Gene Summary: The enzyme encoded by this gene is classified as a thiolesterase and is responsible for the hydrolysis of S-lactoyl-glutathione to reduced glutathione and D-lactate. Two transcript variants encoding different isoforms have been found for this gene.General information above from NCBI
Catalytic activity: S-(2-hydroxyacyl)glutathione + H(2)O = glutathione + a 2-hydroxy carboxylate.
Cofactor: Binds 2 zinc ions per subunit.
Subunit structure: Monomer.
Domain: The CRAL-TRIO domain is known to bind small hydrophobic molecules (By similarity).
Subcellular location: Isoform 1: Mitochondrion matrix.
Isoform 2: Cytoplasm.
Tissue specificity: Expressed in liver and kidney.
Post-translational: Cleaved by CASP3 and CASP7. The potential C-terminal product released by CASP3 cleavage may inhibit the ERK signaling pathway through MAP2K2.
May be ubiquitinated by STUB1.
Involvement in disease: Cerebellar ataxia, cayman type (ATCAY) [MIM:601238]: Found in a population isolate on Grand Cayman Island and causes a marked psychomotor retardation and prominent nonprogressive cerebellar dysfunction including nystagmus, intention tremor, dysarthria, and wide-based ataxic gait. Hypotonia is present from early childhood. Note=The disease is caused by mutations affecting the gene represented in this entry.
Sequence similarity: Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family.
General information above from UniProt