Literature DB >> 6894019

Lipoamide dehydrogenase: rapid heat inactivation in platelets of patients with recessively inherited ataxia.

R A Kark, M M Budelli, D M Becker, L P Weiner, A B Forsythe.   

Abstract

The activity of lipoamide dehydrogenase was abnormally heat-labile in homogenized platelets from seven patients with as recessive ataxia conforming to the syndrome of Friedreich ataxia or clinical variants. Taken together, the abnormality and previous findings of low activity and abnormal kinetic properties are compatible with a change in the conformation of the enzyme in these patients.

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Year:  1981        PMID: 6894019     DOI: 10.1212/wnl.31.2.199

Source DB:  PubMed          Journal:  Neurology        ISSN: 0028-3878            Impact factor:   9.910


  2 in total

1.  Normal mitochondrial malic enzyme levels in Friedreich's ataxia fibroblasts.

Authors:  S Chamberlain; P D Lewis
Journal:  J Neurol Neurosurg Psychiatry       Date:  1983-11       Impact factor: 10.154

2.  Cryptic proteolytic activity of dihydrolipoamide dehydrogenase.

Authors:  Ngolela Esther Babady; Yuan-Ping Pang; Orly Elpeleg; Grazia Isaya
Journal:  Proc Natl Acad Sci U S A       Date:  2007-04-02       Impact factor: 11.205

  2 in total

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