Entry
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
Genes
XLA : 100125665(capn3.L) 108700461
CAUA : 113060520 113117063
MASI : 127411246 127454769
TRU : 101075691 101077986(capn3)
TFS : 130513329(capn3a) 130515702(capn3b)
TBEN : 117486936(capn3b) 117493771(capn3a)
CGOB : 115027228(capn3) 115028992
PGEO : 117439943(capn3b) 117467960(capn3a)
GACU : 117542903 117549038(capn3a) 117555691(capn3b)
EMAC : 134876635(capn3b) 134881072(capn3a)
ELY : 117267460(capn3a) 117270774(capn3b)
EFO : 125897620(capn3b) 125901103(capn3a)
PLEP : 121953730(capn3a) 121955200(capn3b)
SLUC : 116057697(capn3a) 116066425(capn3b)
ECRA : 117936309(capn3a) 117961699(capn3b)
ESP : 116686083(capn3) 116705817
PFLV : 114546330(capn3) 114573031
GAT : 120808321(capn3b) 120833187(capn3a)
PPUG : 119194637(capn3b) 119227420(capn3a)
AFB : 129102973(capn3a) 129107456(capn3b)
CLUM : 117727050(capn3b) 117751379(capn3a)
PSWI : 130201752(capn3a) 130202117(capn3b)
MSAM : 119909041(capn3b) 119909908(capn3a)
SCHU : 122861776(capn3a) 122863461(capn3b)
CUD : 121521109(capn3b) 121526230(capn3a)
ALAT : 119005030(capn3a) 119012575(capn3b)
MZE : 101463601 101466182(capn3)
ONL : 100691885(capn3) 100699569
OAU : 116318066(capn3a) 116324143(capn3b)
OML : 112144429(capn3a) 112158058(capn3b)
CSAI : 133418611(capn3b) 133462927(capn3a)
XMA : 102221680(capn3) 102222528
XCO : 114134598(capn3) 114158499
XHE : 116708429(capn3) 116733763
PRET : 103457431 103458212(capn3)
PFOR : 103133981 103150049(capn3)
PLAI : 106952040 106953715
PMEI : 106919034(capn3) 106924031
GAF : 122825756(capn3b) 122846114(capn3a)
PPRL : 129375248(capn3b) 129377228(capn3a)
CVG : 107093158 107096801(capn3)
GMU : 124856039(capn3a) 124882084(capn3b)
NFU : 107377674 107391866(capn3)
KMR : 108233461(capn3b) 108234555(capn3a)
ALIM : 106524302(capn3) 106537237
NWH : 119418575(capn3b) 119426946(capn3a)
AOCE : 111574533 111587838
MCEP : 124998693(capn3b) 125023329(capn3a)
CSEM : 103380923 103381555
SSEN : 122783338(capn3a) 122784464(capn3b)
HHIP : 117755906(capn3a) 117757990(capn3b)
HSP : 118099302(capn3b) 118117058(capn3a)
SMAU : 118285806(capn3a) 118290255(capn3b)
LCF : 108876634(capn3a) 108895964
SDU : 111225368(capn3) 111237184
SLAL : 111646798(capn3) 111661373
XGL : 120788896(capn3b) 120795487(capn3a)
HCQ : 109508050(capn8) 109517476(capn3)
SSCV : 125981134 125990290
SBIA : 133497039(capn3b) 133513735(capn3a)
PEE : 133412495(capn3a) 133416781(capn3b)
PTAO : 133468094(capn3b) 133487602(capn3a)
BPEC : 110157545(capn3) 110173943
MALB : 109953348 109962461(capn3)
BSPL : 114849042(capn3a) 114849713(capn3b)
SASA : 100306869(can3) 106568467(capn3) 106611143
STRU : 115161830 115173034 115175025
OTW : 112241717 112249945(capn3b) 112255824(capn3a)
OMY : 110498417 110505915(capn3a) 110529398(capn3b)
OGO : 123990572(capn3b) 124002235 124046822(capn3a)
OKI : 109880971 109883062 109900562
SNH : 120024248(capn3b) 120035919(capn3a)
CCLU : 121539129 121544526(capn3a) 121552620(capn3b)
ELS : 105015344 105015896(capn3)
AANG : 118223800(capn3a) 118229185
PMRN : 116939632 116943170 116947735 116947736
LRJ : 133341426 133341428 133342411 133347253 133352610
» show all
Taxonomy
Reference
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 264:20106-11 (1989)
Sequence
Reference
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
Reference
Authors
Rey MA, Davies PL.
Title
The protease core of the muscle-specific calpain, p94, undergoes Ca2+-dependent intramolecular autolysis.
Journal
Reference
Authors
Garcia Diaz BE, Gauthier S, Davies PL.
Title
Ca2+ dependency of calpain 3 (p94) activation.
Journal
Reference
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
Other DBs
ExPASy - ENZYME nomenclature database: 3.4.22.54