A non-enzymatic function of 17beta-hydroxysteroid dehydrogenase type 10 is required for mitochondrial integrity and cell survival.

Deficiency of the mitochondrial enzyme 2-methyl-3-hydroxybutyryl-CoA dehydrogenase involved in isoleucine metabolism causes an organic aciduria with atypical neurodegenerative course. The disease-causing gene is HSD17B10 and encodes 17beta-hydroxysteroid dehydrogenase type 10 (HSD10), a protein also...

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Bibliographic Details
Main Authors:	Rauschenberger, K, Schöler, K, Sass, J, Sauer, S, Djuric, Z, Rumig, C, Wolf, N, Okun, J, Kölker, S, Schwarz, H, Fischer, C, Grziwa, B, Runz, H, Nümann, A, Shafqat, N, Kavanagh, K, Hämmerling, G, Wanders, R, Shield, J, Wendel, U, Stern, D, Nawroth, P, Hoffmann, G, Bartram, C, Arnold, B
Format:	Journal article
Language:	English
Published:	2010

A non-enzymatic function of 17beta-hydroxysteroid dehydrogenase type 10 is required for mitochondrial integrity and cell survival.

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