Literature DB >> 3082666

Mitochondrial abnormalities in fibroblast line GM3093 defective in oxidative metabolism.

G Constantopoulos, M A Greenwood, S H Sorrell.   

Abstract

Fibroblast line GM3093 deficient in the activity of the pyruvate dehydrogenase complex, was derived from a patient reported to have an inherited defect affecting the tricarboxylic acid cycle. Our results suggest a generalized defect consisting of few and abnormal mitochondria and low activities of all mitochondrial enzymes examined.

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Year:  1986        PMID： 3082666     DOI： 10.1007/bf01942519

Source DB:  PubMed          Journal:  Experientia        ISSN： 0014-4754


  11 in total

1.  Protein measurement with the Folin phenol reagent.

Authors:  O H LOWRY; N J ROSEBROUGH; A L FARR; R J RANDALL
Journal:  J Biol Chem       Date:  1951-11       Impact factor: 5.157

2.  Abnormal glutamate metabolism in an adult-onset degenerative neurological disorder.

Authors:  A Plaitakis; S Berl; M D Yahr
Journal:  Science       Date:  1982-04-09       Impact factor: 47.728

3.  An improved procedure for the assay of pyruvate dehydrogenase.

Authors:  G B Kresze
Journal:  Anal Biochem       Date:  1979-09-15       Impact factor: 3.365

4.  Friedreich's disease: IV. Reduced mitochondrial malic enzyme activity in heterozygotes.

Authors:  D A Stumpf; J K Parks; W D Parker
Journal:  Neurology       Date:  1983-06       Impact factor: 9.910

5.  Purification of a plasma membrane-bound adenosine triphosphatase from plant roots.

Authors:  T K Hodges; R T Leonard
Journal:  Methods Enzymol       Date:  1974       Impact factor: 1.600

6.  Friedreich ataxia: III. Mitochondrial malic enzyme deficiency.

Authors:  D A Stumpf; J K Parks; L A Eguren; R Haas
Journal:  Neurology       Date:  1982-03       Impact factor: 9.910

7.  Glutamate dehydrogenase deficiency in three patients with spinocerebellar syndrome.

Authors:  A Plaitakis; W J Nicklas; R J Desnick
Journal:  Ann Neurol       Date:  1980-04       Impact factor: 10.422

8.  Optimal conditions for the assay of lipoamide dehydrogenase in homogenized human platelets.

Authors:  D M Becker; W G Knipprath; S L Perlman; C Nissenson; R A Kark
Journal:  Clin Chim Acta       Date:  1982-05-06       Impact factor: 3.786

9.  An inherited defect affecting the tricarboxylic acid cycle in a patient with congenital lactic acidosis.

Authors:  J P Blass; J D Schulman; D S Young; E Hom
Journal:  J Clin Invest       Date:  1972-07       Impact factor: 14.808

10.  Pyruvate dehydrogenase complex activity in normal and deficient fibroblasts.

Authors:  K F Sheu; C W Hu; M F Utter
Journal:  J Clin Invest       Date:  1981-05       Impact factor: 14.808
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  3 in total

1.  Host metabolism regulates intracellular growth of Trypanosoma cruzi.

Authors:  Kacey L Caradonna; Juan C Engel; David Jacobi; Chih-Hao Lee; Barbara A Burleigh
Journal:  Cell Host Microbe       Date:  2013-01-16       Impact factor: 21.023

Review 2.  The biochemical basis of mitochondrial diseases.

Authors:  H R Scholte
Journal:  J Bioenerg Biomembr       Date:  1988-04       Impact factor: 2.945

3.  Biochemical and ultrastructural study of Blastocystis hominis.

Authors:  C H Zierdt; C T Donnolley; J Muller; G Constantopoulos
Journal:  J Clin Microbiol       Date:  1988-05       Impact factor: 5.948
  3 in total

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