Phospholipid metabolism in murine muscular dystrophy

Kwok, Chea T.; Kuffer, A.D.; Tang, B.Y.; Austin, L.

doi:10.1016/0014-4886(76)90011-x

Cited by 21 publications

(11 citation statements)

References 28 publications

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“…The elevated activity of phospholipase A in the muscle is also responsible for the enhanced amount of phospholipids in the blood because a higher activity of phospholipase A has been reported in human erythrocytes from patients with DMD and MyD (37). Phospholipase C activity has also been found to increase in the dystrophic mouse model (31). The higher activity of phospholipase C is responsible for the increased quantity of triacylglycerols and sphingomyelin in the muscles of the dystrophic mice (30,39,40), which are further responsible for their accumulation in the blood.…”

Section: Discussionmentioning

confidence: 99%

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High resolution NMR based analysis of serum lipids in Duchenne muscular dystrophy patients and its possible diagnostic significance

et al. 2009

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Proton NMR spectroscopic investigations on the lipid extract of the serum of 41 Duchenne muscular dystrophy (DMD) (age, mean +/- SD; 8.0 +/- 3.0 years) patients and 22 healthy subjects (age, mean +/- SD; 9.0 +/- 4.0 years) were performed in the northern Indian population. The concentration of triglycerides, phospholipids, free cholesterol, cholesterol esters and total cholesterol was significantly higher in DMD patients as compared to healthy subjects. Ratio of free-cholesterol to cholesterol-esters was also significantly higher in DMD patients. Among the individual lipids, concentration of phospholipids was found to be consistently higher in DMD patients compared to healthy subjects, with a discriminatory index of 87.5%. The highest discriminatory index of 92% was found along with the ratio of PL (phospholipids) to CHOL (cholesterol), i.e. PL/CHOL. No significant quantitative difference was observed in the serum lipid constituents of positive and negative gene deletion cases of DMD. The inferences drawn from this study may provide the possibility of the diagnostic importance for DMD, especially in cases where genetic analysis fails to provide the diagnosis.

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Section: Discussionmentioning

confidence: 99%

“…Studies of lipid profile in the dystrophic chicken and mouse model (30)(31)(32)(33)(34) showed high plasma PL concentration in the serum of the animals. Clinical symptoms, family history, Gower's sign and valley sign are helpful in making the first step of the diagnosis in DMD patients.…”

Section: Discussionmentioning

confidence: 99%

High resolution NMR based analysis of serum lipids in Duchenne muscular dystrophy patients and its possible diagnostic significance

et al. 2009

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“…The results also predict an increased production of lysophosphatidyl choline and this is indeed found particularly on the low-PU diet ( Table 3 ) . Kwok et al (1976) enumerated previously observed pathological changes that could be explained on the basis of an increased level of LPC. Unlike muscle and nerve, brain and liver appear relatively free from changes in PL metabolism (Kwok et al, 1976;Kwok and Austin, 1978) and unaltered in the levels of various enzymes of intermediary metabolism (Soothill et al, 1981;Watkins and Watts, 1984).…”

Section: Comparison Of Mutants With Controlsmentioning

confidence: 86%

“…Elegant work by Austin and colleagues supports the latter view. They found enhanced PL turnover in both muscles and nerves (Kwok et al, 1976). Choline phosphotransferase activity was normal in both tissues.…”

Section: Comparison Of Mutants With Controlsmentioning

confidence: 95%

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Gastrocnemius Muscle Lipids in Relation to Diet in Two Mouse Mutants, 129Re‐dy and A2G‐adr, with Abnormal Muscle Function

Rebello

Watts

1985

Journal of Neurochemistry

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The lipids of gastrocnemius muscle from normal and dystrophic (dy) mice of the Bar Harbor, 129Re strain were studied. Animals were fed diets containing either 3.1% or 1.1% of total calories as linoleic acid. Lipid analyses were also done on muscle from a new mouse mutant, A2G-adr, which has abnormal muscle function, characterised by an arrested development of the righting response. These animals were fed the "high" linoleic acid diet only. Total lipid, triacylglycerol, and cholesterol were elevated in the 129Re-dy irrespective of the diet, whereas A2G-adr possessed significantly higher levels of cholesterol. Total phosphorus (micrograms P/g muscle) and cholesterol/phospholipid ratios were elevated in the dy strains only. Cardiolipin was raised in the dy ("low" linoleic diet) and adr muscle, whereas phosphatidylcholine was lower in the adr strain only. Linoleic acid esterified to phosphatidylcholine, phosphatidylethanolamine, and phosphatidylserine was elevated whereas arachidonic acid in phosphatidylserine was decreased in both mutants. Docosahexanoic acid (22:6) in all three dy phospholipids was decreased, independent of dietary treatment. The adr strain possessed normal levels of this fatty acid. The results specifically point to an abnormality in long-chain polyunsaturated fatty acid metabolism in gastrocnemius muscle in the 129Re-dy mutant; in the adr mutant they could reflect an abnormal increase in the number of muscle mitochondria.

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Neurotrophic control of skeletal muscle phospholipids

1979

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The phospholipid composition of rat gastrocnemius muscles was assessed one to nine days after sciatic nerve transection was performed either close to the muscle (5-8 mm from the point of entrance of the nerve into the muscle for short-stump nerve sections) or far from it (30-35 mm central to the nerve's point of entrance for long-stump nerve sections). In both instances, denervation did not cause striking changes in total phospholipid content but resulted in a selective loss of 1,2-diacyl-sn-glycero-3-phosphorylethanolamine (phosphatidyl ethanolamine, GPE). The quantity of GPE in denervated muscles was found to be significantly less than that it controls (p less than 0.01) within one day for short-stump preparations, as compared to three days for long-stump preparations. Also, direct comparison of short- and long-stump GPE values showed significant differences (p less than 0.01) at all times from the first to the fifth day following denervation, with no difference detected thereafter. These results imply that maintenance of skeletal muscle GPE involves a neurogenic influence which is independent of nerve-evoked muscle activity. This conclusion may help us understand neuromuscular diseases in which perturbation of phospholipid components has been implicated.

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Phospholipid metabolism in murine muscular dystrophy

Cited by 21 publications

References 28 publications

High resolution NMR based analysis of serum lipids in Duchenne muscular dystrophy patients and its possible diagnostic significance

High resolution NMR based analysis of serum lipids in Duchenne muscular dystrophy patients and its possible diagnostic significance

Gastrocnemius Muscle Lipids in Relation to Diet in Two Mouse Mutants, 129Re‐dy and A2G‐adr, with Abnormal Muscle Function

Neurotrophic control of skeletal muscle phospholipids

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