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
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: | https://repository.cshl.edu/id/eprint/30426 |
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