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Immunogen sequence: MNPTLILAA FCLGIASATL TFDHSLEAQW TKWKAMHNRL YGMNEEGWRR AVWEKNVKMI ELHNQEYREG KHSFTMAMNA FGDMTSEEFR QVMNGFQNRK PRKGKVFQEP LFYEAPRSVD WREKGYVTPV KNQGQCGSCW AFSATGALEG QMFRKTGRLI SLSEQNLVDC SGPQGNEGCN GGLMDYAFQY VQDNGGLDSE ESYPYEATEE SCKYNPKYSV ANDTGFVDIP KQEKALMKAV ATVGPISVAI DAGHESFLFY KEGIYFEPDC SSEDMDHGVL VVGYGFESTE SDNNKYWLVK NSWGEEWGMG GYVKMAKDRR NHCGIASAAS YPTV (1-333 aa encoded by BC012612)
The protein encoded by this gene is a lysosomal cysteine proteinase that plays a major role in intracellular protein catabolism. Its substrates include collagen and elastin, as well as alpha-1 protease inhibitor, a major controlling element of neutrophil elastase activity. The encoded protein has been implicated in several pathologic processes, including myofibril necrosis in myopathies and in myocardial ischemia, and in the renal tubular response to proteinuria. This protein, which is a member of the peptidase C1 family, is a dimer composed of disulfide-linked heavy and light chains, both produced from a single protein precursor. At least two transcript variants encoding the same protein have been found for this gene.
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