Calpain 3 (CAPN3) (C-Term) antibody

Antigen: Calpain 3 (CAPN3)

Synonyms: p94, CANP3, LGMD2, nCL-1, CANPL3, LGMD2A, MGC4403, MGC10767, MGC11121, MGC14344, Lp82, Capa3, Capa-3, AI323605, Lp84, Lp85, CAPN3, SKM-CALP, MGC160055, nCL1, SKM-CALPOV, capn3, MGC80925, zgc:92687

Binding Site: C-Term

Clonality: Polyclonal

Host: Rabbit

Reactivity: Human

Application: Immunohistochemistry (IHC)

Catalog no.: ABIN965743

Additional Information

Alternative name: CAPN3

Immunogen: Polyclonal antibody produced in rabbits immunizing with a synthetic peptide corresponding to C-terminal residues of human CAPN3(Calpain-3)

Description: 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 Details

Application Notes: ELISA, Western blotting: 1µg/ml for 2hrs.

Purification: Purified by antigen-specific affinity chromatography.

Buffer: This antibody is stored in PBS, 50% glycerol

Preservative: 0.01% Sodium Azide

Storage: Store at -20°C.

Restrictions: For Research Use only

Publications

Product: 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).

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).

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, 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).

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).

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).