产品描述： background: This gene encodes 3-hydroxyacyl-CoA dehydrogenase type II, a member of the short-chain dehydrogenase/reductase superfamily.

The gene product is a mitochondrial protein that catalyzes the oxidation of a wide variety of fatty acids, alcohols, and steroids. The

protein has been implicated in the development of Alzheimer's disease, and mutations in the gene are the cause of 2-methyl-3-

hydroxybutyryl-CoA dehydrogenase deficiency (MHBD). Several alternatively spliced transcript variants have been identified, but the

full-length nature of only two transcript variants has been determined. [provided by RefSeq, Jul 2008].

Function: Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/RG9MTD1,

MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. By interacting with intracellular amyloid-

beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD).

Subcellular Location: Mitochondrion

Tissue Specificity: Expressed in normal tissues but is overexpressed in neurons affected in AD.