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PMID: 2157434 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Amyloid protein in familial amyloidosis (Finnish type) is homologous to gelsolin, an actin-binding protein.

Biochemical and biophysical research communications ·Vol. 167 ·No. 3 ·1990-03-30 ·Pages 927-32

Haltia M, Prelli F, Ghiso J, Kiuru S, Somer H, Palo J, Frangione B

Abstract

Familial amyloidosis, Finnish type, is clinically characterized by cranial neuropathy and lattice corneal dystrophy. It is an autosomal dominant form of systemic amyloidosis with small deposits of congophilic material occurring in most tissues, particularly in association with blood vessel walls and basement membranes. Amyloid fibrils were extracted from the kidney of patient VUO, and rabbit antiserum raised against the 12 kDa purified amyloid subunit displayed strong immunohistochemical reactivity with the amyloid deposits. The amino terminal sequence of this 12 kDa amyloid protein (ATEVPVSWESFNNGD) showed homology with gelsolin (or actin depolymerizing factor), a 93 kDa plasma protein. The amyloid peptide is a degradation product, starting at position 173, of the gelsolin molecule.

MeSH Terms
Actins/metabolism Amino Acid Sequence Amyloid/genetics,isolation & purification Amyloidosis/genetics,metabolism Calcium-Binding Proteins/genetics Chromatography, Gel Electrophoresis, Polyacrylamide Gel Finland Gelsolin Humans Immunoblotting Kidney/analysis Macromolecular Substances Microfilament Proteins/genetics Molecular Sequence Data Molecular Weight Sequence Homology, Nucleic Acid
Chemicals
Actins Amyloid Calcium-Binding Proteins Gelsolin Macromolecular Substances Microfilament Proteins
Authors & Affiliations
7 authors, click to expand affiliations / ORCID
Haltia M
Department of Pathology, New York University Medical Center, New York 10016.
Prelli F
Ghiso J
Kiuru S
Somer H
Palo J
Frangione B
Article Info
Journal
Biochemical and biophysical research communications
Abbr.
Biochem Biophys Res Commun
ISSN
0006-291X
Published
1990-03-30
Pages
927-32
Language
English
Region
United States
NLM ID
0372516
Subset
IM
Grants
NIA NIH HHS · AG 05891 · United States
NIAMS NIH HHS · AR01431 · United States
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