In
enzymology, a hydroxyacid-oxoacid transhydrogenase () is an
enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products ...
that
catalyzes the
chemical reaction
A chemical reaction is a process that leads to the IUPAC nomenclature for organic transformations, chemical transformation of one set of chemical substances to another. Classically, chemical reactions encompass changes that only involve the pos ...
:(S)-3-hydroxybutanoate + 2-oxoglutarate
acetoacetate + (R)-2-hydroxyglutarate
Thus, the two
substrates of this enzyme are
(S)-3-hydroxybutanoate and
2-oxoglutarate, whereas its two
products
Product may refer to:
Business
* Product (business), an item that serves as a solution to a specific consumer problem.
* Product (project management), a deliverable or set of deliverables that contribute to a business solution
Mathematics
* Produ ...
are
acetoacetate and
(R)-2-hydroxyglutarate.
This enzyme belongs to the family of
oxidoreductase
In biochemistry, an oxidoreductase is an enzyme that catalyzes the transfer of electrons from one molecule, the reductant, also called the electron donor, to another, the oxidant, also called the electron acceptor. This group of enzymes usually u ...
s, specifically those acting on the CH-OH group of donor with other acceptors. The
systematic name A systematic name is a name given in a systematic way to one unique group, organism, object or chemical substance, out of a specific population or collection. Systematic names are usually part of a nomenclature.
A semisystematic name or semitrivial ...
of this enzyme class is (S)-3-hydroxybutanoate:2-oxoglutarate oxidoreductase. This enzyme is also called transhydrogenase, hydroxy acid-oxo acid.
See also
*
D2HGDH
*
L2HGDH
*
2-hydroxyglutarate synthase
*
2-hydroxyglutarate dehydrogenase
*
Alpha-Hydroxyglutaric acid
α-Hydroxyglutaric acid (2-hydroxyglutaric acid) is an alpha hydroxy acid form of glutaric acid.
In biology
In humans the compound is formed by a hydroxyacid-oxoacid transhydrogenase whereas in bacteria is formed by a 2-hydroxyglutarate synthas ...
*
2-Hydroxyglutaric aciduria
2-hydroxyglutaric aciduria is a rare neurometabolic disorder characterized by the significantly elevated levels of hydroxyglutaric acid in one's urine. It is either autosomal recessive or autosomal dominant.
Presentation
The signs/symptoms of thi ...
References
EC 1.1.99
Enzymes of unknown structure
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