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ADHFE1
ADHFE1 (alcohol dehydrogenase iron containing 1) is a mitochondrial enzyme, also known as hydroxyacid-oxoacid transhydrogenase (HOT) and by the alias FLJ32430. It oxidises gamma-hydroxybutyrate to succinic semialdehyde while reducing 2-ketoglutarate to D-2-hydroxyglutarate. Browse Assay Genie's current range of ADHFE1 research reagents below, or read on for the biology behind the target.
ADHFE1 catalyses the cofactor-independent, reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA). This reaction is coupled to the reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG), so the enzyme works as a transhydrogenase between a hydroxyacid and an oxoacid. D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate are also substrates, although with about ten-fold lower activity.
The ADHFE1 gene encodes hydroxyacid-oxoacid transhydrogenase (EC 1.1.99.24), which is responsible for the oxidation of 4-hydroxybutyrate in mammalian tissues. The protein is located in the mitochondrion, and its UniProt name is hydroxyacid-oxoacid transhydrogenase, mitochondrial. It belongs to the iron-containing alcohol dehydrogenase family, in the hydroxyacid-oxoacid transhydrogenase subfamily, and HGNC places the gene in the alcohol dehydrogenases group.
Because its products include D-2-hydroxyglutarate, ADHFE1 is of interest to researchers studying GHB metabolism and the origin of this metabolite. Orthologues are annotated in mouse and rat (both Adhfe1), which allows the enzyme to be studied in common model systems. Researchers working on the ADHFE1 protein or gene can use this page to find reagents for mitochondrial metabolism studies.
ADHFE1 oxidises gamma-hydroxybutyrate (4-hydroxybutyrate) to succinic semialdehyde in mammalian tissues, making it central to studies of this pathway.
The enzyme reduces 2-ketoglutarate to D-2-hydroxyglutarate, so it is relevant to research on how this metabolite is generated.
As a cofactor-independent, iron-containing alcohol dehydrogenase family member in the mitochondrion, ADHFE1 is a useful subject for studies of transhydrogenase mechanism.
ADHFE1 also acts on 3-hydroxyisobutyrate and L-3-hydroxybutyrate at lower rates, supporting work on hydroxyacid substrate preference.
ADHFE1 is a mitochondrial hydroxyacid-oxoacid transhydrogenase. It reversibly oxidises gamma-hydroxybutyrate to succinic semialdehyde without a free cofactor, coupling this to the reduction of 2-ketoglutarate to D-2-hydroxyglutarate. It is responsible for 4-hydroxybutyrate oxidation in mammalian tissues.
The HGNC name is alcohol dehydrogenase iron containing 1, and FLJ32430 is a listed alias. UniProt calls the protein hydroxyacid-oxoacid transhydrogenase, mitochondrial, often shortened to HOT. Its enzyme classification is EC 1.1.99.24.
Yes. Mouse and rat both have an orthologue named Adhfe1, so rodent models can be used to study the enzyme. Please check the datasheet of any product for its validated reactivity.
Other alcohol dehydrogenases and enzymes of hydroxyglutarate and GHB metabolism with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt Q8IWW8; product specifications from Assay Genie datasheets.
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