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Liver transplantation for type IV glycogen storage disease

Selby, R and Starzl, TE and Yunis, E and Tzakis, A and Brown, BI and Kendall, RS (1991) Liver transplantation for type IV glycogen storage disease. New England Journal of Medicine, 324 (1). 39 - 42. ISSN 0028-4793

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Abstract

TYPE IV glycogen storage disease is a rare autosomal recessive disorder (also called Andersen's disease1 or amylopectinosis) in which the activity of branching enzyme alpha-1, 4-glucan: alpha-1, 4-glucan 6-glucosyltransferase is deficient in the liver as well as in cultured skin fibroblasts and other tissues.2,3 This branching enzyme is responsible for creating branch points in the normal glycogen molecule. In the relative or absolute absence of this enzyme, an insoluble and irritating form of glycogen, an amylopectin-like polysaccharide that resembles plant starch, accumulates in the cells. The amylopectin-like form is less soluble than normal glycogen, with longer outer and inner chains. © 1991, Massachusetts Medical Society. All rights reserved.


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Details

Item Type: Article
Status: Published
Creators/Authors:
CreatorsEmailPitt UsernameORCID
Selby, R
Starzl, TEtes11@pitt.eduTES11
Yunis, E
Tzakis, A
Brown, BI
Kendall, RS
Centers: Other Centers, Institutes, or Units > Thomas E. Starzl Transplantation Institute
Date: 3 January 1991
Date Type: Publication
Journal or Publication Title: New England Journal of Medicine
Volume: 324
Number: 1
Page Range: 39 - 42
DOI or Unique Handle: 10.1056/nejm199101033240107
Institution: University of Pittsburgh
Refereed: Yes
ISSN: 0028-4793
Other ID: uls-drl:31735062117415, Starzl CV No. 1189
Date Deposited: 08 Apr 2010 17:20
Last Modified: 16 Feb 2019 13:55
URI: http://d-scholarship.pitt.edu/id/eprint/4575

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