TSTD1
Description
The TSTD1 (thiosulfate sulfurtransferase like domain containing 1) is a protein-coding gene located on chromosome 1.
The TSTD1 gene encodes the enzyme thiosulfate:glutathione sulfurtransferase (TST), which plays a crucial role in hydrogen sulfide (H2S) metabolism. TST catalyzes the production of S-sulfanylglutathione (GSS-), a key intermediate in this process. It bridges the first step of H2S metabolism, catalyzed by sulfide:quinone oxidoreductase (SQOR), which converts H2S to thiosulfate, and the subsequent step involving sulfur dioxygenase (SDO) that utilizes GSS- as a substrate. The thermodynamic balance between the irreversible SDO reaction and the reversible TST reaction creates a mechanism for the physiological reaction where thiosulfate acts as a sulfane donor.
TSTD1 is also known as KAT, TST.
Associated Diseases
- primary familial polycythemia due to EPO receptor mutation
- breast cancer
- erythrocytosis, familial, 3
- erythrocytosis, familial, 6
- hemolytic anemia due to diphosphoglycerate mutase deficiency
- alpha thalassemia-intellectual disability syndrome type 1
- erythrocytosis, familial, 4
- hereditary persistence of fetal hemoglobin-sickle cell disease syndrome
- hemolytic anemia due to adenylate kinase deficiency
- Heinz body anemia
- X-linked sideroblastic anemia 1
- delta-beta-thalassemia
- hemoglobin E-beta-thalassemia syndrome
- familial isolated congenital asplenia
