Calpain 3 (CAPN3) (C-Term) antibody

Details for Product No. ABIN965743
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Synonyms capn3, MGC80925, zgc:92687, CAPN3, AI323605, Capa-3, Capa3, Lp82, p94, Lp84, Lp85, CANP3, CANPL3, LGMD2, LGMD2A, nCL-1, SKM-CALP, SKM-CALPOV, nCL1
(5), (3), (2), (2), (1), (1), (1), (1), (1), (1)
(23), (2), (2), (1)
(17), (6)
Immunohistochemistry (IHC)
(23), (7), (6), (4), (3), (2), (1), (1)
Pubmed 6 references available
Quantity 0.1 mg
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Catalog No. ABIN965743
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Immunogen Polyclonal antibody produced in rabbits immunizing with a synthetic peptide corresponding to C-terminal residues of human CAPN3(Calpain-3)
Purification Purified by antigen-specific affinity chromatography.
Alternative Name CAPN3
Background CAPN3(Calpain-3) is a calcium-regulated non-lysosomal thiol-protease. It has a broad endopeptidase specificity. CAPN3 is activated by micromolar concentrations of calcium and inhibited by calpastatin. It interacts with TTN/titin. Isoform I is skeletal muscle specific. Defects in CAPN3 are the cause of limb-girdle muscular dystrophy type 2A (LGMD2A). LGMD2A is both autosomal dominantly and recessively transmitted. It is characterized by progressive symmetrical atrophy and weakness of the proximal limb muscles and elevated serum creatine kinase. CAPN3 belongs to the peptidase C2 family and contains 1 calpain catalytic domain and 4 EF-hand domains.
Application Notes ELISA, Western blotting: 1µg/ml for 2hrs.
Restrictions For Research Use only
Format Liquid
Buffer This antibody is stored in PBS, 50% glycerol
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage -20 °C
Product cited in: Miller, Bang, Witt et al.: "The muscle ankyrin repeat proteins: CARP, ankrd2/Arpp and DARP as a family of titin filament-based stress response molecules." in: Journal of molecular biology, Vol. 333, Issue 5, pp. 951-64, 2003 (PubMed).

Richard, Roudaut, Saenz et al.: "Calpainopathy-a survey of mutations and polymorphisms." in: American journal of human genetics, Vol. 64, Issue 6, pp. 1524-40, 1999 (PubMed).

Richard, Brenguier, Dinçer et al.: "Multiple independent molecular etiology for limb-girdle muscular dystrophy type 2A patients from various geographical origins." in: American journal of human genetics, Vol. 60, Issue 5, pp. 1128-38, 1997 (PubMed).

Fardeau, Hillaire, Mignard et al.: "Juvenile limb-girdle muscular dystrophy. Clinical, histopathological and genetic data from a small community living in the Reunion Island." in: Brain : a journal of neurology, Vol. 119 ( Pt 1), pp. 295-308, 1996 (PubMed).

Richard, Broux, Allamand et al.: "Mutations in the proteolytic enzyme calpain 3 cause limb-girdle muscular dystrophy type 2A." in: Cell, Vol. 81, Issue 1, pp. 27-40, 1995 (PubMed).

Sorimachi, Imajoh-Ohmi, Emori et al.: "Molecular cloning of a novel mammalian calcium-dependent protease distinct from both m- and mu-types. Specific expression of the mRNA in skeletal muscle." in: The Journal of biological chemistry, Vol. 264, Issue 33, pp. 20106-11, 1989 (PubMed).

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