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cystatin 3 (cystatin-C, CST3)

Function: - inhibitor of cysteine proteinases - must inhibit protease that hydrolyzes gamma-trace Structure: - homodimer - belongs to the cystatin family Compartment: secreted Expression: - found in various body fluids, including CSF & plasma - expressed in highest levels in epididymis, vas deferens, brain, thymus, & ovary - lowest levels in the submandibular gland Pathology: - defects in CST3 are a cause of amyloidosis type 6 - potential CSF marker for diagnosis of Creutzfeldt-Jakob disease Laboratory: - cystatin C in serum/plasma

Related

cystatin C in serum/plasma cystatin C in urine

General

cystatin

Properties

SIZE: entity length = 146 aa MW = 16 kD COMPARTMENT: cerebrospinal fluid milk synovial fluid urine semen MOTIF: signal sequence {1-26} glycine residue {37} Secondary area of contact {81-85} cysteine residue {C99} MODIFICATION: cysteine residue {C109} cysteine residue {C109} MODIFICATION: cysteine residue {C99} cysteine residue {C123} MODIFICATION: cysteine residue {C143} cysteine residue {C143} MODIFICATION: cysteine residue {C123} SECRETED-BY: fibroblast neuroglia INHIBITS: cysteine proteinase

Database Correlations

OMIM correlations MORBIDMAP 604312 UniProt P01034 Pfam PF00031 Entrez Gene 1471 Kegg hsa:1471

References

  1. UniProt :accession P01034
  2. OMIM :accession 105150