Protein
Probable hydroxyacid-oxoacid transhydrogenase, mitochondrial
Function
Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). L-3-hydroxybutyrate (L-3-OHB) is also a substrate for HOT when using 2-KG as hydrogen acceptor, resulting in the formation of D-2-HG (By similarity).
Similarity
Belongs to the iron-containing alcohol dehydrogenase family. Hydroxyacid-oxoacid transhydrogenase subfamily.
Sequence
MMSKARISSLMKVISSGSCSCPAHSRSSTGGLNVSSENEYAFEMSSSTIRYGPGVSKELGHDLQNLNAKNVCIVTDRNVAKLNSVKVAFDSLTRCGIQYQVYDETRVEPTDQSLLHAAEYARQNKFDSFVAIGGGSVIDTCKVANLFSADREAEFLDYVNAPIGKAKEVNVKLKPLIAVPTTAGTGSETTGVVIFDYKPLHAKTGISSKYLRPQLGLIDPLHTLSQPEKVAAYCGFDVFCHALESFTAIPYTERGSAPMNPNLRPPYQGSNPISDVWARFALKIIRENFISAVFNQDDLKARSNMHLASTMAGVGFGNAGVHLCHGLSYPISGHVKKFVPDGYSGDHPIIPHGLSVVMTAPAVFKFTAASCPERHLEAAELLGANVSKANRNDAGSILSDVVRQYMYKLKIENGLDSLGFTRDDIQSLVKGTLPQERITKLAPRAQTEEDLASLFENSMTVY