Literature DB >> 1206418

Leigh's disease: significance of the biochemical changes in brain.

J V Murphy, L Craig.   

Abstract

Analysis of five brains from patients with Leigh's disease demonstrates an accumulation of thiamine pyrophosphate and a deficiency of thiamine triphosphate. The enzyme which converts thiamine pyrophosphate to thiamine triphosphate was normally active in two of these brains, suggesting that the inhibitor found in Leigh's disease is probably producing the observed neurochemical changes. Reasons for the histological similarity between Leigh's and Wernicke's diseases are suggested.

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Year:  1975        PMID: 1206418      PMCID: PMC492163          DOI: 10.1136/jnnp.38.11.1100

Source DB:  PubMed          Journal:  J Neurol Neurosurg Psychiatry        ISSN: 0022-3050            Impact factor:   10.154


  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.  Subacute necrotizing encephalomyelopathy (Leigh's disease): a consideration of clinical features and etiology.

Authors:  J H Pincus
Journal:  Dev Med Child Neurol       Date:  1972-02       Impact factor: 5.449

3.  Biochemical studies and therapy in subacute necrotizing encephalomyelopathy (Leigh's syndrome).

Authors:  W D Grover; V H Auerbach; M S Patel
Journal:  J Pediatr       Date:  1972-07       Impact factor: 4.406

4.  Efficacy of recommended therapeutic regimens in Leigh's disease.

Authors:  J V Murphy
Journal:  Dev Med Child Neurol       Date:  1974-06       Impact factor: 5.449

5.  Leigh's encephalomyelopathy: an inborn error of gluconeogenesis.

Authors:  F A Hommes; H A Polman; J D Reerink
Journal:  Arch Dis Child       Date:  1968-08       Impact factor: 3.791

6.  Subacute necrotizing encephalomyelopathy (Leigh's disease): detection of the heterozygous carrier state.

Authors:  J V Murphy
Journal:  Pediatrics       Date:  1973-04       Impact factor: 7.124

7.  Role of thiamine triphosphate in subacute necrotizing encephalomyelopathy.

Authors:  J R Cooper; J H Pincus
Journal:  J Agric Food Chem       Date:  1972 May-Jun       Impact factor: 5.279

8.  Ion movements and thiamine. II. The release of the vitamin from membrane fragments.

Authors:  Y Itokawa; J R Cooper
Journal:  Biochim Biophys Acta       Date:  1970

9.  Subacute necrotizing encephalomyelopathy. A review and a study of two families.

Authors:  V J Montpetit; F Andermann; S Carpenter; J S Fawcett; D Zborowska-Sluis; H R Giberson
Journal:  Brain       Date:  1971       Impact factor: 13.501

10.  Thiamine triphosphate deficiency in subacute necrotizing encephalomyelopathy.

Authors:  J R Cooper; Y Itokawa; J H Pincus
Journal:  Science       Date:  1969-04-04       Impact factor: 47.728

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  2 in total

1.  Pyruvate dehydrogenase activity is not deficient in the brain of three autopsied cases with Leigh disease (subacute necrotizing encephalomyelopathy, SNE).

Authors:  B Kustermann-Kuhn; K Harzer; R Schröder; W Permanetter; J Peiffer
Journal:  Hum Genet       Date:  1984       Impact factor: 4.132

2.  Morphometric, subcellular, in vitro fertilisation and embryonic developmental assessment of mouse oocytes produced by anti-inhibin serum or pregnant mare serum gonadotrophin superovulation.

Authors:  Liga Wuri; Cansu Agca; Yuksel Agca
Journal:  Reprod Fertil Dev       Date:  2020-03       Impact factor: 2.311

  2 in total

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