*611083
ALCOHOL DEHYDROGENASE, IRON-CONTAINING, 1; ADHFE1

Alternative titles; symbols
HYDROXYACID-OXOACID TRANSHYDROGENASE; HOT
ALCOHOL DEHYDROGENASE 8; ADH8

HGNC Approved Gene Symbol: ADHFE1

Cytogenetic location: 8q13.1     Genomic coordinates (GRCh37): 8:67,344,717 - 67,381,043 (from NCBI)

TEXT
Description
The ADHFE1 gene encodes hydroxyacid-oxoacid transhydrogenase (EC 1.1.99.24), which is responsible for the oxidation of 4-hydroxybutyrate in mammalian tissues (Kardon et al., 2006).

Cloning
Deng et al. (2002) cloned a novel iron-containing alcohol dehydrogenase (ADH) cDNA, designated ADHFE1, from a human fetal brain cDNA library. ADHFE1 encodes a deduced 467-amino acid protein with a predicted molecular mass of 50 kD. The protein shares 21 to 38% sequence identity with the type III ADHs of microbial organisms and 88%, 67%, and 53% identity with homologs in mouse, Drosophila, and C. elegans, respectively. Northern blot analysis detected a 2.4-kb transcript in liver only. MTC-panel screening PCR detected expression in adult liver, kidney, thymus, and spleen.

Gene Function
Kardon et al. (2006) demonstrated that overexpression of mouse Adhfe1 in human embryonic kidney cells catalyzed the oxidation of 4-hydroxybutyrate to succinate semialdehyde. Adhfe1 enzyme activity required both 4-hydroxybutyrate and alpha-ketoglutarate.

Gene Structure
Deng et al. (2002) determined that the ADHFE1 gene contains 14 exons and spans more than 36 kb.

Mapping
By genomic sequence analysis, Deng et al. (2002) mapped the ADHFE1 gene to chromosome 8q12.3-q13.1. By the same method, Kardon et al. (2006) mapped the gene to 8q13.1.

REFERENCES
1. Deng, Y., Wang, Z., Gu, S., Ji, C., Ying, K., Mao, Y. Cloning and characterization of a novel human alcohol dehydrogenase gene (ADHFe1). DNA Seq. 13: 301-306, 2002. [PubMed: 12592711, related citations] [Full Text: Pubget]

2. Kardon, T., Noel, G., Vertommen, D., Van Schaftingen, E. Identification of the gene encoding hydroxyacid-oxoacid transhydrogenase, an enzyme that metabolizes 4-hydroxybutyrate. FEBS Lett. 580: 2347-2350, 2006. [PubMed: 16616524, related citations] [Full Text: Elsevier Science, Pubget]

Contributors: Carol A. Bocchini - updated : 06/06/2007
Creation Date: Dorothy S. Reilly : 6/5/2007
Edit History: carol : 06/06/2007