TMX1


Description

The TMX1 (thioredoxin related transmembrane protein 1) is a protein-coding gene located on chromosome 14.

Thioredoxin-related transmembrane protein 1 is a protein that in humans is encoded by the TMX1 gene.

TMX1 is a thioredoxin domain-containing protein that participates in various redox reactions through the reversible oxidation of its active center dithiol to a disulfide and catalyzes dithiol-disulfide exchange reactions. It acts as a key inhibitor of the alternative triglyceride biosynthesis pathway by inhibiting the activity of TMEM68/DIESL at the endoplasmic reticulum, thereby restricting accumulation of triacylglycerol. The alternative triglyceride biosynthesis pathway mediates formation of triacylglycerol from diacylglycerol and membrane phospholipids. TMX1 acts as a protein disulfide isomerase by catalyzing formation or reduction of disulfide bonds, specifically mediating formation of disulfide bonds of transmembrane proteins at the endoplasmic reticulum membrane. It is involved in endoplasmic reticulum-associated degradation (ERAD) via its protein disulfide isomerase activity by acting on folding-defective polypeptides at the endoplasmic reticulum membrane. TMX1 acts as a negative regulator of platelet aggregation following secretion in the extracellular space. It acts as a regulator of endoplasmic reticulum-mitochondria contact sites via its ability to regulate redox signals. TMX1 regulates endoplasmic reticulum-mitochondria Ca(2+) flux.

TMX1 is also known as PDIA11, TMX, TXNDC, TXNDC1.

Associated Diseases



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