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Abstract
Experiments have been performed to determine if fibroblasts from patients with Duchenne
muscular dystrophy (DMD) are defective in a process of membrane repair. Normal and
DMD fibroblasts were treated with phospholipase C from Clostridium perfringens to degrade plasma membrane phosphatidylcholine, and then phosphatidylcholine synthesis
was measured as the incorporation of [3H]choline into lipid. Phosphatidylcholine synthesis was stimulated by phospholipase
C treatment to a similar extent in normal and DMD fibroblasts. The activity of CTP:
phosphocholine cytidylyltransferase, the enzyme regulating phosphatidylcholine synthesis
in phospholipase C-treated mammalian cells, was also stimulated to the same extent
in both cell types. The subcellular location of the cytidylyltransferase was changed
by phospholipase C treatment from mostly cytosolic to mostly particulate in both normal
and DMD fibroblasts. It appears, therefore, that at least one type of membrane repair
system functions normally in DMD fibroblasts.
Keywords
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References
- A rapid method of total lipid extraction and purification.Can. J. Physiol. 1959; 37: 911-917
- Duchenne muscular dystrophy — Plasma membrane loss initiates muscle cell necrosis unless it is repaired.Brain. 1979; 102: 147-161
- Linkage analysis of two cloned DNA sequences flanking the Duchenne muscular dystrophy locus on the short arm of the X chromosome.Nucleic Acids Res. 1983; 11: 2303-2312
- Reduced adhesiveness between skin fibroblasts from patients with Duchenne muscular dystrophy.J. Neurol. Sci. 1979; 43: 465-470
- Stimulation of phospholipid metabolism in embryonic muscle cells treated with phospholipase C.in: Proc. Nat. Acad. Sci. (USA). 76. 1979: 4474-4478
- Increased rate of cell-substratum detachment of fibroblasts from patients with Duchenne muscular dystrophy.in: Proc. Nat. Acad. Sci. (USA). 80. 1983: 3086-3090
- Phospholipase C from Clostridium perfringens — Preparation and characterization of homogeneous enzyme.Arch. Biochem. Biophys. 1984; (In the press)
- A simplified assay for phospholipase C.Anal. Biochem. 1979; 97: 43-47
- Protein measurement with the Folin phenol reagent.J. Biol. Chem. 1951; 193: 265-275
- Duchenne dystrophy — Electron microscope findings pointing to a basic or early abnormality in the plasma membrane.Neurol. (Minneap.). 1975; 25: 1111-1120
- Biochemistry of muscle membranes in Duchenne muscular dystrophy.Muscle and Nerve. 1980; 3: 3-20
- Segmented fiber breakdown and defects of the plasmalemma in diseased human muscles.Acta Neuropath. (Berl.). 1975; 33: 129-141
- Elevated creatine kinase MB isoenzyme levels in marathon runners.J. Amer. Med. Ass. 1981; 246: 2049-2056
- Regulation of phosphatidylcholine biosynthesis in cultured chick embryonic muscle treated with phospholipase C.J. Biol. Chem. 1980; 255: 10644-10650
- Regulation of phosphatidylcholine biosynthesis in mammalian cells, Part 1 (Effects of phospholipase C treatment on phosphatidylcholine metabolism in Chinese hamster ovary cells and LM mouse fibroblasts).J. Biol. Chem. 1983; 258: 824-830
- Regulation of phosphatidylcholine biosynthesis in mammalian cells, Part 2 (Effects of phospholipase C treatment on the activity and subcellular distribution of CPT: phosphocholine cytidylyltransferase in Chinese hamster ovary and LM cell lines).J. Biol. Chem. 1983; 258: 831-835
- Regulation of phosphatidylcholine biosynthesis in mammalian cells, Part 3 (Effects of alterations in the phospholipid compositions of Chinese hamster ovary and LM cells in the activity and distribution of CTP: phosphocholine cytidylyltransferase.J. Biol. Chem. 1983; 258: 836-839
- Duchenne muscular dystrophy — A membrane abnormality?.Trends in Biochem. Sci. 1981; (June 1981): ix-xii
Article info
Publication history
Accepted:
January 27,
1984
Received:
October 27,
1983
Footnotes
☆This research was supported in part by a grant from the Muscular Dystrophy Association. E.M. was supported by a scholarship from the Minority Access to Research Careers Program, National Institutes of Health.
☆This is paper number 9661 from the Purdue Agricultural Experiment Station.
Identification
Copyright
© 1984 Published by Elsevier Inc.