Der einbau von [14C] fettsäuren in muskellipide bei progressiver muskeldystrophie (duchenne)

Kunze, D; Olthopf, D.; Bönsch, G

doi:10.1016/0009-8981(71)90495-5

Cited by 11 publications

(2 citation statements)

References 11 publications

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“…This may reflect an attempt by the tissue to compensate for the accumulation of lysophosphatidylcholine, but, if so, it is not completely successful in the spinal cord. It should be noted that in some human muscle diseases (Kunze et al, 1971(Kunze et al, , 1973, alterations in the activity of lysophosphatidylcholine acyltransferase have been found. The rate of incorporation oflinoleic acid was decreased in muscle but increased in erythrocytes.…”

Section: Discussionmentioning

confidence: 99%

Phospholipid composition and metabolism in mouse muscular dystrophy

Kwok

Austin

1978

Biochemical Journal

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1. The composition and metabolism of phospholipids were studied in various tissues from both normal and dystrophic mice of the 129 ReJ strain. Phospholipids extracted from forebrain, spinal cord, sciatic nerve and plasma were fractionated by t.l.c. and measured. 2. Very significant alterations were found in the choline phospholipids from these tissues, except forebrain. Plasma phosphatidylcholine in the dystrophic mouse was increased by 38%. There was a 2-fold increase in lysophosphatidylcholine in the spinal cord of dystrophic mice. The sciatic nerve showed a marked decrease in sphingomyelin content, which is approximately half of that in the controls. 3. Five enzymes involved in phosphatidylcholine metabolism [namely cholinephosphotransferase (EC 2.7.8.2); phospholipases A (EC 3.1.1.4, EC 3.1.1.32); lysophospholipase (EC 3.1.1.5); lysophosphatidylcholine acyltransferase (EC 2.3.1.23); phospholipase C (EC 3.1.4.3)] were studied in tissue preparations from forebrain, spinal cord, sciatic nerves, gastrocnemius muscles and liver. 4. Activities of phospholipases A and C were significantly increased, about 5-fold and 60% respectively, in gastrocnemius muscle of dystrophic mice compared with controls. Phospholipases A also showed 50% higher activity in the sciatic nerves of dystrophic than of normal mice. Lysophosphatidylcholine acyltransferase activities were significantly increased in the sciatic nerves and spinal cord, by 50-100% over that of the controls. The forebrain and spinal cord from dystrophic mice, however, had only 60% of lysophospholipase activities of that of the normal control. Cholinephosphotransferase activity was unchanged in these tissues from both normal and dystrophic mice. 5. It is suggested that are number of features of mouse muscular dystrophy related to altered membrane structure and function can be rationalized in terms of changes in lipid composition and metabolism.

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Section: Discussionmentioning

confidence: 99%

Phospholipid composition and metabolism in mouse muscular dystrophy

Kwok

Austin

1978

Biochemical Journal

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“…Therefore a direct comparison of our results with the data obtained by Bruce (16) is impossible. Fatty aldehydes are found with the chain length and saturation 16:0, 16:l, 18:O and l a : ] , chiefly in PE with a 40 X share of aldehydes; the total percentage will be shifted.…”

Section: Resu 1 Tsmentioning

confidence: 96%

Fatty Acid Pattern of Lipids in Normal and Dystrophic Human Muscle

Kunze

Reichmann

Egger

et al. 1975

Eur J Clin Investigation

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The fatty acid distribution of the main lipid fractions: triglycerides (TG), phosphatidylcholine (PCh) , phosphatidylethanolamine (PE) , and sphingomyelin (Sph) of muscle from 6 patients with progressive muscular dystrophy (p.m.d.), Duchenne, 8 to 12 years old was estimated and compared with normal controls of different age. In view of the results of several authors about varied fatty acid distribution in immature muscle a third group comprising samples of neonatal muscle was studied. 1 . The fatty acid pattern of the lipid fractions TG, Sph, and PE from muscle of patients with p.m.d. shows no important variation in comparison to normal controls. In contrast to this the fatty acid distribution in PCh is extremely varied: the percentage of 18:2 is decreased and correspondingly the content of 18:l is increased. In view of the high percentage (nearly 10 %) in which linoleic acid is substituted by oleic acid in PCh, effects on the plasma membrane are to be expected. 2 . The fatty acid pattern in neonatal muscle shows in nearly all positions of the fractions TG, Sph, PE, and PCh a different distribution from normal or dystrophic muscle. In view of the most important variation in dystrophic muscle it must be stated that generally 18:2 is decreased. This deficit was replaced by an increase of ail other fatty acids (not only at a substitution by 18:l as given in p.m.d.). Therefore the diminished content of linoleic acid in PCh of neonatal and dystrophic muscle cannot be interpreted as expression of a corresponding or similar lipid metabolism in both tissues. The results were seen as signs of significant qualitative alterations especially of PCh in p.m.d. They were discussed as proof of our thesis that the basic defect in p.m.d. concerns the specific acylation of PCh with linoleic acid.

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Skeletal Muscle

Waku¹

1977

Lipid Metabolism in Mammals

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Der einbau von [14C] fettsäuren in muskellipide bei progressiver muskeldystrophie (duchenne)

Cited by 11 publications

References 11 publications

Phospholipid composition and metabolism in mouse muscular dystrophy

Phospholipid composition and metabolism in mouse muscular dystrophy

Fatty Acid Pattern of Lipids in Normal and Dystrophic Human Muscle

Skeletal Muscle

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