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Journal article

Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency.

Abstract:

Short-chain hydroxyacyl CoA dehydrogenase deficiency is an ill-defined, severe pediatric disorder of mitochondrial fatty acid beta-oxidation of short-chain hydroxyacyl CoAs. To understand the relative contributions of the two known short-chain hydroxyacyl CoA dehydrogenases (HADH) tissue biopsies of six distinct family individuals were analyzed and kinetic parameters were compared. Steady-state kinetic constants for HADH 1 and HADH 2 suggest that type 1 is the major enzyme involved in mitocho...

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Publication status:
Published

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Publisher copy:
10.1016/j.bbrc.2007.10.188

Authors


Filling, C More by this author
Hirschberg, D More by this author
Marschall, HU More by this author
Jörnvall, H More by this author
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Journal:
Biochemical and biophysical research communications
Volume:
368
Issue:
1
Pages:
6-11
Publication date:
2008-03-05
DOI:
EISSN:
1090-2104
ISSN:
0006-291X
URN:
uuid:13869920-1dd8-45c9-9853-b8216d5ef589
Source identifiers:
108499
Local pid:
pubs:108499

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