PMID: 6168726Sep 1, 1981Paper

A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin

The Journal of Experimental Medicine
M PrasB Frangione

Abstract

Amyloid fibrils were isolated from spleen and thyroid obtained at autopsy from one patient (S.K.O.) of Jewish origin with familial amyloidotic polyneuropathy. Gel filtration on Sephadex G100 after solubilization in 5 M guanidine HCl yielded three major components with 14,000, 9,000, and 5,000 mol wt, respectively. The two larger components shared antigenic determinants with human prealbumin. Amino acid analysis and amino terminal sequence studies revealed the 14,000-mol wt protein to be an intact prealbumin subunit. The 9,000-mol wt fragment obtained in highest yield encompassed the region from position 49-127 and the 5,000 mol wt fraction encompassed the amino terminal of prealbumin (position 1-48). An amino acid substitution (Gly/Thr) was detected at position 49, where enzymatic cleavage occurred. Thus, several prealbumin-derived fragments, predominantly the carboxyl end, constitute the amyloid fibrils in a heredofamilial amyloidosis syndrome of dominant inheritance.

References

Sep 1, 1978·Proceedings of the National Academy of Sciences of the United States of America·P P CostaF R Bravo
Oct 1, 1972·The Journal of Clinical Investigation·M LevinM Pras
Nov 1, 1970·Arthritis and Rheumatism·C AndradeM W Van Allen
Apr 1, 1968·The Journal of Clinical Investigation·M PrasE C Franklin
Jun 5, 1980·The New England Journal of Medicine·G G Glenner

❮ Previous
Next ❯

Citations

Dec 1, 1989·Journal of the Neurological Sciences·G G Glenner, M A Murphy
Jan 1, 1986·Molecular Immunology·F E DwuletM D Benson
Oct 24, 1987·Lancet·G J CooperJ B Rothbard
Jan 1, 1983·Proceedings of the National Academy of Sciences of the United States of America·M PrasB Frangione
Feb 1, 1984·Proceedings of the National Academy of Sciences of the United States of America·F E Dwulet, M D Benson
Feb 1, 1987·Journal of Clinical Pathology·G G CornwellP Westermark
Mar 1, 1984·American Journal of Ophthalmology·B H DoftA S Cohen
May 30, 2013·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Elisabet IhsePer Westermark
Oct 1, 1982·The American Journal of Medicine·R MornaghiE C Franklin
Mar 1, 1986·Scandinavian Journal of Immunology·G Husby, K Sletten
Jun 1, 1986·Scandinavian Journal of Immunology·A HusebekkK Nordstoga
Aug 1, 1987·Acta Neurologica Scandinavica·O JenssonB Frangione
Jan 1, 1988·Scandinavian Journal of Immunology·M NordlieP J Ranløv
Dec 1, 1985·Scandinavian Journal of Immunology·F E DwuletM D Benson
May 1, 1989·Acta Neurologica Scandinavica·C M ChangC Y Huang
Jan 1, 1984·Scandinavian Journal of Rheumatology·C P Maury
Dec 15, 1987·Biochemistry·F PrelliB Frangione
Oct 1, 1985·Arthritis and Rheumatism·A SigsbeeD N Glass
Jun 6, 2021·Cardiology and Therapy·Haruki KoikeMasahisa Katsuno
Aug 1, 1988·Seminars in Arthritis and Rheumatism·J Varga, J R Wohlgethan
Nov 1, 1985·Muscle & Nerve·A H KoeppenR O Bailey
Mar 15, 1988·Biochemical and Biophysical Research Communications·R L MartoneE A Schon

❮ Previous
Next ❯

Related Concepts

Related Feeds

Amyloidogenesis-associated Polyneuropathy

Amyloid deposits around nerves can damage and disrupt smooth information processing between the brain and organs. Here is the latest research on amyloidosis-associated polyneuropathies.

© 2021 Meta ULC. All rights reserved