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Specific protease deficiency in polymorphonuclear leukocytes of Chediak- Higashi syndrome and beige mice

Peripheral blood leukocytes of three patients with Chediak-Higashi syndrome (CHS) contained very low or undetectable levels of elastase, the major neutral protease in these cells. Likewise, peritoneal exudate leukocytes of beige mice (the murine counterpart of CHS) contained correspondingly reduced...

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Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 1978
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2184240/
https://www.ncbi.nlm.nih.gov/pubmed/650154
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collection PubMed
description Peripheral blood leukocytes of three patients with Chediak-Higashi syndrome (CHS) contained very low or undetectable levels of elastase, the major neutral protease in these cells. Likewise, peritoneal exudate leukocytes of beige mice (the murine counterpart of CHS) contained correspondingly reduced levels of their major neutral protease, a serine enzyme of mol wt 27,000. The elastase deficiency in CHS polymorphonuclear leukocytes might account in part for the high incidence of infections in these patients.
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spelling pubmed-21842402008-04-17 Specific protease deficiency in polymorphonuclear leukocytes of Chediak- Higashi syndrome and beige mice J Exp Med Articles Peripheral blood leukocytes of three patients with Chediak-Higashi syndrome (CHS) contained very low or undetectable levels of elastase, the major neutral protease in these cells. Likewise, peritoneal exudate leukocytes of beige mice (the murine counterpart of CHS) contained correspondingly reduced levels of their major neutral protease, a serine enzyme of mol wt 27,000. The elastase deficiency in CHS polymorphonuclear leukocytes might account in part for the high incidence of infections in these patients. The Rockefeller University Press 1978-04-01 /pmc/articles/PMC2184240/ /pubmed/650154 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
Specific protease deficiency in polymorphonuclear leukocytes of Chediak- Higashi syndrome and beige mice
title Specific protease deficiency in polymorphonuclear leukocytes of Chediak- Higashi syndrome and beige mice
title_full Specific protease deficiency in polymorphonuclear leukocytes of Chediak- Higashi syndrome and beige mice
title_fullStr Specific protease deficiency in polymorphonuclear leukocytes of Chediak- Higashi syndrome and beige mice
title_full_unstemmed Specific protease deficiency in polymorphonuclear leukocytes of Chediak- Higashi syndrome and beige mice
title_short Specific protease deficiency in polymorphonuclear leukocytes of Chediak- Higashi syndrome and beige mice
title_sort specific protease deficiency in polymorphonuclear leukocytes of chediak- higashi syndrome and beige mice
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2184240/
https://www.ncbi.nlm.nih.gov/pubmed/650154