Summary for peptidase C02.004: calpain-3

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature Human EST Mouse EST Substrates Inhibitors

 

Names
MEROPS Namecalpain-3
Other namescalpain LP74 (Rattus norvegicus), calpain-3 form Lp82, calpain-3 form Lp85, calpain p94, CANP3 g.p., CAPN3 g.p. (Homo sapiens), lens-specific calpain, Lp82, Lp85, muscle calpain, nCL-1, calpain Rt88 (Rattus norvegicus)
Domain architecture
MEROPS Classification
Classification Clan CA >> Subclan (none) >> Family C2 >> Subfamily A >> C02.004
Holotypecalpain-3 (Homo sapiens), Uniprot accession P20807 (peptidase unit: 99-400), MERNUM MER0001446
History Identifier created: Handbook of Proteolytic Enzymes (1998) Academic Press, London.
Activity
Catalytic typeCysteine
PeplistIncluded in the Peplist with identifier PL00079
NC-IUBMBSubclass 3.4 (Peptidases) >> Sub-subclass 3.4.22 (Cysteine endopeptidases) >> Peptidase 3.4.22.54
EnzymologyBRENDA database
Proteolytic eventsCutDB database (4 cleavages)
Activity statushuman: active (Guyon et al., 2003)
mouse: active (Azuma et al., 2003)
KnockoutIn humans, naturally-occurring mutations in the gene encoding C02.004 cause limb girdle muscular dystrophy type 2A (LGMD2A) (Sorimachi et al., 1995). Transgenic mice in which the active site Cys129 was replaced by Ser showed muscular abnormalities (Tagawa et al., 2000; Kramerova et al., 2004).
Pharmaceutical relevancePossible target for gene therapy.
Other databases WIKIPEDIAhttp://en.wikipedia.org/wiki/Calpain-3
Cleavage site specificity Explanations of how to interpret the following cleavage site sequence logo and specificity matrix can be found here.
Cleavage pattern-/-/l/-Scissile bond-/-/-/- (based on 19 cleavages)
weblogo
Specificity matrix
 
Amino acid P4 P3 P2 P1 P1' P2' P3' P4'
Gly 1 1 0 2 4 0 0 1
Pro 1 0 1 1 0 3 4 4
Ala 1 0 1 1 2 1 1 2
Val 1 1 3 0 1 1 1 2
Leu 0 4 5 1 0 2 2 2
Ile 2 1 0 0 1 0 2 1
Met 1 2 0 0 0 0 0 1
Phe 0 1 0 1 0 0 0 0
Tyr 0 0 0 2 0 1 0 0
Trp 0 0 0 0 0 0 0 0
Ser 1 2 3 1 2 1 1 1
Thr 2 1 2 0 3 4 0 1
Cys 0 0 0 1 0 1 0 0
Asn 2 0 0 1 0 0 1 0
Gln 2 0 2 1 1 0 0 0
Asp 1 1 1 1 0 1 0 0
Glu 2 2 0 1 1 1 4 1
Lys 0 1 1 3 3 2 0 1
Arg 2 1 0 1 1 1 3 2
His 0 1 0 1 0 0 0 0
Human genetics
Gene symbol Locus Megabases Ensembl Entrez gene Gene Cards OMIM
CAPN3 15q15.1 ENSG00000092529 825 CAPN3 114240
Mouse genetics
Gene symbol Position Megabases Ensembl Entrez gene MGI
Capn3 2:F1 ENSMUSG00000027289 12335 MGI:107437