KEGG   ENZYME: 3.4.22.54Help
Entry
EC 3.4.22.54                Enzyme                                 

Name
calpain-3;
p94;
calpain p94;
CAPN3;
muscle calpain;
calpain 3;
calcium-activated neutral proteinase 3;
muscle-specific calcium-activated neutral protease 3;
CANP 3;
calpain L3
Class
Hydrolases;
Acting on peptide bonds (peptidases);
Cysteine endopeptidases
BRITE hierarchy
Reaction(IUBMB)
Broad endopeptidase activity
Comment
This Ca2+-dependent enzyme is found in skeletal muscle and is genetically linked to limb girdle muscular dystrophy type 2A [1,4]. The enzyme is activated by autoproteolytic cleavage of insertion sequence 1 (IS1), which allows substrates and inhibitors gain access to the active site [4]. Substrates include the protein itself [3,4] and connectin/titin [2,5]. Belongs in peptidase family C2.
History
EC 3.4.22.54 created 2007
Orthology
K08573  
calpain-3
Genes
HSA: 
825(CAPN3)
PTR: 
PPS: 
100984344(CAPN3)
GGO: 
PON: 
100449804(CAPN3)
NLE: 
MCC: 
707913(CAPN3)
MCF: 
102115794(CAPN3)
CJC: 
100389438(CAPN3)
MMU: 
12335(Capn3)
RNO: 
29155(Capn3)
CGE: 
100758723(Capn3)
NGI: 
103744953(Capn3)
HGL: 
101707543(Capn3)
OCU: 
100008726(CAPN3)
TUP: 
102468105(CAPN3)
CFA: 
100856545(CAPN3)
AML: 
UMR: 
103673424(CAPN3)
FCA: 
101081304(CAPN3)
PTG: 
102962051(CAPN3)
BTA: 
281663(CAPN3)
BOM: 
102284625(CAPN3)
PHD: 
102336527(CAPN3)
CHX: 
102189762(CAPN3)
OAS: 
443038(CAPN3)
SSC: 
397349(CAPN3)
CFR: 
102518250(CAPN3)
LVE: 
103088026(CAPN3)
ECB: 
100056798(CAPN3)
MYB: 
102245181(CAPN3)
MYD: 
102768421(CAPN3)
PALE: 
102886269(CAPN3)
MDO: 
SHR: 
100916328(CAPN3)
GGA: 
423233(CAPN3)
MGP: 
APLA: 
101792647(CAPN3)
TGU: 
100225837(CAPN3)
FAB: 
101814350(CAPN3)
PHI: 
102107172(CAPN3)
FPG: 
101924532(CAPN3)
FCH: 
102055944(CAPN3)
CLV: 
102094715(CAPN3)
ASN: 
102379878(CAPN3)
AMJ: 
102577345(CAPN3)
PSS: 
102450185(CAPN3)
CMY: 
102936637(CAPN3)
ACS: 
100559629(capn3)
PBI: 
103066997(CAPN3)
XTR: 
DRE: 
447832(capn3a)
TRU: 
MZE: 
OLA: 
XMA: 
LCM: 
CMK: 
103190049(capn3)
CIN: 
 » show all
Taxonomy
Reference
1  [PMID:2555341]
  Authors
Sorimachi H, Imajoh-Ohmi S, Emori Y, Kawasaki H, Ohno S, Minami Y, Suzuki K.
  Title
Molecular cloning of a novel mammalian calcium-dependent protease distinct from both m- and mu-types. Specific expression of the mRNA in skeletal muscle.
  Journal
J. Biol. Chem. 264 (1989) 20106-11.
  Sequence
[hsa:825] [rno:29155]
Reference
2  [PMID:8537379]
  Authors
Sorimachi H, Kinbara K, Kimura S, Takahashi M, Ishiura S, Sasagawa N, Sorimachi N, Shimada H, Tagawa K, Maruyama K, et al.
  Title
Muscle-specific calpain, p94, responsible for limb girdle muscular dystrophy type 2A, associates with connectin through IS2, a p94-specific sequence.
  Journal
J. Biol. Chem. 270 (1995) 31158-62.
Reference
3  [PMID:12482600]
  Authors
Rey MA, Davies PL.
  Title
The protease core of the muscle-specific calpain, p94, undergoes Ca2+-dependent intramolecular autolysis.
  Journal
FEBS. Lett. 532 (2002) 401-6.
Reference
4  [PMID:16533054]
  Authors
Garcia Diaz BE, Gauthier S, Davies PL.
  Title
Ca2+ dependency of calpain 3 (p94) activation.
  Journal
Biochemistry. 45 (2006) 3714-22.
Reference
5  [PMID:16627476]
  Authors
Ono Y, Torii F, Ojima K, Doi N, Yoshioka K, Kawabata Y, Labeit D, Labeit S, Suzuki K, Abe K, Maeda T, Sorimachi H.
  Title
Suppressed disassembly of autolyzing p94/CAPN3 by N2A connectin/titin in a genetic reporter system.
  Journal
J. Biol. Chem. 281 (2006) 18519-31.
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 

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