Calpain-1 catalytic subunit

Calcium-regulated non-lysosomal thiol-protease which catalyzes limited proteolysis of substrates involved in cytoskeletal remodeling and signal transduction (PubMed:19617626, PubMed:21531719, PubMed:2400579). Proteolytically cleaves CTBP1 at 'Asn-375', 'Gly-387' and 'His-409' (PubMed:23707407). Cleaves and activates caspase-7 (CASP7) (PubMed:19617626).

Spastic paraplegia 76, autosomal recessive

SPG76

A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.

None

The disease is caused by variants affecting the gene represented in this entry.

Undergoes calcium-induced successive autoproteolytic cleavages that generate a membrane-bound 78 kDa active form and an intracellular 75 kDa active form. Calpastatin reduces with high efficiency the transition from 78 kDa to 75 kDa calpain forms.

Belongs to the peptidase C2 family.

Ubiquitous.

• Cytoplasm
• Cell membrane
• Translocates to the plasma membrane upon Ca(2+) binding. In granular keratinocytes and in lower corneocytes, colocalizes with FLG and FLG2 (PubMed:21531719).

CANPL1, PIG30, CAPN1, Calpain-1 catalytic subunit, Calcium-activated neutral proteinase 1, Calpain mu-type, Calpain-1 large subunit, Cell proliferation-inducing gene 30 protein, Micromolar-calpain, CANP 1, muCANP

• entrezGene:823
• omim:114220
• swissprot:P07384

Proteins

Neuroscience

81890Da