≡

Calpain 3 (CAPN3)

[Edit]

CANPL3; p94; LGMD2; LGMD2A; nCL1; Calcium-activated neutral proteinase 3; Muscle-specific calcium-activated neutral protease 3; New calpain 1

Calpain 3 (CAPN3)
CCC C960
HGNC 1480
MGI 107437
PubMed 11371436
RGD 2269
UniProt P20807
Wiki CAPN3
Calpain, a heterodimer consisting of a large and a small subunit, is a major intracellular protease, although its function has not been well established. CAPN3 encodes a muscle-specific member of the calpain large subunit family that specifically binds to titin.Alternate promoters and alternative splicing result in multiple transcript variants encoding different isoforms and some variants are ubiquitously expressed. In melanocytic cells CAPN3 gene expression may be regulated by MITF.Defects in CAPN3 are the cause of limb-girdle muscular dystrophy type 2A (LGMD2A). LGMD2A is an autosomal recessive degenerative myopathy characterized by progressive symmetrical atrophy and weakness of the proximal limb muscles and elevated serum creatine kinase.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPC960Hu01 Recombinant Calpain 3 (CAPN3) Positive Control; Immunogen; SDS-PAGE; WB.
APC960Hu01 Active Calpain 3 (CAPN3) Cell culture; Activity Assays.
Antibodies PAC960Hu01 Polyclonal Antibody to Calpain 3 (CAPN3) WB; IHC; ICC; IP.
MAC960Hu22 Monoclonal Antibody to Calpain 3 (CAPN3) WB; IHC; ICC; IP.
Assay Kits SEC960Hu ELISA Kit for Calpain 3 (CAPN3) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Calpain 3 (CAPN3) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Calpain 3 (CAPN3) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Calpain 3 (CAPN3) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Calpain 3 (CAPN3) CLIA Kit Customized Service Offer
n/a ELISA Kit for Calpain 3 (CAPN3) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Calpain 3 (CAPN3) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Calpain 3 (CAPN3) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Calpain 3 (CAPN3) Polyclonal Antibody Customized Service Offer
Assay Kits SEC960Ra ELISA Kit for Calpain 3 (CAPN3) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Bos taurus; Bovine (Cattle)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Calpain 3 (CAPN3) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Calpain 3 (CAPN3) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Calpain 3 (CAPN3) Polyclonal Antibody Customized Service Offer
Assay Kits SEC960Bo ELISA Kit for Calpain 3 (CAPN3) Enzyme-linked immunosorbent assay for Antigen Detection.
  1. "Mutations in the proteolytic enzyme calpain 3 cause limb-girdle muscular dystrophy type 2A."Cell 81:27-40(1995) [PubMed] [Europe PMC] [Abstract]
  2. "Analysis of the DNA sequence and duplication history of human chromosome 15." Nature 440:671-675(2006) [PubMed] [Europe PMC] [Abstract]
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  4. "Molecular cloning of a novel mammalian calcium-dependent protease distinct from both m- and mu-types. Specific expression of the mRNA in skeletal muscle."J. Biol. Chem. 264:20106-20111(1989) [PubMed] [Europe PMC] [Abstract]
  5. "The muscle ankyrin repeat proteins: CARP, ankrd2/Arpp and DARP as a family of titin filament-based stress response molecules."J. Mol. Biol. 333:951-964(2003) [PubMed] [Europe PMC] [Abstract]
  6. "Calpainopathy -- a survey of mutations and polymorphisms." Am. J. Hum. Genet. 64:1524-1540(1999) [PubMed] [Europe PMC] [Abstract]
  7. "Interactions with M-band titin and calpain 3 link myospryn (CMYA5) to tibial and limb-girdle muscular dystrophies."J. Biol. Chem. 285:30304-30315(2010) [PubMed] [Europe PMC] [Abstract]
  8. "Juvenile limb-girdle muscular dystrophy. Clinical, histopathological and genetic data from a small community living in the Reunion island."Brain 119:295-308(1996) [PubMed] [Europe PMC] [Abstract]
  9. "Multiple independent molecular etiology for limb-girdle muscular dystrophy type 2A patients from various geographical origins."Am. J. Hum. Genet. 60:1128-1138(1997) [PubMed] [Europe PMC] [Abstract]
  10. "A biochemical, genetic, and clinical survey of autosomal recessive limb girdle muscular dystrophies in Turkey."Ann. Neurol. 42:222-229(1997) [PubMed] [Europe PMC] [Abstract]
  11. "Limb-girdle muscular dystrophy in Guipuzcoa (Basque Country, Spain)."Brain 121:1735-1747(1998) [PubMed] [Europe PMC] [Abstract]
  12. "A small in-frame deletion within the protease domain of muscle-specific calpain, p94 causes early-onset limb-girdle muscular dystrophy 2A."Hum. Mutat. Suppl. 1:S298-S300(1998) [PubMed] [Europe PMC] [Abstract]
  13. "Pseudometabolic expression and phenotypic variability of calpain deficiency in two siblings."Muscle Nerve 21:1078-1080(1998) [PubMed] [Europe PMC] [Abstract]
  14. "Clinical, pathological, and genetic features of limb-girdle muscular dystrophy type 2A with new calpain 3 gene mutations in seven patients from three Japanese families."Muscle Nerve 21:1493-1501(1998) [PubMed] [Europe PMC] [Abstract]