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A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin
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...
Formato: | Texto |
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Lenguaje: | English |
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The Rockefeller University Press
1981
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2186473/ https://www.ncbi.nlm.nih.gov/pubmed/6168726 |
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collection | PubMed |
description | 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. |
format | Text |
id | pubmed-2186473 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 1981 |
publisher | The Rockefeller University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-21864732008-04-17 A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin J Exp Med Articles 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. The Rockefeller University Press 1981-09-01 /pmc/articles/PMC2186473/ /pubmed/6168726 Text en This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/4.0/). |
spellingShingle | Articles A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin |
title | A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin |
title_full | A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin |
title_fullStr | A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin |
title_full_unstemmed | A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin |
title_short | A variant of prealbumin from amyloid fibrils in familial polyneuropathy of Jewish origin |
title_sort | variant of prealbumin from amyloid fibrils in familial polyneuropathy of jewish origin |
topic | Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2186473/ https://www.ncbi.nlm.nih.gov/pubmed/6168726 |