SitesBLAST

165:174 (vs. 128:137, 100% identical) binding
S174 (= S137) binding
WIT 198:200 (= WIT 161:163) binding
SR 222:223 (≠ -H 184) binding
G250 (= G211) to A: in IVA; unknown pathological significance
Y277 (≠ K238) binding
DLER 284:287 (≠ DYER 245:248) binding
E286 (= E247) 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 (≠ S252) to V: in IVA; unknown pathological significance; dbSNP:rs886042098
R312 (= R273) binding
Q323 (= Q284) binding
I379 (= I340) to T: in IVA; unknown pathological significance
QCFGG 380:384 (≠ QILGG 341:345) binding
R398 (= R359) to Q: in IVA; unknown pathological significance; dbSNP:rs1477527791
Y403 (= Y364) to N: in IVA; unknown pathological significance
A407 (= A368) mutation to E: Does not affect isovaleryl-CoA dehydrogenase activity; when associated with 286-D.
AG 407:408 (= AG 368:369) binding
TSE 409:411 (= TSE 370:372) binding

Sites not aligning to the query:

1:32 modified: transit peptide, Mitochondrion

1ivhA Structure of human isovaleryl-coa dehydrogenase at 2.6 angstroms resolution: structural basis for substrate specificity (see paper)
65% identity, 96% coverage: 10:380/387 of query aligns to 8:382/387 of 1ivhA

query
sites
1ivhA

L

L

G

S

E

E

T

E

I

Q

D

R

M

Q

L

L

R

R

D

Q

Q

T

V

M

Q

A

S

K

F

F

V

L

A

Q

K

E

E

H

I

L

A

A

P

P

R

K

A

A

A

Q

Q

E

I

I

D

D

I

R

D

S

N

N

L

E

F

F

P

K

-

N

-

L

A

R

D

E

L

F

W

W

R

K

K

Q

F

L

G

G

D

N

M

L

G

G

L

V

L

L

G

G

I

I

T

T

V

A

P

P

E

V

E

Q

Y

Y

G

G

A

S

G

G

L

L

G

G

Y

Y

L

L

A

E

H

H

V

V

V

L

A

V

M

M

E

E

I

I

S

S

R

R

G

A

S

S

A

G

S

A

V

V

A

G

L

L

S

S

Y

Y

G

G

A

A

H

H

S

S

N

N

L

L

C

C

V

I

N

N

Q

Q

I

L

N

V

R

R

N

N

G

G

T

N

H

E

E

A

Q

Q

K

K

S

E

K

K

Y

Y

L

L

P

P

K

K

L

L

I

I

S

S

G

G

E

E

H

Y

I

I

G

G

A

A

L
|
L

A
|
A

M
|
M

S
|
S

E

E

P

P

N

N

A

A

G
|
G

S
|
S

D

D

V
|
V

S

S

M

M

K

K

L

L

R

K

A

A

D

E

K

K

R

K

G

G

D

N

H

H

Y

Y

V

I

L

L

N

N

G

G

S

N

K

K

T

F

W
|
W

I
|
I

T
|
T

N

N

G

G

P

P

D

D

A

A

N

D

T

V

Y

L

V

I

I

V

Y

Y

A

A

K

K

T

T

D

D

L

L

E

A

K

A

G

V

P

P

-

A

-
x
S

H
x
R

G

G

I

I

T

T

A

A

F

F

I

I

V

V

E

E

R

K

D

G

S

M

K

P

G

G

F

F

S

S

R

T

S

S

N

K

K

K

F

L

D

D

K

K

L

L

G

G

M

M

R

R

G

G

S

S

N

N

T
|
T

C

C

E

E

L

L

F

I

F

F

D

E

D

D

V

C

E

K

V

I

P

P

E

A

N

N

I

I

L

L

G

G

V

H

L

E

N

N

G

K

G

G

V
|
V

K
x
Y

V

V

L

L

M
|
M

S

S

G

G

L
|
L

D
|
D

Y

L

E
|
E

R
|
R

V

L

V

V

L

L

S

A

G

G

P

P

T

L

G

G

I

L

M

M

Q

Q

A

A

C

V

M

L

D

D

L

H

I

T

V

I

P

P

Y

Y

I

L

H

H

D

V

R
|
R

K

E

Q
x
A

F
|
F

G

G

Q

Q

S

K

I
|
I

G

G

E

H

F
|
F

Q

Q

L
|
L

I
x
M

Q

Q

G

G

K

K

V

M

A

A

D

D

M

M

Y

Y

T

T

Q

R

L

L

N

M

A

A

S

C

R

R

A

Q

Y

Y

L

V

Y

Y

A

N

V

V

A

A

Q

K

A

A

C

C

E

D

R

E

G

G

E

H

T

C

A

T

R

A

K

K

D

D

A

C

A

A

G

G

V

V

I

I

L

L

Y

Y

S

S

A

A

E

E

C

C

A

A

T

T

R

Q

M

V

A

A

L

L

D

D

A

G

I

I

Q
|
Q

I
x
C

L

F

G
|
G

N

N

G

G

Y

Y

I

I

N

N

E

D

F

F

P

P

A

M

G

G

R

R

L

F

L

L

R

R

D

D

A

A

K

K

L
|
L

Y

Y

E

E

I
|
I

G
|
G

A
|
A

G
|
G

T
|
T

S

S

E
|
E

I
x
V

R

R

M
x
L

L

V

I

I

G

G

R
|
R

active site: M130 (= M130), S131 (= S131), E249 (= E247), A370 (= A368), R382 (= R380)
binding coenzyme a persulfide: M130 (= M130), G136 (= G136), S137 (= S137), V139 (= V139), V140 (= V140), S185 (vs. gap), R186 (≠ H184), V239 (= V237), Y240 (≠ K238), M243 (= M241), L246 (= L244), D247 (= D245), E249 (= E247), R250 (= R248), G369 (= G367), A370 (= A368), G371 (= G369), V375 (≠ I373), R382 (= R380)
binding flavin-adenine dinucleotide: L128 (= L128), A129 (= A129), M130 (= M130), S131 (= S131), G136 (= G136), S137 (= S137), W161 (= W161), I162 (= I162), T163 (= T163), T216 (= T214), R275 (= R273), A277 (≠ Q275), F278 (= F276), I282 (= I280), F285 (= F283), L287 (= L285), M288 (≠ I286), Q343 (= Q341), C344 (≠ I342), G346 (= G344), G347 (= G345), L365 (= L363), I368 (= I366), T372 (= T370), E374 (= E372), L378 (≠ M376)

5lnxD Crystal structure of mmgc, an acyl-coa dehydrogenase from bacillus subtilis.
43% identity, 95% coverage: 16:383/387 of query aligns to 8:373/374 of 5lnxD

query
sites
5lnxD

M

M

L

M

R

R

D

K

Q

M

V

V

Q

R

S

D

F

F

V

A

A

R

K

K

E

E

I

I

A

A

P

P

R

A

A

A

A

E

Q

I

I

M

D

E

I

K

D

T

N

D

L

E

F

F

P

P

A

F

D

Q

L

L

W

I

R

K

K

K

F

M

G

G

D

K

M

H

G

G

L

L

L

M

G

G

I

I

T

P

V

V

P

P

E

E

E

Q

Y

Y

G

G

A

A

G

G

L

A

G

D

Y

V

L

V

A

S

H

Y

V

I

V

L

A

A

M

I

E

H

E

E

I

I

S

S

R

R

G

I

S

S

A

A

S

A

V

V

A

G

L

V

S

I

Y

L

G

S

A

V

H

H

S

T

N

S

L

V

C

G

V

T

N

N

Q

P

I

I

N

L

R

Y

N

F

G

G

T

N

H

E

E

E

Q

Q

K

K

S

M

K

K

Y

Y

L

I

P

P

K

N

L

L

I

A

S

S

G

G

E

D

H

H

I

L

G

G

A

A

L
x
F

A

A

M
x
L

S
x
T

E

E

P

P

N

H

A

S

G
|
G

S
|
S

D

D

V

A

V

G

S

S

M

L

K

R

L

T

R

T

A

A

D

I

K

K

R

K

G

N

D

G

H

K

Y

Y

V

L

L

L

N

N

G

G

S

S

K

K

T

I

W
x
F

I
|
I

T
|
T

N

N

G

G

P

G

D

A

A

A

N

D

T

I

Y

Y

V

I

I

T

Y

F

A

A

K

L

T

T

D

A

L

P

E

D

K

Q

G

G

P

R

H

H

G

G

I

I

T

S

A

A

F

F

I

I

V

V

E

E

R

K

D

N

S

T

K

P

G

G

F

F

S

T

R

V

S

G

N

K

K

K

F

E

D

R

K

K

L

L

G

G

M

L

R

Y

G

G

S

S

N

N

T

T

C

T

E

E

L

L

F

I

F

F

D

D

D

N

V

A

E

E

V

V

P

P

E

E

E

A

N

N

I

L

L

L

G

G

V

K

L

E

N

G

G

D

G

G

V

F

K

H

V

I

L

A

M

M

S

A

G

N

L

L

D

N

Y

V

E
x
G

R

R

V

I

V

G

L

I

S

A

G

A

G

Q

P

A

T

L

G

G

I

I

M

A

Q

E

A

A

C

A

M

L

D

E

L

H

I

A

V

V

P

D

Y

Y

I

A

H

K

D

Q

R
|
R

K

V

Q
|
Q

F
|
F

G

G

Q

R

S

P

I
|
I

G

A

E

A

F
x
N

Q

Q

L

G

I
|
I

Q

S

G

F

K

K

V

L

A

A

D

D

M

M

Y

A

T

T

Q

R

L

A

N

E

A

A

S

A

R

R

A

H

Y

L

L

V

Y

Y

A

H

V

A

A

A

Q

D

A

L

C

H

E

N

R

R

G

L

E

N

T

C

A

G

R

-

K

K

D

E

A

A

A

S

G

M

V

A

I

K

L

Q

Y

F

S

A

A

S

E

D

C

A

A

A

T

V

R

K

M

-

A

A

L

L

D

D

A

A

I

V

Q
|
Q

I
|
I

L

Y

G
|
G

N
x
Y

G

G

Y

Y

I

M

N

K

E

D

F

Y

P

P

A

V

G

E

R

R

L

L

R

R

D

D

A

A

K

K

L

V

Y

T

E

Q

I

I

G
x
Y

A
x
E

G

G

T
|
T

S

N

E
|
E

I

I

R

Q

R

R

M

L

L

I

I

I

G

S

R
x
K

E

Y

L

L

F

L

active site: L122 (≠ M130), T123 (≠ S131), G239 (≠ E247), E358 (≠ A368), K370 (≠ R380)
binding flavin-adenine dinucleotide: F120 (≠ L128), L122 (≠ M130), T123 (≠ S131), G128 (= G136), S129 (= S137), F153 (≠ W161), I154 (= I162), T155 (= T163), R265 (= R273), Q267 (= Q275), F268 (= F276), I272 (= I280), N275 (≠ F283), I278 (= I286), Q331 (= Q341), I332 (= I342), G334 (= G344), G335 (= G345), Y336 (≠ N346), Y357 (≠ G367), T360 (= T370), E362 (= E372)

1jqiA Crystal structure of rat short chain acyl-coa dehydrogenase complexed with acetoacetyl-coa (see paper)
41% identity, 98% coverage: 10:387/387 of query aligns to 7:384/384 of 1jqiA

query
sites
1jqiA

L

L

G

P

E

E

T

T

I

H

D

Q

M

M

L

L

R

R

D

Q

Q

T

V

C

Q

R

S

D

F

F

V

A

A

E

K

K

E

E

I

L

A

V

P

P

R

I

A

A

Q

Q

I

L

D

D

I

K

D

E

N

H

L

L

F

F

P

P

A

T

D

S

L

Q

W

V

R

K

K

K

F

M

G

G

D

E

M

L

G

G

L

L

G

A

I

M

T

D

V

V

P

P

E

E

Y

L

G

S

G

G

A

A

G

G

L

L

G

D

Y

Y

L

L

A

A

H

Y

V

S

V

I

A

A

M

L

E

E

I

I

S

S

R

R

G

G

S

C

A

A

S

S

V

T

A

G

L

V

S

I

Y

M

G

S

A

V

H

N

S

N

N

S

L
|
L

C

Y

V

L

N

G

Q

P

I

I

N

L

R

K

N

F

G

G

T

S

H

S

E

Q

Q

Q

K

K

S

Q

K

Q

Y

W

L

I

P

T

K

P

L

F

I

T

S

N

G

G

E

D

H

K

I

I

G

G

A

C

L
x
F

A
|
A

M
x
L

S
|
S

E

E

P

P

N

G

A

N

G
|
G

S
|
S

D

D

V
x
A

V

G

S

A

M

A

K

S

L

T

R

T

A

A

D

R

K

E

R

E

G

G

D

D

H

S

Y

W

V

V

L

L

N

N

G

G

S

T

K

K

T

A

W
|
W

I
|
I

T
|
T

N

N

G

S

P

W

D

E

A

A

N

S

T

A

Y

T

V

V

I

V

Y

F

A

A

K

S

T

T

D

D

L

R

E

S

K

R

G

Q

P

N

H
x
K

G

G

I

I

T

S

A

A

F

F

I

L

V

V

E

P

R

M

D

P

S

T

K

P

G

G

F

L

S

T

R

L

S

G

N

K

K

K

F

E

D

D

K
|
K

L

L

G

G

M

I

R

R

G

A

S

S

N

S

T
|
T

C

A

E

N

L

L

F

I

F

F

D

E

D

D

V

C

E

R

V

I

P

P

E

K

E

E

N

N

I

L

L

L

G

G

V

E

L

P

N

G

G

M

G

G

V
x
F

K
|
K

V

I

L

A

M
|
M

S
x
Q

G

T

L
|
L

D
|
D

Y

M

E

G

R
|
R

V

I

V

G

L

I

S

A

G

S

G

Q

P

A

T

L

G

G

I

I

M

A

Q

Q

A

A

C

S

M

L

D

D

L

C

I

A

V

V

P

K

Y

Y

I

A

H

E

D

N

R
|
R

K

H

Q

A

F
|
F

G

G

Q

A

S

P

I
x
L

G

T

E

K

F
x
L

Q

Q

L

N

I
|
I

Q

Q

G

F

K

K

V

L

A

A

D

D

M

M

Y

A

T

L

Q

A

L

L

N

E

A

S

S

A

R

R

A

L

Y

L

L

T

Y

W

A

R

V

A

A

A

Q

M

A

L

C

K

E

D

R

N

G

K

E

K

T

P

A

F

R
x
T

K

K

D

E

A

S

A

A

G

M

V

A

I

K

L

L

Y

A

S

A

A

S

E

E

C

A

A

A

T

T

R

A

M

I

A

S

L

H

D

Q

A

A

I

I

Q
|
Q

I
|
I

L

L

G
|
G

N

M

G

G

Y
|
Y

I

V

N

T

E

E

F

M

P

P

A

A

G

E

R

R

L

Y

R

R

D

D

A

A

K

R

L
x
I

Y

T

E

E

I

I

G
x
Y

A
x
E

G
|
G

T
|
T

S

S

E
|
E

I
|
I

R

Q

R

R

M
x
L

L

V

I

I

G

A

R
x
G

E

H

L

L

F

L

N

R

E

S

T

Y

R

R

active site: G377 (≠ R380)
binding acetoacetyl-coenzyme a: L95 (= L98), F125 (≠ L128), L127 (≠ M130), G133 (= G136), S134 (= S137), A136 (≠ V139), K181 (≠ H184), F234 (≠ V237), K235 (= K238), M238 (= M241), Q239 (≠ S242), L241 (= L244), D242 (= D245), R245 (= R248), T315 (≠ R318), Y364 (≠ G367), E365 (≠ A368), G366 (= G369), I370 (= I373)
binding flavin-adenine dinucleotide: F125 (≠ L128), A126 (= A129), L127 (≠ M130), S128 (= S131), G133 (= G136), S134 (= S137), W158 (= W161), I159 (= I162), T160 (= T163), K203 (= K206), T211 (= T214), R270 (= R273), F273 (= F276), L277 (≠ I280), L280 (≠ F283), I283 (= I286), Q338 (= Q341), I339 (= I342), G341 (= G344), G342 (= G345), Y345 (= Y348), I360 (≠ L363), Y364 (≠ G367), T367 (= T370), E369 (= E372), I370 (= I373), L373 (≠ M376)

P15651 Short-chain specific acyl-CoA dehydrogenase, mitochondrial; SCAD; Butyryl-CoA dehydrogenase; EC 1.3.8.1 from Rattus norvegicus (Rat) (see 2 papers)
41% identity, 98% coverage: 10:387/387 of query aligns to 34:411/412 of P15651

query
sites
P15651

L

L

G

P

E

E

T

T

I

H

D

Q

M

M

L

L

R

R

D

Q

Q

T

V

C

Q

R

S

D

F

F

V

A

A

E

K

K

E

E

I

L

A

V

P

P

R

I

A

A

Q

Q

I

L

D

D

I

K

D

E

N

H

L

L

F

F

P

P

A

T

D

S

L

Q

W

V

R

K

K

K

F

M

G

G

D

E

M

L

G

G

L

L

G

A

I

M

T

D

V

V

P

P

E

E

Y

L

G

S

G

G

A

A

G

G

L

L

G

D

Y

Y

L

L

A

A

H

Y

V

S

V

I

A

A

M

L

E

E

I

I

S

S

R

R

G

G

S

C

A

A

S

S

V

T

A

G

L

V

S

I

Y

M

G

S

A

V

H

N

S

N

N

S

L

L

C

Y

V

L

N

G

Q

P

I

I

N

L

R

K

N

F

G

G

T

S

H

S

E

Q

Q

Q

K

K

S

Q

K

Q

Y

W

L

I

P

T

K

P

L

F

I

T

S

N

G

G

E

D

H

K

I

I

G

G

A

C

L
x
F

A
|
A

M
x
L

S
|
S

E
|
E

P
|
P

N
x
G

A
x
N

G
|
G

S
|
S

D

D

V

A

V

G

S

A

M

A

K

S

L

T

R

T

A

A

D

R

K

E

R

E

G

G

D

D

H

S

Y

W

V

V

L

L

N

N

G

G

S

T

K

K

T

A

W
|
W

I
|
I

T
|
T

N

N

G

S

P

W

D

E

A

A

N

S

T

A

Y

T

V

V

I

V

Y

F

A

A

K

S

T

T

D

D

L

R

E

S

K

R

G

Q

P

N

H

K

G

G

I

I

T

S

A

A

F

F

I

L

V

V

E

P

R

M

D

P

S

T

K

P

G

G

F

L

S

T

R

L

S

G

N

K

K

K

F

E

D

D

K

K

L

L

G

G

M

I

R

R

G

A

S

S

N

S

T

T

C

A

E

N

L

L

F

I

F

F

D

E

D

D

V

C

E

R

V

I

P

P

E

K

E

E

N

N

I

L

L

L

G

G

V

E

L

P

N

G

G

M

G

G

V

F

K

K

V

I

L

A

M

M

S

Q

G

T

L

L

D
|
D

Y
x
M

E
x
G

R
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R

V

I

V

G

L

I

S

A

G

S

G

Q

P

A

T

L

G

G

I

I

M

A

Q

Q

A

A

C

S

M

L

D

D

L

C

I

A

V

V

P

K

Y

Y

I

A

H

E

D

N

R
|
R

K

H

Q

A

F

F

G

G

Q

A

S

P

I

L

G

T

E

K

F

L

Q

Q

L

N

I

I

Q

Q

G

F

K

K

V

L

A

A

D

D

M

M

Y

A

T

L

Q

A

L

L

N

E

A

S

S

A

R

R

A

L

Y

L

L

T

Y

W

A

R

V

A

A

A

Q

M

A

L

C

K

E

D

R

N

G

K

E

K

T

P

A

F

R

T

K

K

D

E

A

S

A

A

G

M

V

A

I

K

L

L

Y

A

S

A

A

S

E

E

C

A

A

A

T

T

R

A

M

I

A

S

L

H

D

Q

A

A

I

I

Q
|
Q

I
|
I

L
|
L

G
|
G

N

M

G

G

Y

Y

I

V

N

T

E

E

F

M

P

P

A

A

G

E

R

R

L

Y

R

R

D

D

A

A

K

R

L

I

Y

T

E

E

I

I

G

Y

A
x
E

G

G

T
|
T

S
|
S

E
|
E

I

I

R

Q

R

R

M

L

L

V

I

I

G

A

R

G

E

H

L

L

F

L

N

R

E

S

T

Y

R

R

152:161 (vs. 128:137, 60% identical) binding
S161 (= S137) binding
WIT 185:187 (= WIT 161:163) binding
DMGR 269:272 (≠ DYER 245:248) binding
R297 (= R273) binding
QILGG 365:369 (= QILGG 341:345) binding
E392 (≠ A368) active site, Proton acceptor
TSE 394:396 (= TSE 370:372) binding

Sites not aligning to the query:

1:24 modified: transit peptide, Mitochondrion

P16219 Short-chain specific acyl-CoA dehydrogenase, mitochondrial; SCAD; Butyryl-CoA dehydrogenase; EC 1.3.8.1 from Homo sapiens (Human) (see 3 papers)
42% identity, 98% coverage: 10:387/387 of query aligns to 34:411/412 of P16219

query
sites
P16219

L

L

G

P

E

E

T

T

I

H

D

Q

M

M

L

L

R

L

D

Q

Q

T

V

C

Q

R

S

D

F

F

V

A

A

E

K

K

E

E

I

L

A

F

P

P

R

I

A

A

Q

Q

I

V

D

D

I

K

D

E

N

H

L

L

F

F

P

P

A

A

D

A

L

Q

W

V

R

K

K

K

F

M

G

G

D

G

M

L

G

G

L

L

G

A

I

M

T

D

V

V

P

P

E

E

Y

L

G

G

G
|
G

A

A

G

G

L

L

G

D

Y

Y

L

L

A

A

H

Y

V

A

V

I

A

A

M

M

E

E

E
|
E

I

I

S

S

R

R

G

G

S

C

A

A

S

S

V

T

A

G

L

V

S

I

Y

M

G

S

A

V

H

N

S

N

N

S

L

L

C

Y

V

L

N

G

Q

P

I

I

N

L

R

K

N

F

G

G

T

S

H

K

E

E

Q

Q

K

K

S

Q

K

A

Y

W

L

V

P

T

K

P

L

F

I

T

S

S

G

G

E

D

H

K

I

I

G

G

A

C

L
x
F

A
|
A

M
x
L

S
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S

E
|
E

P
|
P

N
x
G

A
x
N

G
|
G

S
|
S

D

D

V

A

V

G

S

A

M

A

K

S

L

T

R

T

A

A

D
x
R

K

A

R

E

G

G

D

D

H

S

Y

W

V

V

L

L

N

N

G

G

S

T

K

K

T

A

W
|
W

I
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I

T
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T

N

N

G

A

P

W

D

E

A
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A

N

S

T

A

Y

A

V

V

I

V

Y

F

A

A

K

S

T

T

D

D

L

R

E

A

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G

I

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A

A

F

F

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L

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V

E

P

R

M

D

P

S

T

K

P

G

G

F

L

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T

R

L

S

G

N

K

K

K

F

E

D

D

K

K

L

L

G

G

M

I

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R

G

G

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S

N

S

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C

A

E

N

L

L

F

I

F

F

D

E

D

D

V

C

E

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V

I

P

P

E

K

E

D

N

S

I

I

L

L

G

G

V

E

L

P

N

G

G

M

G

G

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F

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K

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I

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A

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M

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L

L

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Y

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A

G

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L

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I

M

A

Q

Q

A

T

C

A

M

L

D

D

L

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A

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V

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N

Y

Y

I

A

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R
|
R

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M

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A

F

F

G

G

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A

S

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I

L

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E

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F

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Q

L

V

I

I

Q

Q

G

F

K

K

V

L

A

A

D

D

M

M

Y

A

T

L

Q

A

L

L

N

E

A

S

S

A

R
|
R

A

L

Y

L

L

T

Y

W

A

R

V

A

A

A

Q

M

A

L

C

K

E

D

R

N

G

K

E

K

T

P

A

F

R

I

K

K

D

E

A

A

G

M

V

A

I

K

L

L

Y

A

S

A

A
x
S

E

E

C

A

A

A

T

T

R

A

M

I

A

S

L

H

D

Q

A

A

I

I

Q
|
Q

I
|
I

L
|
L

G
|
G

N

M

G

G

Y

Y

I

V

N

T

E

E

F

M

P

P

A

A

G

E

R
|
R

L

H

L

Y

R

R

D

D

A

A

K

R

L

I

Y

T

E

E

I

I

G

Y

A

E

G

G

T
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T

S
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S

E
|
E

I

I

R

Q

R

R

M

L

L

V

I

I

G

A

R

G

E

H

L

L

F

L

N

R

E

S

T

Y

R

R

G90 (= G66) to S: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs121908005
E104 (= E80) natural variant: Missing (in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs387906308)
152:161 (vs. 128:137, 60% identical) binding in other chain
R171 (≠ D147) to W: 69% of wild-type acyl-CoA dehydrogenase activity; confers susceptibility to ethylmalonicaciduria; dbSNP:rs1800556
WIT 185:187 (= WIT 161:163) binding in other chain
A192 (= A168) to V: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs28940874
G209 (= G185) to S: 86% of wild-type acyl-CoA dehydrogenase activity; confers susceptibility to ethylmalonicaciduria; dbSNP:rs1799958
R297 (= R273) binding
Q308 (= Q284) binding in other chain
R325 (= R301) to W: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs121908006
S353 (≠ A329) to L: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs28941773
QILGG 365:369 (= QILGG 341:345) binding
R380 (= R356) to W: in ACADSD; loss of acyl-CoA dehydrogenase activity; dbSNP:rs28940875
TSE 394:396 (= TSE 370:372) binding in other chain

Sites not aligning to the query:

1:24 modified: transit peptide, Mitochondrion

7y0bA Short-chain specific acyl-CoA dehydrogenase, mitochondrial
42% identity, 98% coverage: 10:387/387 of query aligns to 7:384/385 of 7y0bA

query
sites
7y0bA

L

L

G

P

E

E

T

T

I

H

D

Q

M

M

L

L

R

L

D

Q

Q

T

V

C

Q

R

S

D

F

F

V

A

A

E

K

K

E

E

I

L

A

F

P

P

R

I

A

A

Q

Q

I

V

D

D

I
x
K

D

E

N

H

L

L

F

F

P

P

A

A

D

A

L

Q

W

V

R

K

K

K

F

M

G

G

D

G

M

L

G

G

L

L

G

A

I

M

T

D

V

V

P

P

E

E

Y

L

G

G

A

A

G

G

L

L

G

D

Y

Y

L

L

A

A

H

Y

V

A

V

I

A

A

M

M

E

E

I

I

S

S

R

R

G

G

S

C

A

A

S

S

V

T

A

G

L

V

S

I

Y

M

G

S

A

V

H

N

S

N

N

S

L

L

C

Y

V

L

N

G

Q

P

I

I

N

L

R

K

N

F

G

G

T

S

H

K

E

E

Q

Q

K

K

S

Q

K

A

Y

W

L

V

P

T

K

P

L

F

I

T

S

S

G

G

E

D

H

K

I

I

G

G

A

C

L
x
F

A

A

M
x
L

S
|
S

E

E

P

P

N

G

A

N

G
|
G

S
|
S

D

D

V

A

V

G

S

A

M

A

K

S

L

T

R

T

A

A

D

R

K

A

R

E

G

G

D

D

H

S

Y

W

V

V

L

L

N

N

G

G

S

T

K

K

T

A

W
|
W

I
|
I

T
|
T

N

N

G

A

P

W

D

E

A

A

N

S

T

A

Y

A

V

V

I

V

Y

F

A

A

K

S

T

T

D

D

L

R

E

A

K

L

G

Q

P

N

H

K

G

G

I

I

T

S

A

A

F

F

I

L

V

V

E

P

R

M

D

P

S

T

K

P

G

G

F

L

S

T

R

L

S

G

N

K

K

K

F

E

D

D

K

K

L

L

G

G

M

I

R

R

G

G

S

S

N

S

T
|
T

C

A

E

N

L

L

F

I

F

F

D

E

D

D

V

C

E

R

V

I

P

P

E

K

E

D

N

S

I

I

L

L

G

G

V

E

L

P

N

G

G

M

G

G

V

F

K

K

V

I

L

A

M

M

S

Q

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T

L

L

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D

Y

M

E

G

R

R

V

I

V

G

L

I

S

A

G

S

G

Q

P

A

T

L

G

G

I

I

M

A

Q

Q

A

T

C

A

M

L

D

D

L

C

I

A

V

V

P

N

Y

Y

I

A

H

E

D

N

R
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R

K

M

Q

A

F
|
F

G

G

Q

A

S

P

I
x
L

G

T

E

K

F
x
L

Q

Q

L
x
V

I
|
I

Q

Q

G

F

K

K

V

L

A

A

D

D

M

M

Y

A

T

L

Q

A

L

L

N

E

A

S

S

A

R

R

A

L

Y

L

L

T

Y

W

A

R

V

A

A

A

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M

A

L

C

K

E

D

R

N

G

K

E

K

T

P

A

F

R

I

K

K

D

E

A

A

G

M

V

A

I

K

L

L

Y

A

S

A

A

S

E

E

C

A

A

A

T

T

R

A

M

I

A

S

L

H

D

Q

A

A

I

I

Q
|
Q

I
|
I

L

L

G
|
G

N
x
M

G

G

Y
|
Y

I

V

N
x
T

E
|
E

F

M

P

P

A

A

G

E

R

R

L

H

L

Y

R

R

D

D

A

A

K

R

L
x
I

Y

T

E

E

I
|
I

G
x
Y

A

E

G

G

T
|
T

S

S

E
|
E

I
|
I

R

Q

R

R

M
x
L

L

V

I

I

G

A

R

G

E

H

L

L

F

L

N

R

E

S

T

Y

R

R

binding (2S,3R,4R,5S,6R)-2-[4-chloranyl-3-[[4-[(3S)-oxolan-3-yl]oxyphenyl]methyl]phenyl]-6-(hydroxymethyl)oxane-3,4,5-triol: K35 (≠ I38), M343 (≠ N346), T347 (≠ N350), E348 (= E351)
binding flavin-adenine dinucleotide: F125 (≠ L128), L127 (≠ M130), S128 (= S131), G133 (= G136), S134 (= S137), W158 (= W161), I159 (= I162), T160 (= T163), T211 (= T214), R270 (= R273), F273 (= F276), L277 (≠ I280), L280 (≠ F283), V282 (≠ L285), I283 (= I286), Q338 (= Q341), I339 (= I342), G341 (= G344), G342 (= G345), Y345 (= Y348), I360 (≠ L363), I363 (= I366), Y364 (≠ G367), T367 (= T370), E369 (= E372), I370 (= I373), L373 (≠ M376)

7y0aC Short-chain specific acyl-CoA dehydrogenase, mitochondrial
42% identity, 98% coverage: 10:387/387 of query aligns to 10:387/387 of 7y0aC

query
sites
7y0aC

L

L

G

P

E

E

T

T

I

H

D

Q

M

M

L

L

R

L

D

Q

Q

T

V

C

Q

R

S

D

F

F

V

A

A

E

K

K

E

E

I

L

A

F

P

P

R

I

A

A

Q

Q

I

V

D

D

I

K

D

E

N

H

L

L

F

F

P

P

A

A

D

A

L

Q

W

V

R

K

K

K

F

M

G

G

D

G

M

L

G

G

L

L

G

A

I

M

T

D

V

V

P

P

E

E

Y

L

G

G

A

A

G

G

L

L

G

D

Y

Y

L

L

A

A

H

Y

V

A

V

I

A

A

M

M

E

E

I

I

S

S

R

R

G

G

S

C

A

A

S

S

V

T

A

G

L

V

S

I

Y

M

G

S

A

V

H

N

S

N

N

S

L

L

C

Y

V

L

N

G

Q

P

I

I

N

L

R

K

N

F

G

G

T

S

H

K

E

E

Q

Q

K

K

S

Q

K

A

Y

W

L

V

P

T

K

P

L

F

I

T

S

S

G

G

E

D

H

K

I

I

G

G

A

C

L
x
F

A

A

M
x
L

S
|
S

E

E

P

P

N

G

A

N

G
|
G

S
|
S

D

D

V

A

V

G

S

A

M

A

K

S

L

T

R

T

A

A

D

R

K

A

R

E

G

G

D

D

H

S

Y

W

V

V

L

L

N

N

G

G

S

T

K

K

T
x
A

W
|
W

I
|
I

T
|
T

N

N

G

A

P

W

D

E

A

A

N

S

T

A

Y

A

V

V

I

V

Y

F

A

A

K

S

T

T

D

D

L

R

E

A

K

L

G

Q

P

N

H

K

G

G

I

I

T

S

A

A

F

F

I

L

V

V

E

P

R

M

D

P

S

T

K

P

G

G

F

L

S

T

R

L

S

G

N

K

K

K

F

E

D

D

K
|
K

L

L

G

G

M

I

R

R

G

G

S

S

N

S

T
|
T

C

A

E

N

L

L

F

I

F

F

D

E

D

D

V

C

E

R

V

I

P

P

E

K

E

D

N

S

I

I

L

L

G

G

V

E

L

P

N

G

G

M

G

G

V

F

K

K

V

I

L

A

M

M

S

Q

G

T

L

L

D

D

Y

M

E

G

R

R

V

I

V

G

L

I

S

A

G

S

G

Q

P

A

T

L

G

G

I

I

M

A

Q

Q

A

T

C

A

M

L

D

D

L

C

I

A

V

V

P

N

Y

Y

I

A

H

E

D

N

R
|
R

K

M

Q
x
A

F
|
F

G

G

Q

A

S

P

I
x
L

G

T

E

K

F
x
L

Q

Q

L
x
V

I
|
I

Q

Q

G

F

K

K

V

L

A

A

D

D

M

M

Y

A

T

L

Q

A

L

L

N

E

A

S

S

A

R

R

A

L

Y

L

L

T

Y

W

A

R

V

A

A

A

Q

M

A

L

C

K

E

D

R

N

G

K

E

K

T

P

A

F

R

I

K

K

D

E

A

A

G

M

V

A

I

K

L

L

Y

A

S

A

A

S

E

E

C

A

A

A

T

T

R

A

M

I

A

S

L

H

D

Q

A

A

I

I

Q
|
Q

I
|
I

L

L

G
|
G

N

M

G

G

Y
|
Y

I

V

N

T

E

E

F

M

P

P

A

A

G

E

R

R

L

H

L

Y

R

R

D

D

A

A

K

R

L
x
I

Y

T

E

E

I
|
I

G
x
Y

A

E

G
|
G

T
|
T

S

S

E
|
E

I
|
I

R

Q

R

R

M
x
L

L

V

I

I

G

A

R

G

E

H

L

L

F

L

N

R

E

S

T

Y

R

R

binding flavin-adenine dinucleotide: F128 (≠ L128), L130 (≠ M130), S131 (= S131), G136 (= G136), S137 (= S137), A160 (≠ T160), W161 (= W161), I162 (= I162), T163 (= T163), K206 (= K206), T214 (= T214), R273 (= R273), A275 (≠ Q275), F276 (= F276), L280 (≠ I280), L283 (≠ F283), V285 (≠ L285), I286 (= I286), Q341 (= Q341), I342 (= I342), G344 (= G344), G345 (= G345), Y348 (= Y348), I363 (≠ L363), I366 (= I366), Y367 (≠ G367), G369 (= G369), T370 (= T370), E372 (= E372), I373 (= I373), L376 (≠ M376)

4l1fA Electron transferring flavoprotein of acidaminococcus fermentans: towards a mechanism of flavin-based electron bifurcation (see paper)
39% identity, 98% coverage: 6:385/387 of query aligns to 1:380/380 of 4l1fA

query
sites
4l1fA

L

M

N

D

F

F

A

N

L
|
L

G
x
T

E

E

T

D

I

Q

D
x
Q

M

M

L

I

R

K

D

D

Q

M

V

A

Q

A

S

E

F

F

V

A

A

E

K

K

E

F

I

L

A

A

P

P

R

T

A

V

A

E

Q

E

I

R

D

D

I

K

D

A

N

H

L

I

F

W

P

D

A

R

D

K

L

L

W

I

R

D

K

K

F

M

G

G

D

E

M

A

G

G

L

F

L

C

G

G

I

I

T

C

V

F

P

P

E

E

Y

Y

G

G

A

M

G

G

L

L

G

D

Y

V

L

L

A

S

H

Y

V

I

V

L

A

A

M

V

E

E

I

L

S

S

R

K

G

V

S

D

A

D

S

G

V

T

A

G

L

I

S

T

Y

L

G

S

A

A

H

N

S

V

N

S

L

L

C

C

V

A

N

T

Q

P

I

I

N

Y

R

M

N

F

G

G

T

T

H

E

E

E

Q

Q

K

K

S

Q

K

K

Y

Y

L

L

P

A

K

P

L

I

I

A

S

E

G

G

E

T

H

H

I

V

G

G

A

A

L
x
F

A

G

M
x
L

S
x
T

E

E

P

P

N

S

A

A

G
|
G

S
x
T

D

D

V
x
A

V

S

S

A

M

Q

K

Q

L

T

R

T

A

A

D

V

K

L

R

K

G

G

D

D

H

K

Y

Y

V

I

L

L

N

N

G

G

S

S

K

K

T

I

W
x
F

I
|
I

T
|
T

N

N

G

G

P

K

D

E

A

A

N

D

T

T

Y

Y

V

V

I

V

Y

F

A

A

K

M

T

T

D

D

L

K

E

S

K

Q

G

G

P
x
V

H
|
H

G

G

I

I

T

S

A

A

F

F

I

I

V

L

E

E

R

K

D

G

S

M

K

P

G

G

F

F

S

R

R

F

S

G

N

K

K

I

F

E

D

D

K

K

L

M

G

G

M

G

R

H

G

T

S

S

N

I

T
|
T

C

A

E

E

L

L

F

I

F

F

D

E

D

D

V

C

E

E

V

V

P

P

E

K

E

E

N

N

I

L

L

L

G

G

V

K

L

E

N

G

G

E

G

G

V
x
F

K

K

V

I

L

A

M
|
M

S
x
E

G

T

L
|
L

D
|
D

Y

G

E
x
G

R
|
R

V

I

V

G

L

V

S

A

G

A

G

Q

P

A

T

L

G

G

I

I

M

A

Q

E

A

G

C

A

M

L

D

A

L

A

I

A

V

V

P

K

Y

Y

I

S

H

K

D

E

R
|
R

K

E

Q
|
Q

F
|
F

G

G

Q

R

S

S

I
|
I

G

S

E

K

F
|
F

Q

Q

L

A

I
x
L

Q

Q

G

F

K

M

V

M

A

A

D

D

M

M

Y

A

T

T

Q

K

L

I

N

E

A

A

S

A

R

R

A

Y

Y

L

L

V

Y

Y

A

H

V

A

A

A

Q

M

A

L

C

K

E

N

R

E

G

G

E

K

T

P

A

Y

R
x
S

K

E

D

A

A

A

G

M

V

A

I

K

L

C

Y

F

S

A

A

S

E

D

C

V

A

A

T

M

R

E

M

V

A

T

L

T

D

D

A

A

I

V

Q
|
Q

I
|
I

L

F

G
|
G

N
x
Y

G

G

Y

Y

I

T

N

V

E

D

F

Y

P

P

A

A

G

E

R

R

L

Y

L

M

R

R

D

N

A

A

K

K

L
x
I

Y

T

E

Q

I

I

G
x
Y

A
x
E

G
|
G

T
|
T

S

N

E
x
Q

I
x
V

R

M

R

R

M
x
I

L

V

I

T

G

S

R
|
R

E

A

L

L

F

L

N

R

E

D

active site: L125 (≠ M130), T126 (≠ S131), G242 (≠ E247), E363 (≠ A368), R375 (= R380)
binding coenzyme a persulfide: F123 (≠ L128), T132 (≠ S137), A134 (≠ V139), V178 (≠ P183), H179 (= H184), F232 (≠ V237), M236 (= M241), E237 (≠ S242), L239 (= L244), D240 (= D245), R243 (= R248), S313 (≠ R318), Y362 (≠ G367), E363 (≠ A368), G364 (= G369), V368 (≠ I373), R375 (= R380)
binding flavin-adenine dinucleotide: F123 (≠ L128), L125 (≠ M130), T126 (≠ S131), G131 (= G136), T132 (≠ S137), F156 (≠ W161), I157 (= I162), T158 (= T163), T209 (= T214), R268 (= R273), Q270 (= Q275), F271 (= F276), I275 (= I280), F278 (= F283), L281 (≠ I286), Q336 (= Q341), I337 (= I342), G339 (= G344), G340 (= G345), Y341 (≠ N346), I358 (≠ L363), Y362 (≠ G367), T365 (= T370), Q367 (≠ E372), I371 (≠ M376)
binding 1,3-propandiol: L5 (= L10), T6 (≠ G11), Q10 (≠ D15)

2vigB Crystal structure of human short-chain acyl coa dehydrogenase
42% identity, 98% coverage: 10:387/387 of query aligns to 1:371/371 of 2vigB

query
sites
2vigB

L

L

G

P

E

E

T

T

I

H

D

Q

M

M

L

L

R

L

D

Q

Q

T

V

C

Q

R

S

D

F

F

V

A

A

E

K

K

E

E

I

L

A

F

P

P

R

I

A

A

Q

Q

I

V

D

D

I

K

D

E

N

H

L

L

F

F

P

P

A

A

D

A

L

Q

W

V

R

K

K

K

F

M

G

G

D

G

M

L

G

G

L

L

G

A

I

M

T

D

V

V

P

P

E

E

Y

L

G

G

A

A

G

G

L

L

G

D

Y

Y

L

L

A

A

H

Y

V

A

V

I

A

A

M

M

E

E

I

I

S

S

R

R

G

G

S

C

A

A

S

S

V

T

A

G

L

V

S

I

Y

M

G

S

A

V

H

N

S

N

N

S

L

L

C

Y

V

L

N

G

Q

P

I

I

N

L

R

K

N

F

G

G

T

S

H

K

E

E

Q

Q

K

K

S

Q

K

A

Y

W

L

V

P

T

K

P

L

F

I

T

S

S

G

G

E

D

H

K

I

I

G

G

A

C

L
x
F

A
|
A

M
x
L

S
|
S

E

E

P

P

N

G

A

N

G
|
G

S
|
S

D

D

V

A

V

G

S

A

M

A

K

S

L

T

R

T

A

A

D

R

K

A

R

E

G

G

D

D

H

S

Y

W

V

V

L

L

N

N

G

G

S

T

K

K

T
x
A

W
|
W

I
|
I

T
|
T

N

N

G

A

P

W

D

E

A

A

N

S

T

A

Y

A

V

V

I

V

Y

F

A

A

K

S

T

T

D

S

L

-

E

-

K

-

G

-

P

-

H

-

G

-

I

I

T

S

A

A

F

F

I

L

V

V

E

P

R

M

D

P

S

T

K

P

G

G

F

L

S

T

R

L

S

G

N

K

K

K

F

E

D

D

K
|
K

L

L

G

G

M

I

R

R

G

G

S

S

N

S

T
|
T

C

A

E

N

L

L

F

I

F

F

D

E

D

D

V

C

E

R

V

I

P

P

E

K

E

D

N

S

I

I

L

L

G

G

V

E

L

P

N

G

G

M

G

G

V
x
F

K
|
K

V

I

L

A

M
|
M

S
x
Q

G

T

L
|
L