Literature DB >> 10472534

A simple screening test for fatty acid oxidation defects using whole-blood palmitate oxidation.

L E Seargeant1, K Balachandra, C Mallory, L A Dilling, C R Greenberg.   

Abstract

We report that measurement of whole-blood palmitate oxidation is a rapid and inexpensive screening test for fatty acid oxidation defects. The assay has been adapted from published assays using cultured fibroblasts or isolated white blood cells. Micro whole-blood samples are incubated with tritiated palmitic acid as substrate. The tritiated water produced is proportional to the mitochondrial beta-oxidation of palmitic acid. Patients with confirmed beta-oxidation defects show low whole-blood palmitate oxidation.

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Year:  1999        PMID: 10472534     DOI: 10.1023/a:1005500318194

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


  12 in total

1.  Octanoate and palmitate beta-oxidation in human leukocytes: implications for the rapid diagnosis of fatty acid beta-oxidation disorders.

Authors:  R J Wanders; L Ijlst; E van Elk; J B de Klerk; H Przyrembel
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982

2.  A comparison of [9,10-3H]palmitic and [9,10-3H]myristic acids for the detection of defects of fatty acid oxidation in intact cultured fibroblasts.

Authors:  N J Manning; S E Olpin; R J Pollitt; J Webley
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

3.  Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.

Authors:  C A Stanley; Y K Lieu; B Y Hsu; A B Burlina; C R Greenberg; N J Hopwood; K Perlman; B H Rich; E Zammarchi; M Poncz
Journal:  N Engl J Med       Date:  1998-05-07       Impact factor: 91.245

4.  Diagnosing disorders of fatty acid oxidation.

Authors:  W L Nyhan
Journal:  Clin Chem       Date:  1995-01       Impact factor: 8.327

5.  Fatty acid oxidation in fibroblasts from patients with defects in beta-oxidation and in the respiratory chain.

Authors:  N Venizelos; U von Döbeln; L Hagenfeldt
Journal:  J Inherit Metab Dis       Date:  1998-06       Impact factor: 4.982

6.  Analysis of fatty acid oxidation intermediates in cultured fibroblasts to detect mitochondrial oxidation disorders.

Authors:  M Pourfarzam; J Schaefer; D M Turnbull; K Bartlett
Journal:  Clin Chem       Date:  1994-12       Impact factor: 8.327

7.  Differential diagnosis of hydroxydicarboxylic aciduria based on release of 3H2O from [9,10-3H]myristic and [9,10-3H]palmitic acids by intact cultured fibroblasts.

Authors:  S E Olpin; N J Manning; K Carpenter; B Middleton; R J Pollitt
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

8.  Rapid diagnosis of long chain and medium chain fatty acid oxidation disorders using lymphocytes.

Authors:  M Brivet; A Slama; J M Saudubray; A Legrand; A Lemonnier
Journal:  Ann Clin Biochem       Date:  1995-03       Impact factor: 2.057

9.  Fatty acid oxidation in peripheral blood cells: characterization and use for the diagnosis of defects of fatty acid oxidation.

Authors:  J Schaefer; M Pourfarzam; K Bartlett; S Jackson; D M Turnbull
Journal:  Pediatr Res       Date:  1995-03       Impact factor: 3.756

Review 10.  Disorders of mitochondrial long-chain fatty acid oxidation.

Authors:  R J Pollitt
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

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  4 in total

1.  Statin therapy depresses total body fat oxidation in the absence of genetic limitations to fat oxidation.

Authors:  N M Fisher; K Meksawan; A Limprasertkul; P J Isackson; D R Pendergast; G D Vladutiu
Journal:  J Inherit Metab Dis       Date:  2007-04-05       Impact factor: 4.982

2.  In Vitro Monitoring of the Mitochondrial Beta-Oxidation Flux of Palmitic Acid and Investigation of Its Pharmacological Alteration by Therapeutics.

Authors:  Renata Murgasova; Ester Tor Carreras; Julien Bourgailh
Journal:  Eur J Drug Metab Pharmacokinet       Date:  2018-12       Impact factor: 2.441

3.  Palmitoyl-carnitine production by blood cells associates with the concentration of circulating acyl-carnitines in healthy overweight women.

Authors:  Maria Chondronikola; Rabia Asghar; Xiaojun Zhang; Edgar L Dillon; William J Durham; Zhanpin Wu; Craig Porter; Maria Camacho-Hughes; Yingxin Zhao; Allan R Brasier; Elena Volpi; Melinda Sheffield-Moore; Nicola Abate; Labros Sidossis; Demidmaa Tuvdendorj
Journal:  Clin Nutr       Date:  2016-09-06       Impact factor: 7.324

4.  The distribution of white blood cell fat oxidation in health and disease.

Authors:  D R Pendergast; N M Fisher; K Meksawan; M Doubrava; G D Vladutiu
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982

  4 in total

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