y

K

R

K

K

E

-

D

-

K

-

L

-

G

I

I

D

R

G

G

G

S

R

S

A

T

A

A

E

N

V

L

T

I

L

F

D

E

N

D

V

C

T

R

L

I

S

P

D

K

D

D

A

S

L

I

L

L

G

G

T

E

P

P

D

G

A

M

G

G

F

F

A

K

T

I

L

A

E

M

Y

Q

A

T

I

L

G

D

C

M

G

G

V

R

F

I

A

G

L

I

C

A

S

S

E

Q

A

A

V

L

G

G

A

I

M

A

D

Q

V

T

A

A

K

L

D

D

Y

C

T

A

L

V

E

N

Y

Y

L

A

R

E

T

N

R
|
R

K

M

Q

A

F

F

G

G

V

A

P

P

I

L

G

T

S

K

F

L

Q
|
Q

A

V

L

I

Q

Q

H

F

R

K

M

L

A

A

D

D

L

M

L

A

L

L

E

A

I

L

E

E

Q

S

A

A

R
|
R

S

-

A

L

V

L

I

T

N

W

A

R

A

A

A

M

L

L

G

K

A

D

E

N

R

K

K

K

V

P

R

F

E

I

R

K

A

E

V

A

S

A

A

M

A

A

K

K

Y

L

S

A

I

A

G
x
S

R

E

I

A

G

A

A

T

L

A

V

I

A

S

E

H

E

Q

C

A

I

I

Q
|
Q

L
x
I

H
x
L

G
|
G

I

M

G

G

M

Y

T

V

W

T

E

E

L

M

P

P

L

A

A

E

-
x
R

H

H

Y

Y

G90 (= G66) to S: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs121908005
E104 (≠ S77) natural variant: Missing (in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs387906308)
152:161 (vs. 121:130, 30% identical) binding in other chain
R171 (≠ E140) to W: 69% of wild-type acyl-CoA dehydrogenase activity; confers susceptibility to ethylmalonicaciduria; dbSNP:rs1800556
WIT 185:187 (≠ VVQ 154:156) binding in other chain
A192 (= A161) to V: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs28940874
G209 (= G179) to S: 86% of wild-type acyl-CoA dehydrogenase activity; confers susceptibility to ethylmalonicaciduria; dbSNP:rs1799958
R297 (= R264) binding
Q308 (= Q275) binding in other chain
R325 (= R292) to W: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs121908006
S353 (≠ G321) to L: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs28941773
QILGG 365:369 (≠ QLHGG 333:337) binding
R380 (vs. gap) to W: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs28940875

Sites not aligning to the query:

1:24 modified: transit peptide, Mitochondrion
394:396 binding in other chain

2vigB Crystal structure of human short-chain acyl coa dehydrogenase
27% identity, 81% coverage: 45:349/375 of query aligns to 36:342/371 of 2vigB

query
sites
2vigB

S

A

R

Q

F

V

A

K

E

K

L

M

G

G

I

G

I

L

G

G

A

L

L

L

F

A

D

M

E

D

A

V

D

P

G

E

F

L

G

G

G

A

G

G

F

L

D

D

I

Y

-

L

-

A

-

Y

A

A

V

I

V

A

F

M

E

E

S

E

L

I

G

S

R

R

G

G

L

C

-

A

-

S

-

T

-

G

V

V

V

I

E

M

P

S

F

V

L

N

D

N

T

S

L

L

I

Y

V

L

G

G

R

P

A

I

I

L

A

K

R

F

S

G

G

S

N

K

E

E

A

Q

Q

K

K

Q

A

A

V

W

L

V

G

T

E

P

L

F

I

T

D

S

G

G

S

D

R

K

I

I

V

G

A

C

L
x
F

A
|
A

H
x
L

G
x
S

E

E

P

P

D

G

S

N

H
x
G

Y
x
S

E

D

L

A

S

G

R

A

V

A

S

S

A

T

R

T

A

A

E

R

R

A

S

E

G

G

S

D

T

S

W

W

Q

V

L

L

N

N

G

G

A

T

K

K

A
|
A

V
x
W

V
x
I

Q
x
T

H

N

G

A

E

W

H

E

A

A

S

S

L

A

F

A

L

V

V

V

S

F

A

A

R

S

T

T

G

S

G

-

D

-

S

-

D

-

A

-

G

-

I

I

T

S

L

A

F

F

L

L

V

V

P

P

R

M

D

P

A

T

A

P

G

G

V

L

S

T

V

L

R

-

G

G

Y

K

R

K

K

E

-

D

-
x
K

-

L

-

G

I

I

D

R

G

G

G

S

R

S

A
x
T

A

A

E

N

V

L

T

I

L

F

D

E

N

D

V

C

T

R

L

I

S

P

D

K

D

D

A

S

L

I

L

L

G

G

T

E

P

P

D

G

A

M

G

G

F
|
F

A
x
K

T

I

L

A

E
x
M

Y
x
Q

A

T

I
x
L

G
x
D

C

M

G
|
G

V
x
R

F

I

A

G

L

I

C

A

S

S

E

Q

A

A

V

L

G

G

A

I

M

A

D

Q

V

T

A

A

K

L

D

D

Y

C

T

A

L

V

E

N

Y

Y

L

A

R

E

T

N

R
|
R

K

M

Q

A

F
|
F

G

G

V

A

P

P

I
x
L

G

T

S

K

F
x
L

Q

Q

A

V

L
x
I

Q

Q

H

F

R

K

M

L

A

A

D

D

L

M

L

A

L

L

E

A

I

L

E

E

Q

S

A

A

R

R

S

-

A

L

V

L

I

T

N

W

A

R

A

A

A

M

L

L

G

K

A

D

E

N

R

K

K

K

V

P

R

F

E
x
I

R

K

A

E

V

A

S

A

A

M

A

A

K

K

Y

L

S

A

I

A

G

S

R

E

I

A

G

A

A

T

L

A

V

I

A

S

E

H

E

Q

C

A

I

I

Q
|
Q

L
x
I

H

L

G
|
G

I

M

G

G

M

Y

T

V

W

T

E

E

L

M

P

P

L

A

A

E

-

R

H

H

Y

Y

active site: L121 (≠ H123), S122 (≠ G124), G231 (= G238)
binding coenzyme a persulfide: F119 (≠ L121), G127 (≠ H129), S128 (≠ Y130), F221 (= F228), K222 (≠ A229), M225 (≠ E232), Q226 (≠ Y233), L228 (≠ I235), D229 (≠ G236), R232 (≠ V239), I302 (≠ E310)
binding flavin-adenine dinucleotide: F119 (≠ L121), A120 (= A122), L121 (≠ H123), S122 (≠ G124), G127 (≠ H129), S128 (≠ Y130), A151 (= A153), W152 (≠ V154), I153 (≠ V155), T154 (≠ Q156), K190 (vs. gap), T198 (≠ A205), R257 (= R264), F260 (= F267), L264 (≠ I271), L267 (≠ F274), I270 (≠ L277), Q325 (= Q333), I326 (≠ L334), G328 (= G336), G329 (= G337)

Sites not aligning to the query:

active site: 352, 364
binding coenzyme a persulfide: 352, 353, 357
binding flavin-adenine dinucleotide: 347, 350, 351, 354, 356, 357, 360

4n5fA Crystal structure of a putative acyl-coa dehydrogenase with bound fadh2 from burkholderia cenocepacia j2315
30% identity, 90% coverage: 1:339/375 of query aligns to 2:343/378 of 4n5fA

query
sites
4n5fA

M

M

N

D

F

E

Q

L

H

Y

T

T

E

E

D

D

R

Q

R

R

M

M

L

I

A

R

D

D

T

A

L

A

N

R

R

A

F

F

V

A

S

T

E

E

Q

M

Y

L

A

A

F

P

S

N

T

A

R

-

D

-

R

-

I

-

A

A

Q

Q

S

W

D

D

Q

H

G

D

-

A

-

H

Y

L

S

P

G

D

E

A

L

I

W

V

S

A

R

Q

F

L

A

G

E

E

L

L

G

G

I

L

G

G

A

M

L

I

F

V

D

P

E

Q

A

E

D

L

G

G

F

S

G

Y

G

T

G

D

G

Y

F

V

D

A

I

Y

A

A

V

L

V

A

F

M

E

E

S

E

L

V

G

A

R

A

G

G

-

D

-

A

-

C

L

A

V

T

V

M

E

M

P

S

F

V

L

H

D

N

T

S

L

V

I

G

V

C

G

G

R

-

A

P

I

I

A

L

R

G

S

F

G

G

N

T

E

P

A

A

Q

Q

K

K

-

D

A

R

V

W

L

L

G

A

E

D

L

M

I

A

D

A

G

G

S

R

R

V

I

I

V

G

A

A

L
x
F

A

C

H
x
L

G
x
T

E

E

P

P

D

H

S

A

H
x
G

Y
x
S

E

E

L

A

S

N

R

N

V

L

S

R

A

T

R

R

A

A

E

E

R

L

S

R

G

D

S

G

T

Q

W

W

Q

V

L

L

N

N

G

G

A

A

K

K

A

Q

V
x
F

V
|
V

Q
x
T

H

N

G

G

E

Q

H

R

A

A

S

G

L

V

F

A

L

I

V

V

S

F

A

A

R

M

T

T

G

-

D

-

D

D

S

P

D

E

A

A

G

G

-

K

-

R

-

G

I

I

T

S

L

A

F

F

L

L

V

V

P

P

R

T

D

D

A

T

A

P

G

G

V

F

S

I

V

V

R

G

G

K

Y

P

R

E

K

K

I

K

D

M

G

G

G

I

R

R

A

A

A

S

E

D

-
x
T

-

C

-

P

V

I

T

T

L

F

D

E

N

N

V

C

T

A

L

I

S

P

D

E

D

D

A

N

L

L

G

G

T

N

P

R

D

G

A

E

G

G

F

L

A

K

T

I

L

A

E

L

Y

S

A

N

I

L

G

E

C

G

G
|
G

V

R

F

I

A

G

L

I

C

A

S

A

E

Q

A

A

V

L

G

G

A

I

M

A

D

R

V

A

A

A

K

F

D

D

Y

K

T

A

L

R

E

R

Y

Y

L

A

R

G

T

E

R

R

K

V

Q

Q

F

F

G

G

V

K

P

P

I

I

G

A

S

E

F

H

Q

Q

A

A

L

I

Q

Q

H

Q

R

K

M

L

A

A

D

D

L

M

L

A

L

V

E

Q

I

I

E

N

Q

A

A

A

R

R

S

L

A

L

V

V

I

H

N

H

A

A

A

K

A

L

L

R

G

T

A

A

E

G

R

L

K

P

V

C

R

L

E

S

R

E

A

A

V

-

S

S

A

Q

A

A

K

K

Y

L

S

F

I

A

G

S

R

E

I

M

G

A

A

E

L

R

V

V

A

C

E

S

E

D

C

A

I

I

Q

Q

L

I

H

H

G

G

I

Y

G

G

active site: L126 (≠ H123), T127 (≠ G124), G243 (= G238)
binding dihydroflavine-adenine dinucleotide: F124 (≠ L121), L126 (≠ H123), T127 (≠ G124), G132 (≠ H129), S133 (≠ Y130), F157 (≠ V154), V158 (= V155), T159 (≠ Q156), T210 (vs. gap)

Sites not aligning to the query:

active site: 364, 376
binding dihydroflavine-adenine dinucleotide: 359, 363, 366, 368, 372

P15651 Short-chain specific acyl-CoA dehydrogenase, mitochondrial; SCAD; Butyryl-CoA dehydrogenase; EC 1.3.8.1 from Rattus norvegicus (Rat) (see 2 papers)
26% identity, 81% coverage: 45:349/375 of query aligns to 69:381/412 of P15651

query
sites
P15651

S

S

R

Q

F

V

A

K

E

K

L

M

G

G

I

E

I

L

G

G

A

L

L

L

F

A

D

M

E

D

A

V

D

P

G

E

F

L

G

S

G

G

G

A

G

G

F

L

D

D

I

Y

-

L

-

A

-

Y

A

S

V

I

V

A

F

L

E

E

S

E

L

I

G

S

R

R

G

G

L

C

-

A

-

S

-

T

-

G

V

V

V

I

E

M

P

S

F

V

L

N

D

N

T

S

L

L

I

Y

V

L

G

G

R

P

A

I

I

L

A

K

R

F

S

G

G

S

N

S

E

Q

A

Q

Q

K

K

Q

A

Q

V

W

L

I

G

T

E

P

L

F

I

T

D

N

G

G

S

D

R

K

I

I

V

G

A

C

L
x
F

A
|
A

H
x
L

G
x
S

E
|
E

P
|
P

D
x
G

S
x
N

H
x
G

Y
x
S

E

D

L

A

S

G

R

A

V

A

S

S

A

T

R

T

A

A

E

R

R

E

S

E