mouse monoclonal IgG2b; Paraplegin Antibody (C-5) is an IgG2b κ mouse monoclonal Paraplegin antibody (also designated Paraplegin antibody) that detects the Paraplegin protein of mouse, rat and human origin by WB, IP, IF and ELISA. Paraplegin Antibody (C-5) is available as both the non-conjugated anti-Paraplegin antibody form, as well as multiple conjugated forms of anti-Paraplegin antibody, including agarose, HRP, PE, FITC and multiple Alexa Fluor® conjugates. Paraplegin, also known as SPG7 (spastic paraplegia protein 7), CAR, CMAR or PGN, is a 795 amino acid metalloprotease that is a member of the AAA protein family. Localized to the mitochrondrial membrane and expressed throughout the body, Paraplegin is a multi-pass membrane protein that is thought to be involved in signal transduction and chaperone-like activities in the mitochrondria. Defects in the gene encoding Paraplegin are the cause of spastic paraplegia type 7 (SPG7), a form of autosomal recessive hereditary spastic paraplegia (AR-HSP). HSPs are degenerative spinal cord disorders that are characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence. Recent studies suggest that SPG7 may be a mitochondrial-based disease, as mutations in the Paraplegin gene lead to ragged-red fibers, oxidase-negative fibers and intense succinate dehydrogenase-stained areas of the mitochrondria. These mitochondrial dysfunctions lead to axonal degeneration and impaired axonal transport, thus causing the neurodegeneration seen in HSPs.

Free sample available. Limit 3 free samples for each full size Santa Cruz Biotechnology primary antibody ordered. All samples must be different. Customer responsible for shipping costs.