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Intrauterine cardiomyopathy and cardiac mitochondrial proliferation in mitochondrial trifunctional protein (TFP) deficiency

โœ Scribed by Ute Spiekerkoetter; Martina Mueller; Eva Cloppenburg; Reinald Motz; Ertan Mayatepek; Burkhard Bueltmann; Christoph Korenke


Book ID
116988368
Publisher
Elsevier Science
Year
2008
Tongue
English
Weight
833 KB
Volume
94
Category
Article
ISSN
1096-7192

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The mitochondrial trifunctional protein (TFP) is a multienzyme complex of the fatty acid beta-oxidation cycle. It is composed of four alpha-subunits (HADHA) harboring long-chain enoyl-CoA hydratase and long-chain L-3-hydroxyacyl-CoA dehydrogenase (LCHAD) and four beta-subunits (HADHB) harboring long