SitesBLAST

6fogA X-ray structure of homo sapiens fumarylacetoacetate hydrolase domain containing protein 1 (fahd1) in complex with inhibitor oxalate at 1.94a resolution. (see paper)
41% identity, 65% coverage: 75:263/293 of query aligns to 13:192/218 of 6fogA

query
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6fogA

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binding magnesium ion: K13 (≠ R75), N14 (= N76), E66 (= E141), E68 (= E143), D97 (= D172)
binding oxalate ion: V18 (= V80), G19 (= G81), R20 (≠ K82), H25 (= H87), F40 (= F114), E66 (= E141), E68 (= E143), K118 (= K189), G186 (= G257), T187 (= T258)

Q6P587 Acylpyruvase FAHD1, mitochondrial; Fumarylacetoacetate hydrolase domain-containing protein 1; FAH domain-containing protein 1; Oxaloacetate decarboxylase; OAA decarboxylase; YisK-like protein; EC 3.7.1.5; EC 4.1.1.112 from Homo sapiens (Human) (see 3 papers)
41% identity, 65% coverage: 75:263/293 of query aligns to 18:197/224 of Q6P587

query
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Q6P587

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H30 (= H87) mutation to A: Impaired oxaloacetate decarboxylase activity.
E33 (= E90) mutation to A: Impaired oxaloacetate decarboxylase activity.
E71 (= E141) binding
E73 (= E143) binding
D102 (= D172) binding ; mutation to A: Loss of catalytic activity; when associated with A-106.
R106 (= R176) mutation to A: Loss of catalytic activity; when associated with A-102.

6sbiA X-ray structure of murine fumarylacetoacetate hydrolase domain containing protein 1 (fahd1) in complex with inhibitor oxalate (see paper)
38% identity, 72% coverage: 75:285/293 of query aligns to 12:206/216 of 6sbiA

query
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6sbiA

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active site: S43 (≠ P118), T175 (= T247), V190 (= V262)
binding magnesium ion: E65 (= E141), E67 (= E143), D96 (= D172)
binding oxalate ion: V17 (= V80), G18 (= G81), R19 (≠ K82), H24 (= H87), F39 (= F114), E65 (= E141), E67 (= E143), D96 (= D172), K117 (= K189), G185 (= G257), T186 (= T258)

1gttA Crystal structure of hpce (see paper)
38% identity, 66% coverage: 70:263/293 of query aligns to 218:392/421 of 1gttA

query
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1gttA

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binding calcium ion: E268 (= E141), E270 (= E143), D299 (= D172)

6j5yA Crystal structure of fumarylpyruvate hydrolase from pseudomonas aeruginosa in complex with mn2+ and pyruvate (see paper)
37% identity, 74% coverage: 73:289/293 of query aligns to 22:227/233 of 6j5yA

query
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6j5yA

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binding manganese (ii) ion: E82 (= E141), E84 (= E143), D113 (= D172)
binding pyruvic acid: V29 (= V80), G30 (= G81), R31 (≠ K82), H36 (= H87), F52 (= F114), E82 (= E141), E84 (= E143), D113 (= D172), K134 (= K189), G202 (= G257), T203 (= T258)

1nkqA Crystal structure of yeast ynq8, a fumarylacetoacetate hydrolase family protein
31% identity, 75% coverage: 75:293/293 of query aligns to 9:231/247 of 1nkqA

query
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1nkqA

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K

A

S

N

N

L

L

A

Q

-

D

-

I

-

F

-

R

L

V

R

K

C

C

W

S

V

V

N

N

D

G

E

Q

L

L

R

R

Q

Q

N

D

A

G

N

G

T

T

R

N

D

L

L

M

I

L

F

H

D

P

I

L

P

H

T

K

M

I

I

L

E

Q

T

H

L

I

S

S

A

T

G

M

I

I

T

S

L

L

Y

E

P

P

G

G

D

D

I

I

A

L

T

T

G

G

T

T

P

P

A

A

G

G

V

V

G

G

I

-

G

-

F

-

N

-

P

-

K

-

F

E

L

L

K

K

P

P

G

G

D

D

R

R

V

V

T

H

I

C

E

E

I

L

D

-

G

-

L

L

G

Q

R

N

L

N

S

D

N

N

V

I

L

V

D

D

binding calcium ion: E76 (= E141), E78 (= E143), D110 (= D172)

3v77A Crystal structure of a putative fumarylacetoacetate isomerase/hydrolase from oleispira antarctica (see paper)
32% identity, 74% coverage: 69:285/293 of query aligns to 10:222/224 of 3v77A

query
sites
3v77A

P

P

I

Y

P

P

R

R

P

D

D

L

R

G

N

K

I

I

F

V

C

C

V

V

G

G

K

R

N

N

Y

Y

H

A

D

A

H

H

A

A

H

K

E

E

F

L

T

N

R

-

S

-

G

-

F

-

D

-

A

-

G

-

S

-

K

-

V

-

A

-

T

-

D

N

A

P

I

I

P

P

E

S

A

S

P

P

I

I

F

L

F

F

T

I

K

K

P

P

P

A

E

S

T

S

V

A

I

V

A

P

N

F

G

G

D

P

P

V

I

F

R

S

Y

I

P

P

H

K

G

D

V

-

S

Q

D

G

S

S

L

V

D

H

Y

H

E

E

A

L

E
|
E

L

I

G

A

V

I

V

L

I

I

G

G

K

K

G

A

G

L

R

S

G

R

I

A

T

S

K

T

A

E

E

Q

A

V

Y

A

D

E

H

S

V

I

F

A

G

G

Y

I

V

G

I

L

I

G

N

L

D
|
D

M

L

T

T

A

L

R

R

D

D

W

V

Q

Q

S

D

R

Q

H

L

K

K

Q

E

-

K

-

G

-

H

-

P

W

W

F

E

L

R

G

A

K

K

S

S

F

F

D

D

T

G

F

A

C

C

P

P

M

L

G

T

P

E

W

F

-

V

-

A

-

V

-

N

L

L

A

A

T

S

T

E

D

D

E

E

V

W

D

Q

A

A

A

I

N

G

L

L

A

T

L

L

R

E

C

-

W

-

V

K

N

N

D

G

E

Q

L

F

R

Q

Q

Q

N

Q

A

G

N

S

T

S

R

A

D

E

L

M

I

L

F

F

D

P

I

I

P

L

T

P

M

L

I

I

E

A

T
x
H

L

M

S

S

A

E

G
x
H

I

F

T

S

L

L

Y

Q

P

P

G

G

D

D

I

V

I

I

A

L

T

T

G

G

T

T

P

P

A

A

G

G

V

V

G

G

-

P

-

L

-

E

I

V

G

G

F

D

N

S

P

L

P

S

K

A

F

K

L

L

K

S

P

L

G

E

D

D

R

N

V

V

T

L

I

L

E

T

I

C

D

D

G

G

L

V

binding zinc ion: E71 (= E143), D100 (= D172), H175 (≠ T241), H179 (≠ G245)

5ti1H Crystal structure of fumarylacetoacetate hydrolase from burkholderia xenovorans lb400
34% identity, 36% coverage: 109:213/293 of query aligns to 167:288/430 of 5ti1H

query
sites
5ti1H

E

E

A

M

P

P

I

I

F

G

F

Y

T

N

K

G

P

R

P

A

E

S

T

S

V

V

I

V

A

V

N

S

G

G

D

T

P

P

I

V

R

R

Y

R

P

P

H

N

G

G

-

Q

-

L

-

K

-

L

-

P

-

D

-

Q

-

E

-

R

-

P

-

V

-

F

-

G

V

A

S

C

D

R

S

K

L

L

D

D

Y

I

E
|
E

A

L

E
|
E

L

T

G

G

V

F

V

V

I

I

G

G

K

A

G

G

-

N

-

A

-

L

G

G

R

E

G

P

I

V

T

T

K

C

A

A

E

D

A

A

Y

E

D

A

H

H

V

I

F

F

G

G

Y

M

V

V

I

L

N

N

D
|
D

M

W

T

S

A

A

R
|
R

D

D

W

I

Q
|
Q

S

-

R

-

H

-

K

-

Q

Q

W

W

-

E

-

Y

-

V

-

P

-

L

-

G

-

P

F

F

L

N

G

A

K
|
K

S

T

F

F

D

A

T
|
T

F

-

C

-

P

T

M

I

G

S

P

P

W

W

L

I

A

V

T

T

T

L

D

D

E

A

V

L

D

E

A

P

A

F

N

R

L

V

A

A

active site: E212 (= E141), E214 (= E143), D246 (= D172), R250 (= R176), Q253 (= Q179), K266 (= K189), T270 (= T193)
binding magnesium ion: E212 (= E141), E214 (= E143), D246 (= D172)

Sites not aligning to the query:

active site: 139, 146, 377
binding magnesium ion: 139

P16930 Fumarylacetoacetase; FAA; Beta-diketonase; Fumarylacetoacetate hydrolase; EC 3.7.1.2 from Homo sapiens (Human) (see 14 papers)
28% identity, 52% coverage: 111:261/293 of query aligns to 156:353/419 of P16930

query
sites
P16930

P

P

I

V

F

G

F

Y

T

H

K

G

P

R

P

A

E

S

T

S

V

V

I

V

A

V

N

S

G

G

D

T

P

P

I

I

R

R

Y

R

P

P

-

M

-

G

-

Q

-

M

-

K

-

P

-

D

-

S

-

K

-

P

-

V

H

Y

G

G

V

A

S
x
C

D

K

S

L

L

L

D

D

Y

M

E

E

A

L

E

E

L

M

G

A

V

F

I

V

G

G

K

P

G

G

-

N

-

R

-

L

G

G

R

E

G

P

I

I

T

P

K

I

A

S

E

K

A

A

Y

H

D

E

H

H

V

I

F

F

G

G

Y

M

V

V

I

L

I

M

N

N

D
|
D

M
x
W

T

S

A

A

R

R

D

D

-

I

-

Q

-

K

W

W

Q

E

S

Y

R

V

H

P

K

L

Q

G

W

P

F

F

L

L

G

G

K

K

S

S

F

F

D

G

T

T

F

-

C

-

P

T

M

V

G

S

P

P

W

W

L

V

A

V

T

P

T

M

D

D

E

A

V

L

-

M

-

P

-

F

-

A

-

V

-

P

-

N

-

P

-

K

-
x
Q

D

D

A

P

A

R

N

P

L

L

A

P

L

Y

R

L

C

C

W

-

V

-

N

H

D

D

E

E

-

P

-

Y

-

T

-

F

-

D

-

I

-

N

-

L

-

S

-

V

-

N

-

L

-

K

-

G

-

E

-

G

L

M

R

S

Q

Q

N

A

A

A

N

T

-

I

-

C

T

K

R

S

D

N

L

F

I

K

F

Y

D

M

I

Y

P

W

T

T

M

M

I

L

E

Q

T

Q

L

L

S

T

A

H

-

H

-

S

-

V

-

N

G

G

I

C

T

N

L

L

Y
x
R

P
|
P

G

G

D

D

I

L

A

A

T

S

G

G

T

T

P

I

A

S

G

G

C193 (≠ S135) to R: in TYRSN1; loss of activity
D233 (= D172) to V: in TYRSN1; loss of activity; dbSNP:rs80338897
W234 (≠ M173) to G: in TYRSN1; loss of activity; dbSNP:rs1555441595
Q279 (vs. gap) to R: in TYRSN1; may affect splicing resulting in skipping of exon 8 alone or together with exon 9; lower activity as compared to wild type; dbSNP:rs121965078
R341 (≠ Y249) to W: does not cause a clinically relevant phenotype; results in lower enzyme activity; dbSNP:rs11555096
P342 (= P250) to L: in TYRSN1; loss of activity; dbSNP:rs779040832

Sites not aligning to the query:

1 modified: Initiator methionine, Removed
2 modified: N-acetylserine
16 N → I: in TYRSN1; loss of activity; dbSNP:rs121965073
35 A → T: in TYRSN1; atypical mild phenotype
62 F → C: in TYRSN1; loss of activity
134 A → D: in TYRSN1; loss of activity; dbSNP:rs121965074
381 R → G: in TYRSN1; loss of activity; dbSNP:rs121965077
405 F → H: in TYRSN1; requires 2 nucleotide substitutions

P35505 Fumarylacetoacetase; FAA; Beta-diketonase; Fumarylacetoacetate hydrolase; EC 3.7.1.2 from Mus musculus (Mouse) (see 3 papers)
27% identity, 52% coverage: 111:261/293 of query aligns to 156:353/419 of P35505

query
sites
P35505

P

P

I

V

F

G

F

Y

T

H

K

G

P

R

P

A

E

S

T

S

V

I

I

V

A

V

N

S

G

G

D

T

P

P

I

I

R

R

Y

R

P

P

-

M

-

G

-

Q

-

M

-

R

-

P

-

D

-

N

-

S

-

K

-

P

-

V

H

Y

G

G

V

A

S

C

D

R

S

L

L

L

D

D

Y

M

E
|
E

A

L

E
|
E

L

M

G

A

V

F

I

V

G

G

K

P

G

G

-

N

-

R

-

F

G

G

R

E

G

P

I

I

T

P

K

I

A

S

E

K

A

A

Y

H

D

E

H

H

V

I

F

F

G

G

Y

M

V

V

I

L

I

M

N

N

D
|
D

M

W

T

S

A

A

R

R

D

D

W

I

Q

Q

S

-

R

-

H

-

K

-

Q

Q

W

W

-

E

-

Y

-

V

-

P

-

L

-

G

-

P

F

F

L

L

G

G

K
|
K

S

S

F

F

D

G

T
|
T

F

-

C

-

P

T

M

I

G

S

P

P

W

W

L

V

A

V

T

P

T

M

D

D

E

-

V

-

D

-

A

-

N

-

L

-

A

A

L

L

R

M

C

P

W

F

V

V

N

V

D

P

E

N

L

P

R

K

Q

Q

N

D

-

P

-

K

-

P

-

L

-

P

-

Y

-

L

A

C

N

H

T

S

R

Q

D

P

L

Y

I

T

F

F