Protein degradation in skin fibroblasts from patients with Duchenne muscular dystrophy

Statham, H. E., Witkowski, J. A., Dubowitz, V. (October 1980) Protein degradation in skin fibroblasts from patients with Duchenne muscular dystrophy. The Biochemical Journal, 192 (1). pp. 257-62. ISSN 0264-6021 (Print)0264-6021

URL: http://www.ncbi.nlm.nih.gov/pubmed/7305898

Abstract

The rates of degradation of [3H]leucine-labelled proteins have been measured in cultures of skin fibroblasts obtained from normal controls (five subjects) and patients with Duchenne muscular dystrophy (six subjects). Cultures were incubated with [3H]leucine (10 microCi/ml) for 60 min to label "short-lived" proteins, and with [3H]leucine (5 microCi/ml) for 60 h to label "long-lived" proteins. Optimal wash procedures were devised for removal of [3H]leucine from the extracellular space and from cell pools before beginning degradation measurements. Re-utilization of [3H]leucine released from degraded labelled proteins was prevented by supplementing the medium with 4mM-leucine. Rates of degradation did not depend on the growth state of the cells or on cell age over the range used (passages eight-20). Degradation of long-lived proteins was approximately linear over a 24h period, at a rate of 1.0% per h. 30% of short-lived protein was degraded within 6h. No differences were observed between protein degradation in normal fibroblasts and in those from patients with Duchenne muscular dystrophy.

Item Type: Paper
Uncontrolled Keywords: Adolescent Adult Cell Cycle Cells, Cultured Child Child, Preschool Fibroblasts/metabolism Humans Infant Leucine/metabolism Muscular Dystrophies/*metabolism Proteins/*metabolism Skin/cytology/*metabolism
Subjects: organs, tissues, organelles, cell types and functions > cell types and functions > cell types > fibroblasts
organs, tissues, organelles, cell types and functions > cell types and functions > cell types > fibroblasts
organs, tissues, organelles, cell types and functions > cell types and functions > cell types > fibroblasts

diseases & disorders > congenital hereditary genetic diseases > muscular dystrophy
CSHL Authors:
Communities: Banbury Center
Depositing User: Matt Covey
Date: 15 October 1980
Date Deposited: 01 Dec 2014 16:13
Last Modified: 01 Dec 2014 16:13
PMCID: PMC1162329
Related URLs:
URI: http://repository.cshl.edu/id/eprint/30426

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