logo CaMPDB: Calpain for Modulatory Proteolysis Database

SB0141 : Ataxin-3, Machado-Joseph disease (spinocerebellar ataxia 3)

[ CaMP Format ]

* Basic Information

OrganismHomo sapiens (human)
Protein NamesMJD1 protein [Homo sapiens]; MJD1 protein
Gene NamesMJD1
Gene LocusNot available
GO FunctionNot available
Entrez Protein Entrez Nucleotide Entrez Gene UniProt OMIM HGNC HPRD KEGG
AAB33571 N/A N/A N/A N/A N/A N/A N/A

* Information From OMIM

Not Available.

* Structure Information

1. Primary Information

Length: 360 aa

Average Mass: 41.531 kDa

Monoisotopic Mass: 41.505 kDa

2. Domain Information

Annotated Domains: Not Available.

Computationally Assigned Domains (Pfam+HMMER):

domain namebeginendscoree-value
Ubiquitin interaction motif 1. 41491.00.9
--- cleavage 55 ---
--- cleavage 62 ---
Uncharacterized conserved protein (DUF2215) 1. 7696126.00.0
--- cleavage 187 ---
--- cleavage 208 ---
Gene regulated by oestrogen in breast cancer 1. 2263181094.011.8
--- cleavage 256 (inside Gene regulated by oestrogen in breast cancer 226..318) ---
--- cleavage 317 (inside Gene regulated by oestrogen in breast cancer 226..318) ---

3. Sequence Information

Fasta Sequence: SB0141.fasta

Amino Acid Sequence and Secondary Structures (PsiPred):

4. 3D Information

Not Available.

* Cleavage Information

6 [sites] cleaved by Calpain 2

Source Reference: [PubMed ID: 17488727] Haacke A, Hartl FU, Breuer P, Calpain inhibition is sufficient to suppress aggregation of polyglutamine-expanded ataxin-3. J Biol Chem. 2007 Jun 29;282(26):18851-6. Epub 2007 May 8.

Cleavage sites (±10aa)

[Site 1] MRMAEGGVTS55-EDYRTFLQQP

Ser55 Glu

P10 P9 P8 P7 P6 P5 P4 P3 P2 P1
Met46Arg47Met48Ala49Glu50Gly51Gly52Val53Thr54Ser55
P1' P2' P3' P4' P5' P6' P7' P8' P9' P10'
Glu56Asp57Tyr58Arg59Thr60Phe61Leu62Gln63Gln64Pro65

Sequence conservation (by blast)

[Site 2] VTSEDYRTFL62-QQPSGNMDDS

Leu62 Gln

P10 P9 P8 P7 P6 P5 P4 P3 P2 P1
Val53Thr54Ser55Glu56Asp57Tyr58Arg59Thr60Phe61Leu62
P1' P2' P3' P4' P5' P6' P7' P8' P9' P10'
Gln63Gln64Pro65Ser66Gly67Asn68Met69Asp70Asp71Ser72

Sequence conservation (by blast)

[Site 3] LQMIRVQQMH187-RPKLIGEELA

His187 Arg

P10 P9 P8 P7 P6 P5 P4 P3 P2 P1
Leu178Gln179Met180Ile181Arg182Val183Gln184Gln185Met186His187
P1' P2' P3' P4' P5' P6' P7' P8' P9' P10'
Arg188Pro189Lys190Leu191Ile192Gly193Glu194Glu195Leu196Ala197

Sequence conservation (by blast)

[Site 4] LKEQRVHKTD208-LERMLEANDG

Asp208 Leu

P10 P9 P8 P7 P6 P5 P4 P3 P2 P1
Leu199Lys200Glu201Gln202Arg203Val204His205Lys206Thr207Asp208
P1' P2' P3' P4' P5' P6' P7' P8' P9' P10'
Leu209Glu210Arg211Met212Leu213Glu214Ala215Asn216Asp217Gly218

Sequence conservation (by blast)

[Site 5] ADLRRAIQLS256-MQGSSRNISQ

Ser256 Met

P10 P9 P8 P7 P6 P5 P4 P3 P2 P1
Ala247Asp248Leu249Arg250Arg251Ala252Ile253Gln254Leu255Ser256
P1' P2' P3' P4' P5' P6' P7' P8' P9' P10'
Met257Gln258Gly259Ser260Ser261Arg262Asn263Ile264Ser265Gln266

Sequence conservation (by blast)

[Site 6] QQQQQQQQQQ317-RDLSGQSSHP

Gln317 Arg

P10 P9 P8 P7 P6 P5 P4 P3 P2 P1
Gln308Gln309Gln310Gln311Gln312Gln313Gln314Gln315Gln316Gln317
P1' P2' P3' P4' P5' P6' P7' P8' P9' P10'
Arg318Asp319Leu320Ser321Gly322Gln323Ser324Ser325His326Pro327

Sequence conservation (by blast)

* References

[PubMed ID: 7874163] Kawaguchi Y, Okamoto T, Taniwaki M, Aizawa M, Inoue M, Katayama S, Kawakami H, Nakamura S, Nishimura M, Akiguchi I, et al., CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat Genet. 1994 Nov;8(3):221-8.