Tricarboxylate transport protein, mitochondrial

Tricarboxylate transport protein, mitochondrial is a protein that in humans is encoded by the SLC25A1 gene. This protein is a member of the mitochondrial carrier family and functions in the transport of citrate across the inner mitochondrial membrane.

Function

The tricarboxylate transport protein, mitochondrial, also known as the citrate carrier, is an integral membrane protein that exchanges cytosolic citrate with mitochondrial oxaloacetate across the inner mitochondrial membrane. This exchange is a key step in the citric acid cycle, also known as the tricarboxylic acid (TCA) cycle or Krebs cycle, which is a central metabolic pathway in all aerobic organisms.

Structure

The SLC25A1 gene encodes a protein of 298 amino acids with a predicted molecular weight of approximately 33 kDa. The protein has three homologous domains, each containing two transmembrane segments, which are thought to form a pore in the inner mitochondrial membrane.

Clinical significance

Mutations in the SLC25A1 gene have been associated with Combined D-2- and L-2-hydroxyglutaric aciduria, a rare neurometabolic disorder characterized by developmental delay, hypotonia, and seizures. In addition, alterations in the expression of the SLC25A1 gene have been implicated in cancer, as the citrate carrier plays a crucial role in energy production and the synthesis of fatty acids and cholesterol.

See also

• SLC25A1
• Mitochondrial carrier
• Citric acid cycle