Literature DB >> 3608187

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

A K Bhuiyan, N J Watmough, D M Turnbull, A Aynsley-Green, J V Leonard, K Bartlett.   

Abstract

We describe a simple inexpensive method for the detection of octanoyl-carnitine in urine by reverse-phase high performance thin-layer chromatography of the p-bromophenacyl derivative. This method provides a rapid and reliable means for the diagnosis of medium-chain acyl-CoA dehydrogenase deficiency which is suitable for routine laboratory use.

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Year:  1987        PMID： 3608187     DOI： 10.1016/0009-8981(87)90216-6

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN： 0009-8981            Impact factor:   3.786


  9 in total

Review 1.  Acylcarnitines in intermediary metabolism.

Authors:  A C Sewell; H J Böhles
Journal:  Eur J Pediatr       Date:  1995-11       Impact factor: 3.183

Review 2.  Defects of fatty acid oxidation in skeletal muscle.

Authors:  D M Turnbull; K Bartlett; N J Watmough; I M Shepherd; H S Sherratt
Journal:  J Inherit Metab Dis       Date:  1987       Impact factor: 4.982

3.  Identification of phenylpropionylcarnitine, a new metabolite of phenylpropionic acid, in a patient with medium chain acyl-CoA dehydrogenase deficiency.

Authors:  R Moore; D S Millington; D Norwood; N Kodo; P Robinson; J F Glasgow
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

Review 4.  Mammalian mitochondrial beta-oxidation.

Authors:  S Eaton; K Bartlett; M Pourfarzam
Journal:  Biochem J       Date:  1996-12-01       Impact factor: 3.857

Review 5.  Medium-chain acyl-CoA dehydrogenase deficiency: molecular aspects.

Authors:  Y Matsubara; K Narisawa; K Tada
Journal:  Eur J Pediatr       Date:  1992-03       Impact factor: 3.183

6.  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

7.  One-step synthesis of radioactive acyl-CoA and acylcarnitines using rat liver mitochondrial outer membrane as enzyme source.

Authors:  A K Bhuiyan; S V Pande
Journal:  Lipids       Date:  1992-05       Impact factor: 1.880

8.  Malonyl coenzyme A decarboxylase deficiency.

Authors:  G B MacPhee; R W Logan; J S Mitchell; D W Howells; E Tsotsis; D R Thorburn
Journal:  Arch Dis Child       Date:  1993-10       Impact factor: 3.791

9.  Intramitochondrial control of the oxidation of hexadecanoate in skeletal muscle. A study of the acyl-CoA esters which accumulate during rat skeletal-muscle mitochondrial beta-oxidation of [U-14C]hexadecanoate and [U-14C]hexadecanoyl-carnitine.

Authors:  S Eaton; A K Bhuiyan; R S Kler; D M Turnbull; K Bartlett
Journal:  Biochem J       Date:  1993-01-01       Impact factor: 3.857
  9 in total

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