Literature DB >> 3119934

Defects of fatty acid oxidation in skeletal muscle.

D M Turnbull1, K Bartlett, N J Watmough, I M Shepherd, H S Sherratt.   

Abstract

Fatty acids are important substrates for resting and exercising skeletal muscle. Defects of fatty acid oxidation result in muscle pain and weakness, and there is often lipid accumulation in muscle fibres. Only a few of the several possible enzyme defects have been found and some of these have responded to therapy. Some techniques for the investigation of fatty acid oxidation are outlined.

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Year:  1987        PMID： 3119934     DOI： 10.1007/bf01812851

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  26 in total

1.  Assay of acyl-CoA dehydrogenases in muscle and liver and identification of four new cases of medium-chain acyl-CoA dehydrogenase deficiency associated with systemic carnitine deficiency.

Authors:  S Zierz; A G Engel; C A Romshe
Journal:  Adv Neurol       Date:  1988

2.  A spectrophotometric procedure for rapid and sensitive measurements of beta-oxidation. Demonstration of factors that can be rate-limiting for beta-oxidation.

Authors:  H Osmundsen; J Bremer
Journal:  Biochem J       Date:  1977-06-15       Impact factor: 3.857

3.  An accurate and sensitive assay of [14C]octanoate oxidation and its application on tissue homogenates and fibroblasts.

Authors:  J H Veerkamp; H T van Moerkerk; J A Bakkeren
Journal:  Biochim Biophys Acta       Date:  1986-03-21

4.  14CO2 production is no adequate measure of [14C]fatty acid oxidation.

Authors:  J H Veerkamp; T B van Moerkerk; J F Glatz; J G Zuurveld; A E Jacobs; A J Wagenmakers
Journal:  Biochem Med Metab Biol       Date:  1986-06

5.  A new simple screening method for the diagnosis of medium chain acyl-CoA dehydrogenase deficiency.

Authors:  A K Bhuiyan; N J Watmough; D M Turnbull; A Aynsley-Green; J V Leonard; K Bartlett
Journal:  Clin Chim Acta       Date:  1987-05-29       Impact factor: 3.786

6.  Short-chain acyl-CoA dehydrogenase deficiency associated with a lipid-storage myopathy and secondary carnitine deficiency.

Authors:  D M Turnbull; K Bartlett; D L Stevens; K G Alberti; G J Gibson; M A Johnson; A J McCulloch; H S Sherratt
Journal:  N Engl J Med       Date:  1984-11-08       Impact factor: 91.245

7.  Fasting hypoglycemia resulting from hepatic carnitine palmitoyl transferase deficiency.

Authors:  P F Bougnères; J M Saudubray; C Marsac; O Bernard; M Odièvre; J Girard
Journal:  J Pediatr       Date:  1981-05       Impact factor: 4.406

8.  Systemic carnitine deficiency simulating recurrent Reye syndrome.

Authors:  A M Glasgow; G Eng; A G Engel
Journal:  J Pediatr       Date:  1980-05       Impact factor: 4.406

9.  Disorders of lipid metabolism in muscle.

Authors:  S Di Mauro; C Trevisan; A Hays
Journal:  Muscle Nerve       Date:  1980 Sep-Oct       Impact factor: 3.217

10.  Intermittent non-ketotic dicarboxylic aciduria in two siblings with hypoglycaemia: an apparent defect in beta-oxidation of fatty acids.

Authors:  E W Naylor; L L Mosovich; R Guthrie; J E Evans; H Tieckelmann
Journal:  J Inherit Metab Dis       Date:  1980       Impact factor: 4.982
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  1 in total

1.  Skeletal muscle mitochondrial beta-oxidation. A study of the products of oxidation of [U-14C]hexadecanoate by h.p.l.c. using continuous on-line radiochemical detection.

Authors:  N J Watmough; A K Bhuiyan; K Bartlett; H S Sherratt; D M Turnbull
Journal:  Biochem J       Date:  1988-07-15       Impact factor: 3.857
  1 in total

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