Literature DB >> 9700598

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

N Venizelos1, U von Döbeln, L Hagenfeldt.   

Abstract

Fatty acid oxidation has been studied with the tritium release assay in cultured fibroblasts from patients with defects in beta-oxidation and in the mitochondrial respiratory chain. Cells from all patients with beta-oxidation defects and cells from 10 of 16 patients with respiratory chain defects showed an impairment of fatty acid oxidation. The result of the tritium release assay is not only dependent on the proper function of the beta-oxidation cycle but is also influenced by the reoxidation of reduced cofactors. The assay can thus be used to study the expression of respiratory chain defects in cultured fibroblasts.

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Year:  1998        PMID： 9700598     DOI： 10.1023/a:1005310809714

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


  17 in total

1.  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

2.  Clinical heterogeneity associated with the mitochondrial DNA T8993C point mutation.

Authors:  F M Santorelli; S C Mak; M E Vazquez-Memije; S Shanske; P Kranz-Eble; K D Jain; D L Bluestone; D C De Vivo; S DiMauro
Journal:  Pediatr Res       Date:  1996-05       Impact factor: 3.756

3.  Determination of protein: a modification of the Lowry method that gives a linear photometric response.

Authors:  E F Hartree
Journal:  Anal Biochem       Date:  1972-08       Impact factor: 3.365

4.  Clinical and biochemical presentation of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.

Authors:  L Hagenfeldt; N Venizelos; U von Döbeln
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

5.  Mitochondropathy presenting with non-ketotic hypoglycaemia as 3-hydroxydicarboxylic aciduria.

Authors:  E Mayatepek; R J Wanders; M Becker; H J Bremer; G F Hoffmann
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

Review 6.  The expanding clinical spectrum of mitochondrial diseases.

Authors:  D C De Vivo
Journal:  Brain Dev       Date:  1993 Jan-Feb       Impact factor: 1.961

Review 7.  New developments in the diagnosis and investigation of mitochondrial fatty acid oxidation disorders.

Authors:  P M Coates
Journal:  Eur J Pediatr       Date:  1994       Impact factor: 3.183

Review 8.  Genetic disorders of mitochondrial fatty acid oxidation.

Authors:  C A Stanley; D E Hale
Journal:  Curr Opin Pediatr       Date:  1994-08       Impact factor: 2.856

9.  Clinical and biochemical variation and family studies in the multiple acyl-CoA dehydrogenation disorders.

Authors:  W J Rhead; J A Wolff; M Lipson; P Falace; N Desai; K Fritchman; A Moon; L Sweetman
Journal:  Pediatr Res       Date:  1987-04       Impact factor: 3.756

10.  Secondary 3-hydroxydicarboxylic aciduria mimicking long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.

Authors:  M J Bennett; M J Weinberger; W G Sherwood; A B Burlina
Journal:  J Inherit Metab Dis       Date:  1994       Impact factor: 4.982
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  9 in total

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

Authors:  L E Seargeant; K Balachandra; C Mallory; L A Dilling; C R Greenberg
Journal:  J Inherit Metab Dis       Date:  1999-08       Impact factor: 4.982

Review 2.  Compromised fatty acid oxidation in mitochondrial disorders.

Authors:  L Hagenfeldt
Journal:  J Inherit Metab Dis       Date:  1998-08       Impact factor: 4.982

3.  Perfluorooctane sulfonate increases β-oxidation of palmitic acid in chicken liver.

Authors:  Marcus Nordén; Ola Westman; Nikolaos Venizelos; Magnus Engwall
Journal:  Environ Sci Pollut Res Int       Date:  2012-03-23       Impact factor: 4.223

4.  An oxygenated metabolite of benzo[a]pyrene increases hepatic β-oxidation of fatty acids in chick embryos.

Authors:  Ola Westman; Maria Larsson; Nikolaos Venizelos; Henner Hollert; Magnus Engwall
Journal:  Environ Sci Pollut Res Int       Date:  2014-01-03       Impact factor: 4.223

5.  Polycyclic aromatic hydrocarbons (PAHs) reduce hepatic β-oxidation of fatty acids in chick embryos.

Authors:  Ola Westman; Marcus Nordén; Maria Larsson; Jessica Johansson; Nikolaos Venizelos; Henner Hollert; Magnus Engwall
Journal:  Environ Sci Pollut Res Int       Date:  2012-12-30       Impact factor: 4.223

6.  Evidence for physical association of mitochondrial fatty acid oxidation and oxidative phosphorylation complexes.

Authors:  Yudong Wang; Al-Walid Mohsen; Stephanie J Mihalik; Eric S Goetzman; Jerry Vockley
Journal:  J Biol Chem       Date:  2010-07-27       Impact factor: 5.157

Review 7.  Metabolism as a complex genetic trait, a systems biology approach: implications for inborn errors of metabolism and clinical diseases.

Authors:  Jerry Vockley
Journal:  J Inherit Metab Dis       Date:  2008-10-05       Impact factor: 4.982

8.  Inflexibility of AMPK-mediated metabolic reprogramming in mitochondrial disease.

Authors:  Dar-Shong Lin; Shu-Huei Kao; Che-Sheng Ho; Yau-Huei Wei; Pi-Lien Hung; Mei-Hsin Hsu; Tsu-Yen Wu; Tuan-Jen Wang; Yuan-Ren Jian; Tsung-Han Lee; Ming-Fu Chiang
Journal:  Oncotarget       Date:  2017-09-01

9.  Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency.

Authors:  Guilhian Leipnitz; Al-Walid Mohsen; Anuradha Karunanidhi; Bianca Seminotti; Vera Y Roginskaya; Desiree M Markantone; Mateus Grings; Stephanie J Mihalik; Peter Wipf; Bennett Van Houten; Jerry Vockley
Journal:  Sci Rep       Date:  2018-01-18       Impact factor: 4.379
  9 in total

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