Hereditary cerebral amyloid angiopathy: the amyloid fibrils contain a protein which is a variant of cystatin C, an inhibitor of lysosomal cysteine proteases.

Abstract

Hereditary Cerebral Hemorrhage With Amyloidosis is an autosomal dominant form of amyloidosis restricted to the cerebral vasculature. We have previously demonstrated that the amyloid protein subunit is similar to Cystatin C (or gamma-trace), an inhibitor of lysosomal cysteine proteinases, and homologous to kininogens. High pressure liquid chromatography tryptic fingerprint analysis was developed to distinguish Cystatin C from the amyloid protein. Moreover, we isolated and sequenced tryptic peptides in which the differences were detected. The data prove that the amyloid protein is 10 residues shorter than Cystatin C and has one amino acid substitution at residue 58.

• Authors: J Ghiso, B Pons-Estel, B Frangione
• Journal: Biochemical and biophysical research communications (Biochem Biophys Res Commun) Vol. 136 Issue 2 Pg. 548-54 (Apr 29 1986) ISSN: 0006-291X [Print] United States
• PMID: 3707586 (Publication Type: Comparative Study, Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Amino Acids
• Amyloid
• CST3 protein, human
• Cystatin C
• Cystatins
• Macromolecular Substances
• Peptide Fragments
• Proteins
• Trypsin

Topics

• Amino Acid Sequence
• Amino Acids (analysis)
• Amyloid (analysis, metabolism)
• Amyloidosis (complications, genetics, metabolism)
• Cerebral Hemorrhage (etiology)
• Chromatography, High Pressure Liquid
• Cystatin C
• Cystatins
• Humans
• Macromolecular Substances
• Peptide Fragments (metabolism)
• Proteins (analysis)
• Proteinuria
• Trypsin (metabolism)