SitesBLAST

SitesBLAST – Find functional sites

SitesBLAST

Comparing WP_026608088.1 NCBI__GCF_000427445.1:WP_026608088.1 to proteins with known functional sites using BLASTp with E ≤ 0.001.

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

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

Q9WZ49 ATP-dependent zinc metalloprotease FtsH; EC 3.4.24.- from Thermotoga maritima (strain ATCC 43589 / DSM 3109 / JCM 10099 / NBRC 100826 / MSB8) (see 2 papers)
48% identity, 96% coverage: 5:597/617 of query aligns to 2:597/610 of Q9WZ49

query
sites
Q9WZ49

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G164 (≠ A163) binding ATP
GTGKT 204:208 (= GTGKT 203:207) binding ATP
L209 (= L208) binding ATP
H343 (= H342) binding ATP
E371 (≠ A370) binding ATP
G404 (= G403) mutation to L: Complete loss of protease activity and of oligomerization.
H423 (= H422) binding Zn(2+)
E424 (= E423) active site
H427 (= H426) binding Zn(2+)
D500 (= D500) binding Zn(2+); mutation to A: Complete loss of protease activity.

P37476 ATP-dependent zinc metalloprotease FtsH; Cell division protease FtsH; EC 3.4.24.- from Bacillus subtilis (strain 168) (see paper)
48% identity, 95% coverage: 12:595/617 of query aligns to 9:595/637 of P37476

query
sites
P37476

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K207 (= K206) mutation to N: Does not complement an ftsH deletion, loss of ATPase activity.
E424 (= E423) mutation to Q: Does not complement an ftsH deletion, loss of protease activity against casein.

P0AAI3 ATP-dependent zinc metalloprotease FtsH; Cell division protease FtsH; EC 3.4.24.- from Escherichia coli (strain K12) (see 4 papers)
45% identity, 94% coverage: 12:594/617 of query aligns to 7:586/644 of P0AAI3

query
sites
P0AAI3

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F

F

A

I

W

S

I

W

I

F

P

P

A

M

L

L

F

L

Y

I

L

G

L

V

W

W

V

I

F

F

L

F

F

M

R

R

G

-

F

-

A

-

E

Q

K

M

Q

Q

G

G

L

G

G

G

-

K

-

G

L

A

M

M

T

S

I

F

G

G

K

K

S

S

H

K

A

A

K

R

V

M

Y

L

V

T

E

E

T

D

D

Q

T

I

K

K

V

T

T

T

F

F

K

A

D

D

V

V

A

A

G

G

V

C

D

D

E

E

A

A

K

K

F

E

E

E

L

V

Q

A

E

E

V

L

V

V

S

E

F

Y

L

L

R

R

D

E

P

P

K

S

S

R

F

F

G

Q

R

K

L

L

G

G

A

G

R

K

A

I

P

P

K

K

G

G

I

V

L

L

L

M

V

V

G

G

P

P

G

G

T

T

G

G

K

K

T

T

L

L

L
|
L

A

A

R

K

A

A

V

I

A

A

G

G

E

E

A

A

G

K

V

V

A

P

F

F

S

T

I

I

S

S

G

G

S

S

E

D

F

F

V

V

E

E

M

M

F
|
F

V

V

G
|
G

V

V

G

G

A

A

A

S

R

R

V

V

R

R

D

D

L

M

F

F

E

E

Q

Q

A

A

R

K

K

K

A

A

P

P

C

C

I

I

F

F

I

I

D

D

E

E

L

I

D

D

A

A

L

V

G

G

R

R

S

Q

R

R

S

G

V

A

G

G

G

L

I

G

G

G

V

H

D

D

E

E

K

R

E

E

Q

Q

T

T

L

L

N

N

Q

Q

L

M

L

L

A

V

E

E

L

M

D

D

G

G

F

F

D

E

P

G

S

N

I

E

G

G

V

I

I

I

L

V

L

I

A

A

T
|
T

N
|
N

R

R

P

P

E

D

I

V

L

L

D
|
D

P

P

A

A

L
|
L

L

L

R
|
R

A

P

G

G

R
|
R

F

F

D

D

R

R

Q

Q

V

V

L

V

V

V

D

G

R

L

P

P

D

D

R

V

I

R

G

G

R

R

S

E

E

Q

I

I

L

L

K

K

V

V

H

H

A

M

R

R

K

R

I

V

V

P

L

L

D

A

P

P

A

D

V

I

D

D

L

A

D

A

Q

I

I

I

A

A

G

R

M

G

T

T

T

P

G

G

F

F

T

S

G

G

A

A

D

D

L

L

A

A

N

N

L

L

V

V

N

N

E

E

A

A

V

L

I

F

A

A

T

A

R

R

R

G

K

N

S

K

A

R

A

V

T

V

T

S

A

M

A

V

D

E

F

F

T

E

A

K

A

A

I

K

E

D

R

K

V

I

I

M

A

M

G

G

I

A

E

E

K

R

S

S

R

M

I

V

L

M

S

T

A

E

P

A

E

Q

R

K

R

E

R

S

V

T

A

A

Y

Y

H
|
H

E
|
E

M

A

G

G

H
|
H

A

A

L

I

V

I

A

G

A

R

T

L

L

V

P

P

D

E

V

H

D

D

P

P

V

V

L

H

K

K

V

V

S

T

I

I

P

P

R

R

G

G

V

R

G

-

A

A

L

L

G

G

Y

V

T

T

I

F

Q

F

R

L

P

P

T

E

E

G

D

D

R

A

F

I

V

S

L

A

A

S

A

R

S

Q

E

K

L

L

R
x
E

H

S

R

Q

I

I

T

S

V

T

L

L

L

Y

G

G

R

R

A

L

A

A

E
|
E

S

E

L

I

F

Y

D

G

G

P

D

E

-

H

I

V

S

S

T

T

G

G

A

A

A

S

D

N

D

D

L

I

Q

K

R

V

A

A

T

T

D

N

I

L

A

A

L

R

E

N

M

M

T

V

T

T

L

Q

Y

W

G

G

M

F

A

S

Q

E

T

K

V

L

G

G

Q

P

R

L

T

L

Y

Y

-

A

R

E

A

E

S

E

P

G

Q

E

F

V

F

F

L

L

D

G

Q

R

A

S

Q

V

S

A

R

K

D

A

K

K

P
x
H

A

M

A

S

S

D

E

E

A

-

T

T

L

A

R

R

E

I

I

I

D

D

V

Q

S

E

V

V

R

K

D

A

I

L

V

I

S

E

E

R

A

N

F

Y

I

N

E

R

A

A

R

R

D

Q

I

L

L

L

K

T

R

D

R

N

R

M

A

D

D

I

L

L

D

H

K

A

G

M

A

K

E

D

L

A

L

L

L

M

E

K

K

Y

E
|
E

A

T

I

I

T

D

A

A

L201 (= L209) mutation to N: No in vivo protease activity, no in vitro ATPase activity.
F225 (= F233) mutation F->A,D,E,G,N,Q,R,S,T: Does not complement ftsH1 at 42 degrees Celsius, no protease activity in vivo.; mutation F->C,H: Partially complements ftsH1 at 42 degrees Celsius, some protease activity in vivo.; mutation F->I,L,M,V,W,Y: Complements ftsH1 at 42 degrees Celsius, restores protease activity in vivo.
G227 (= G235) mutation to A: Does not complement ftsH1 at 42 degrees Celsius, no protease activity in vivo.
T297 (= T305) mutation to A: Low protease activity in vivo, low ATPase activity in vitro, complements ftsH1 at 42 degrees Celsius.
N298 (= N306) mutation to A: No in vivo protease activity.
D304 (= D312) mutation D->A,N: No in vivo protease activity, no in vitro ATPase activity; probably still binds ATP.; mutation to E: Low protease activity in vivo, low ATPase activity in vitro, complements ftsH1 at 42 degrees Celsius.
L307 (= L315) mutation to A: Low protease activity in vivo.
R309 (= R317) mutation R->A,L,K: No in vivo protease activity, no ATPase activity in vitro; probably still binds ATP.
R312 (= R320) mutation R->A,L,K: No in vivo protease activity, no ATPase activity in vitro; probably still binds ATP.
H414 (= H422) mutation to Y: Loss of protease function.
E415 (= E423) mutation to Q: Loss of protease activity in vivo.
H418 (= H426) mutation to Y: In tolZ21; loss of protease function in vivo, retains about 25% ATPase activity, temperature sensitive.
E463 (≠ R472) mutation to K: In ftsH1; a temperature-sensitive mutant which increases the frequency of lysogenization of phage lambda; when associated with A-587.
E476 (= E485) mutation E->D,K,V: Severe loss of protease function that is restored by excess Zn.; mutation to Q: Little effect on protease function.
H536 (≠ P543) mutation to R: In hflB29; increases the frequency of lysogenization of phage lambda.
E582 (= E590) mutation E->D,K,Q: No effect on protease function.; mutation to V: Decreased protease function.

3kdsE Apo-ftsh crystal structure (see paper)
53% identity, 72% coverage: 156:597/617 of query aligns to 2:421/427 of 3kdsE

query
sites
3kdsE

K

R

V

V

T

T

F

F

K

K

D

D

V

V

A

G

G

G

V

A

D

E

E

E

A

A

K

I

F

E

E

E

L

L

Q

K

E

E

V

V

S

E

F

F

L

L

R

K

D

D

P

P

K

S

S

K

F

F

G

N

R

R

L

I

G

G

A

A

R

R

A

M

P

P

K

K

G

G

I

I

L

L

V

V

G

G

P

P

G

G

T

T

G

G

K

A

T

T

L

L

A

A

R

R

A

A

V

V

A

A

G

G

E

E

A

A

G

N

V

V

A

P

F

F

S

H

I

I

S

S

G

G

S

S

E

D

F

F

V

V

E

E

M

L

F

F

V

V

G

G

V

V

G

G

A

A

R

R

V

V

R

R

D

D

L

L

F

F

E

A

Q

Q

A

A

R

K

K

A

A

H

A

A

P

P

C

C

I

I

I

V

F

F

I

I

D

D

E

E

L

I

D

D

A

A

L

V

G

G

R

R

S

H

R

-

S

-

V

-

G

-

I

-

G

-

V

-

D

D

E

E

K

R

E

E

Q

Q

T

T

L

L

N

N

Q

Q

L

L

A

V

E

E

L

M

D

D

G

G

F

F

D

D

P

S

S

K

I

E

G

G

V

I

I

I

L

V

L

M

A

A

T

T

N

N

R

R

P

P

E

D

I

I

L

L

D

D

P

P

A

A

L

L

R

R

A

P

G

G

R

R

F

F

D

D

R

K

Q

K

V

I

L

V

V

V

D

D

R

P

P

P

D

D

R

M

I

L

G

G

R

R

S

K

E

K

I

I

L

L

K

E

V

I

H

H

A

T

R

R

K

N

I

K

V

P

L

L

D

A

P

E

A

D

V

V

D

N

L

L

D

E

Q

I

I

I

A

A

G

K

M

R

T

T

T

P

G

G

F

F

T

V

G

G

A

A

D

D

L

L

A

E

N

N

L

L

V

V

N

N

E

E

A

A

V

L

I

L

A

A

T

A

R

R

R

E

K

G

S

R

A

D

A

K

T

I

T

T

A

M

A

K

D

D

F

F

T

E

A

E

A

A

I

I

E

D

R

R

V

V

I

I

A

A

G

G

I

P

E

A

K

R

K

K

S

S

R

L

I

L

L

I

S

S

A

P

P

A

E

E

R

K

R

R

R

I

V

I

A

A

Y

Y

H
|
H

E
|
E

M

A

G

G

H
|
H

A

A

L

V

V

V

A

S

A

T

T

V

L

V

P

P

D

N

V

G

D

E

P

P

V

V

L

H

K

R

V

I

S

S

I

I

P

P

R

R

G

G

V

Y

G
x
K

A

A

L
|
L

G

G

Y

Y

T

T

I

L

Q

H

R

L

P

P

T

E

E

E

D

D

R

K

F

Y

V

L

L

V

A

S

A

R

S

N

E

E

L

L

R

L

H

D

R

K

I

L

T

T

V

A

L

L

G

G

R

R

A

A

E

E

S

E

L

V

F

F

D

-

G

G

D

D

I

V

S

T

T
x
S

G

G

A

A

A
|
A

D

N

D
|
D

L

I

Q

E

R

R

A

A

T

T

D

E

I

I

A

A

L

R

E

N

M

M

T

V

T

C

L

Q

Y

L

G

G

M

M

A

S

Q

E

T

E

V

L

G

G

Q

P

R

L

T

A

Y

W

R

G

A

K

S

R

P

L

Q

R

F

N

F

Y

L

S

D

E

Q

E

A

V

Q

A

S

S

R

K

D

-

K

-

P

-

A

-

S

-

E

-

A

-

T

-

L

-

R

-

E

-

I

I

D

D

V

E

S

E

V

V

R

K

D

K

I

I

V

V

S

T

E

N

A

C

F

Y

I

E

E

R

A

A

R

K

D

E

I

I

L

I

K

R

R

K

R

Y

R

R

A

K

D

Q

L

L

D

D

K

N

G

I

A

V

E

E

L

I

L

L

E

E

K

K

E

E

A

T

I

I

T

E

A

G

E

D

D

E

F

L

binding N-{(2R)-2-[2-(hydroxyamino)-2-oxoethyl]-4-methylpentanoyl}-3-naphthalen-2-yl-L-alanyl-L-alaninamide: H259 (= H422), E260 (= E423), H263 (= H426), K287 (≠ G450), L289 (= L452), S331 (≠ T495), A334 (= A498), D336 (= D500)
binding zinc ion: H259 (= H422), H263 (= H426), D336 (= D500)

Q8JZQ2 Mitochondrial inner membrane m-AAA protease component AFG3L2; AFG3-like protein 2; EC 3.4.24.-; EC 3.6.-.- from Mus musculus (Mouse) (see 3 papers)
48% identity, 83% coverage: 85:597/617 of query aligns to 227:740/802 of Q8JZQ2

query
sites
Q8JZQ2

T

T

L

L

A

Q

D

Q

K

E

L

L

S

G

A

I

E

E

G

G

-

E

-

N

-

R

-

V

-

P

-

V

I

V

K

Y

V

I

T

A

G

E

A

S

P

D

S

G

S

S

G

F

V

L

L

L

S

S

W

M

I

L

F

P

A

T

W

V

I

L

I

I

P

I

A

A

L

F

L

L

F

L

Y

Y

L

T

L

I

W

-

V

-

F

-

L

-

F

R

R

R

G

G

F

P

A

A

-

G

-

I

-

G

-

R

E

T

K

G

Q

R

G

G

L

M

G

G

L

L

M

F

T

S

I

V

G

G

K

E

S

T

H

T

A

A

K

K

V

V

Y

-

V

L

E

K

T

D

D

E

T

I

K

D

V

V

T

K

F

F

K

K

D

D

V

V

A

A

G

G

V

C

D

E

E

E

A

A

K

K

F

L

E

E

L

I

Q

M

E

E

V

F

V

V

S

N

F

F

L

L

R

K

D

N

P

P

K

K

S

Q

F

Y

G

Q

R

D

L

L

G

G

A

A

R

K

A

I

P

P

K

K

G

G

I

A

L

I

L

L

V

T

G

G

P

P

G

G

T

T

G

G

K

K

T

T

L

L

A

A

R

K

A

A

V

T

A

A

G

G

E

E

A

A

G

N

V

V

A

P

F

F

F

I

S

T

I

V

S

S

G

G

S

S

E

E

F

F

V

L

E

E

M

M

F

F

V

V

G

G

V

V

G

G

A

P

A

A

R

R

V

V

R
|
R

D

D

L

L

F

F

E

A

Q

L

A

A

R

R

K

K

A

N

A

A

P

P

C

C

I

I

I

L

F

F

I

I

D

D

E

E

L

I

D

D

A

A

L

V

G

G

R

R

S

K

R

R

S

G

V

R

G

G

G

N

I

F

G

G

V

Q

D

S

E

E

K

Q

E

E

Q

N

T

T

L

L

N

N

Q

Q

L

L

A

V

E

E

L

M

D

D

G

G

F

F

D

N

P

T

S

T

I

T

G

N

V

V

I

V

L

I

L

L

A

A

A

G

T

T

N

N

R

R

P

P

E

D

I

I

L

L

D

D

P

P

A

A

L

L

R

R

A

P

G

G

R

R

F

F

D

D

R

R

Q

Q

V

I

L

F

V

I

D

G

R

P

P

P

D

D

R

I

I

K

G

G

R

R

S

A

E

S

I

I

L

F

K

K

V

V

H

H

A

L

R

R

K

P

I

L

V

K

L

L

D

D

P

S

A

A

V

L

D

E

L

K

D

D

-

K

-

L

-

A

-

R

Q

K

I

L

A

A

G

S

M

L

T

T

T

P

G

G

F

F

T

S

G

G

A

A

D

D

L

V

A

A

N

N

L

V

V

C

N

N

E

E

A

A

V

L

I

I

A

A

T

A

R

R

R

H

K

L

S

S

A

D

A

A

T

I

T

N

A

E

A

K

D

H

F

F

T

E

A

Q

A

A

I

I

E

E

R

R

V

V

I

I

A

G

G

G

I

L

E

E

K

K

S

T

R

Q

I

V

L

L

S

Q

A

P

P

E

E

E

R

K

R

T

V

V

A

A

Y

Y

H

H

E
|
E

M

A

G

G

H

H

A

A

L

V

V

A

A

G

A

W

T

Y

L

L

P

E

D

H

V

A

D

D

P

P

V

L

L

L

K

K

V

V

S

S

I

I

P

P

R

R

G

G

V

K

G

G

A

-

L

L

G

G

Y

Y

T

A

I

Q

Q

Y

R

L

P

P

T

K

E

E

D

-

R

Q

F

Y

V

L

L

Y

A

T

A

K

S

E

E

Q

L

L

R

L

H

D

R

R

I

M

T

C

V

M

L

T

L

L

G

G

R

R

A

V

A

S

E

E

S

E

L

I

L

F

F

F

D

-

G

G

D

R

I

I

S

T

T

T

G

G

A

A

A

Q

D

D

L

L

Q

R

R

K

A

V

T

T

D

Q

I

S

A

A

L

Y

E

A

M

Q

T

I

T

V

L

Q

Y

F

G

G

M
|
M

A

N

Q

E

T

K

V

V

G

G

Q

Q

R

I

T

S

Y

F

R

D

A

L

S

P

P

R

Q

Q

F

-

L

-

D

-

Q

G

A

D

Q

M

S

V

R

L

D

E

K

K

P

P

A

-

A

Y

S

S

E

E

A

A

T

T

L

A

R

R

E

M

I

I

D

D

V

D

S

E

V

V

R

R

D

I

I

L

V

I

S

S

E

D

A

A

F

Y

I

R

E

R

A

T

R

V

D

A

I

L

L

L

K

T

R

E

R

K

A

A

D

D

L

V

D

E

K

K

G

V

A

A

E

L

L

L

E

E

K

K

E

E

A

V

I

L

T

D

A

K

E

N

D

D

F

M

R389 (= R242) to G: in par
E574 (= E423) mutation to Q: Absence of proteolytic activity. Loss of its processing into the mature form.
M665 (= M517) mutation to R: Knockin heterozygous mice develop normally but adult mice show signs of cerebellar ataxia. Cells from mutant mice display altered mitochondrial bioenergetics, with decreased basal oxygen consumption rate, ATP synthesis and mitochondrial membrane potential. Mitochondrial network formation and morphology is also altered, with greatly reduced expression of fusogenic Opa1 isoforms.

Sites not aligning to the query:

1:38 modified: transit peptide, Mitochondrion

2ceaC Cell division protein ftsh (see paper)
52% identity, 72% coverage: 154:597/617 of query aligns to 6:412/421 of 2ceaC

query
sites
2ceaC

D

N

T

K

K

R

V

V

T

T

F

F

K

K

D

D

V

V

A
x
G

G

G

V

A

D

E

E

E

A

A

K

I

F

E

E

E

L

L

Q

K

E

E

V

V

S

E

F

F

L

L

R

K

D

D

P

P

K

S

S

K

F

F

G

N

R

R

L

I

G

G

A

A

R

R

A

M

P

P

K

K

G

G

I

I

L

L

V

V

G

G

P

P

P
|
P

G
|
G

T
|
T

G
|
G

K
|
K

T
|
T

L
|
L

L

L

A

A

R

R

A

A

V

V

A

A

G

G

E

E

A

A

G

N

V

V

A

P

F

F

S

H

I

I

S

S

G

G

S

S

E

D

F

F

V

V

E

E

M

L

F

F

V

V

G

G

V

V

G

G

A

A

R

R

V

V

R

R

D

D

L

L

F

F

E

A

Q

Q

A

A

R

K

K

A

A

H

A

A

P

P

C

C

I

I

I

V

F

F

I

I

D

D

E

E

L

I

D

D

A

A

L

V

G

G

R

R

S

-

R

-

S

-

V

-

G

-

I

-

G

-

V

-

D

-

E

-

K

-

E

E

Q

Q

T

T

L

L

N

N

Q

Q

L

L

A

V

E

E

L

M

D

D

G

G

F

F

D

D

P

S

S

K

I

E

G

G

V

I

I

I

L

V

L

M

A

A

T

T

N

N

R

R

P

P

E

D

I

I

L

L

D

D

P

P

A

A

L

L

R

R

A

P

G

G

R

R

F

F

D

D

R

K

Q

K

V

I

L

V

V

V

D

D

R

P

P

P

D

D

R

M

I

L

G

G

R

R

S

K

E

K

I
|
I

L

L

K

E

V

I

H
|
H

A

T

R

R

K

N

I

K

V

P

L

L

D

A

P

E

A

D

V

V

D

N

L

L

D

E

Q

I

I

I

A

A

G

K

M

R

T

T

T

P

G

G

F

F

T

V

G
|
G

A
|
A

D

D

L

L

A
x
E

N

N

L

L

V

V

N

N

E

E

A

A

V

L

I

L

A

A

T

A

R

R

R

E

K

G

S

R

A

D

A

K

T

I

T

T

A

M

A

K

D

D

F

F

T

E

A

E

A

A

I

I

E

D

R

R

V

V

I

I

A

-

G

-

I

-

E

-

K

-

S

-

R

-

I

L

L

I

S

S

A

P

P

A

E

E

R

K

R

R

R

I

V

I

A

A

Y

Y

H
|
H

E

E

M

A

G

G

H
|
H

A

A

L

V

V

V

A

S

A

T

T

V

L

V

P

P

D

N

V

G

D

E

P

P

V

V

L

H

K

R

V

I

S

S

I

I

P

P

R

R

G

G

V

Y

G

K

A

A

L

L

G

G

Y

Y

T

T

I

L

Q

H

R

L

P

P

T

E

E

E

D

D

R

K

F

Y

V

L

L

V

A

S

A

R

S

N

E

E

L

L

R

L

H

D

R

K

I

L

T

T

V

A

L

L

G

G

R

R

A

A

E

E

S

E

L

V

F

F

D

-

G

G

D

D

I

V

S

T

T

S

G

G

A

A

D

N

D
|
D

L

I

Q

E

R

R

A

A

T

T

D

E

I

I

A

A

L

R

E

N

M

M

T

V

T

C

L

Q

Y

L

G

G

M

M

A

S

Q

E

T

E

V

L

G

G

Q

P

-

L

-

A

-

W

-

G

R

R

T

N

Y

Y

R

-

A

-

S

-

P

-

Q

-

F

-

L

-

D

-

Q

-

A

-

Q

-

S

-

R

-

D

-

K

-

P

-

A

-

S

S

E

E

A

E

T

V

L

A

R

S

E

K

I

I

D

D

V

E

S

E

V

V

R

K

D

K

I

I

V

V

S

T

E

N

A

C

F

Y

I

E

E

R

A

A

R

K

D

E

I

I

L

I

K

R

R

K

R

Y

R

R

A

K

D

Q

L

L

D

D

K

N

G

I

A

V

E

E

L

I

L

L

E

E

K

K

E

E

A

T

I

I

T

E

A

G

E

D

D

E

F

L

binding adenosine-5'-diphosphate: G15 (≠ A163), P54 (= P202), G55 (= G203), T56 (= T204), G57 (= G205), K58 (= K206), T59 (= T207), L60 (= L208), I177 (= I338), H181 (= H342), G205 (= G366), A206 (= A367), E209 (≠ A370)
binding magnesium ion: P54 (= P202), K58 (= K206)
binding zinc ion: H253 (= H422), H257 (= H426), D330 (= D500)

2ce7C Edta treated (see paper)
52% identity, 72% coverage: 154:597/617 of query aligns to 6:412/421 of 2ce7C

query
sites
2ce7C

D

N

T

K

K

R

V

V

T

T

F

F

K

K

D

D

V

V

A
x
G

G

G

V

A

D

E

E

E

A

A

K

I

F

E

E

E

L

L

Q

K

E

E

V

V

S

E

F

F

L

L

R

K

D

D

P

P

K

S

S

K

F

F

G

N

R

R

L

I

G

G

A

A

R

R

A

M

P

P

K

K

G

G

I

I

L

L

V

V

G

G

P

P

P
|
P

G
|
G

T
|
T

G
|
G

K
|
K

T
|
T

L
|
L

L

L

A

A

R

R

A

A

V

V

A

A

G

G

E

E

A

A

G

N

V

V

A

P

F

F

S

H

I

I

S

S

G

G

S

S

E

D

F

F

V

V

E

E

M

L

F

F

V

V

G

G

V

V

G

G

A

A

R

R

V

V

R

R

D

D

L

L

F

F

E

A

Q

Q

A

A

R

K

K

A

A

H

A

A

P

P

C

C

I

I

I

V

F

F

I

I

D

D

E

E

L

I

D

D

A

A

L

V

G

G

R

R

S

-

R

-

S

-

V

-

G

-

I

-

G

-

V

-

D

-

E

-

K

-

E

E

Q

Q

T

T

L

L

N

N

Q

Q

L

L

A

V

E

E

L

M

D

D

G

G

F

F

D

D

P

S

S

K

I

E

G

G

V

I

I

I

L

V

L

M

A

A

T

T

N

N

R

R

P

P

E

D

I

I

L

L

D

D

P

P

A

A

L

L

R

R

A

P

G

G

R

R

F

F

D

D

R

K

Q

K

V

I

L

V

V

V

D

D

R

P

P

P

D

D

R

M

I

L

G

G

R

R

S

K

E

K

I
|
I

L

L

K

E

V

I

H
|
H

A

T

R

R

K

N

I

K

V

P

L

L

D

A

P

E

A

D

V

V

D

N

L

L

D

E

Q

I

I

I

A

A

G

K

M

R

T

T

T

P

G

G

F

F

T

V

G
|
G

A
|
A

D

D

L

L

A
x
E

N

N

L

L

V

V

N

N

E

E

A

A

V

L

I

L

A

A

T

A

R

R

R

E

K

G

S

R

A

D

A

K

T

I

T

T

A

M

A

K

D

D

F

F

T

E

A

E

A

A

I

I

E

D

R

R

V

V

I

I

A

-

G

-

I

-

E

-

K

-

S

-

R

-

I

L

L

I

S

S

A

P

P

A

E

E

R

K

R

R

R

I

V

I

A

A

Y

Y

H
|
H

E

E

M

A

G

G

H
|
H

A

A

L

V

V

V

A

S

A

T

T

V

L

V

P

P

D

N

V

G

D

E

P

P

V

V

L

H

K

R

V

I

S

S

I

I

P

P

R

R

G

G

V

Y

G

K

A

A

L

L

G

G

Y

Y

T

T

I

L

Q

H

R

L

P

P

T

E

E

E

D

D

R

K

F

Y

V

L

L

V

A

S

A

R

S

N

E

E

L

L

R

L

H

D

R

K

I

L

T

T

V

A

L

L

G

G

R

R

A

A

E

E

S

E

L

V

F

F

D

-

G

G

D

D

I

V

S

T

T

S

G

G

A

A

D

N

D
|
D

L

I

Q

E

R

R

A

A

T

T

D

E

I

I

A

A

L

R

E

N

M

M

T

V

T

C

L

Q

Y

L

G

G

M

M

A

S

Q

E

T

E

V

L

G

G

Q

P

-

L

-

A

-

W

-

G

R

R

T

N

Y

Y

R

-

A

-

S

-

P

-

Q

-

F

-

L

-

D

-

Q

-

A

-

Q

-

S

-

R

-

D

-

K

-

P

-

A

-

S

S

E

E

A

E

T

V

L

A

R

S

E

K

I

I

D

D

V

E

S

E

V

V

R

K

D

K

I

I

V

V

S

T

E

N

A

C

F

Y

I

E

E

R

A

A

R

K

D

E

I

I

L

I

K

R

R

K

R

Y

R

R

A

K

D

Q

L

L

D

D

K

N

G

I

A

V

E

E

L

I

L

L

E

E

K

K

E

E

A

T

I

I

T

E

A

G

E

D

D

E

F

L

binding adenosine-5'-diphosphate: G15 (≠ A163), P54 (= P202), G55 (= G203), T56 (= T204), G57 (= G205), K58 (= K206), T59 (= T207), L60 (= L208), I177 (= I338), H181 (= H342), G205 (= G366), A206 (= A367), E209 (≠ A370)
binding zinc ion: H253 (= H422), H257 (= H426), D330 (= D500)

Q9Y4W6 Mitochondrial inner membrane m-AAA protease component AFG3L2; AFG3-like protein 2; Paraplegin-like protein; EC 3.4.24.-; EC 3.6.-.- from Homo sapiens (Human) (see 18 papers)
48% identity, 83% coverage: 85:597/617 of query aligns to 228:741/797 of Q9Y4W6

query
sites
Q9Y4W6

T

T

L

L

A

Q

D

Q

K

E

L

L

S

G

A

I

E

E

G

G

-

E

-

N

-

R

-

V

-

P

-

V

I

V

K

Y

V

I

T

A

G

E

A

S

P

D

S

G

S

S

G

F

V

L

L

L

S

S

W

M

I

L

F

P

A

T

W

V

I

L

I

I

P

I

A

A

L

F

L

L

F

L

Y

Y

L

T

L

I

W

-

V

-

F

-

L

-

F

R

R

R

G

G

F

P

A

A

-

G

-

I

-

G

-

R

E

T

K

G

Q

R

G

G

L

M

G

G

L

L

M
x
F

T

S

I

V

G

G

K

E

S

T

H

T

A

A

K

K

V

V

Y

-

V
x
L

E

K

T

D

D

E

T

I

K

D

V

V

T
x
K

F

F

K

K

D

D

V

V

A

A

G

G

V

C

D

E

E

E

A

A

K

K

F

L

E

E

L

I

Q

M

E

E

V

F

V

V

S

N

F

F

L

L

R

K

D

N

P

P

K

K

S

Q

F

Y

G

Q

R

D

L

L

G
|
G

A

A

R

K

A

I

P

P

K

K

G

G

I

A

L

I

L
|
L

V

T

G

G

P

P

G

G

T

T

G

G

K
|
K

T

T

L

L

A

A

R

K

A

A

V

T

A

A

G

G

E

E

A

A

G

N

V

V

A

P

F

F

F

I

S

T

I

V

S

S

G

G

S

S

E
|
E

F
|
F

V

L

E

E

M
|
M

F

F

V

V

G

G

V

V

G

G

A

P

A

A

R

R

V

V

R

R

D

D

L

L

F

F

E

A

Q

L

A

A

R

R

K

K

A

N

A

A

P

P

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F289 (≠ M140) mutation to A: Reduced rate of protein degradation.
L299 (≠ V151) mutation to A: Reduced rate of protein degradation.
K306 (≠ T158) to E: in SPAX5; uncertain significance
G337 (= G189) to E: in OPA12; loss-of-function variant resulting in aberrant OPA1 processing and mitochondrial fragmentation; dbSNP:rs1908566777
L346 (= L198) to F: in OPA12; uncertain significance; dbSNP:rs755893615
K354 (= K206) mutation to A: Does not effect activity of the m-AAA protease complex.
E376 (= E228) to K: in OPA12; uncertain significance
F377 (= F229) to S: in OPA12; uncertain significance; dbSNP:rs1908507433
M380 (= M232) mutation to K: Abolished ATPase and protease activities.; mutation to V: Increased ATP hydrolysis.
D407 (= D259) to G: in OPA12; uncertain significance; decreased proteolytic function; dbSNP:rs1908371616
E408 (= E260) mutation to Q: Abolished ATPase activity, leading to impaired activity of the m-AAA protease complex, preventing cleavage and degradation of substrate proteins.
R416 (= R268) to S: in OPA12; uncertain significance
F421 (≠ I273) mutation to A: Impairted protease activity without affecting the ATPase activity.
T430 (= T282) to I: in OPA12; uncertain significance; dbSNP:rs1908369114
N432 (= N284) to T: in SCA28; abolished ability to degrade substrate proteins; dbSNP:rs151344512
A462 (= A314) to V: in OPA12 and SPAX5; decreased proteolytic function; dbSNP:rs912546325
R465 (= R317) to K: in OPA12; decreased proteolytic function; dbSNP:rs1908309088
R468 (= R320) to C: in OPA12; decreased proteolytic function resulting in impaired autocatalytic processing and impaired proteolytic maturation of SPG7; does not affect the interaction with SPG7; dbSNP:rs1020764190
P514 (≠ T362) to L: in OPA12; decreased proteolytic function; dbSNP:rs1908300748
A572 (= A420) to T: found in patients from a consanguineous family with progressive microcephaly, early onset seizures, spasticity and basal ganglia involvement; uncertain significance; decreased ATPase and ability to degrade substrate proteins
H574 (= H422) binding Zn(2+)
E575 (= E423) mutation to Q: Abolished protease activity. Loss of autocatalytic processing. Impaired proteolytic maturation of SPG7.
H578 (= H426) binding Zn(2+)
Y605 (= Y454) to C: in OPA12; uncertain significance; dbSNP:rs773240455
Y616 (≠ L466) to C: in SPAX5 and SCA28; hypomorphic mutation in SPAX5; in SCA28, compound heterozygous with M-723; impaired oligomerization with itself and SPG7; increased ATPase and proteolytic activities; dbSNP:rs387906889
Q620 (≠ E470) to K: in SPAX5; impaired function shown in a yeast complementation assay; dbSNP:rs1907907851
L621 (= L471) to V: in SCA28; uncertain significance
T644 (= T495) to S: in OPA12; uncertain significance; dbSNP:rs1226952405
D649 (= D500) binding Zn(2+)
M666 (= M517) to R: in SCA28; abolished ability to degrade substrate proteins; dbSNP:rs151344515
P688 (= P543) to T: in SCA28; uncertain significance; slightly decreased ability to degrade substrate proteins
E691 (= E547) to K: in SCA28; impaired function shown in a yeast complementation assay; dbSNP:rs151344520
V723 (≠ L579) to M: in SCA28; compound heterozygous with C-616

Sites not aligning to the query:

74 E → A: in OPA12; uncertain significance
191 V → I: in SCA28; uncertain significance
779 W→R: Impaired ability to degrade substrates without affecting the ATPase activity.

P39925 Mitochondrial inner membrane m-AAA protease component YTA10; ATPase family gene 3 protein; Tat-binding homolog 10; EC 3.4.24.-; EC 3.6.-.- from Saccharomyces cerevisiae (strain ATCC 204508 / S288c) (Baker's yeast) (see 3 papers)
51% identity, 74% coverage: 139:596/617 of query aligns to 266:724/761 of P39925

query
sites
P39925

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