Phosphate carrier protein, mitochondrial

Phosphate carrier protein, mitochondrial (also known as SLC25A3) is a protein that in humans is encoded by the SLC25A3 gene. This protein is a member of the solute carrier family and is primarily located in the mitochondria, the energy-producing organelles in cells.

Function

The phosphate carrier protein, mitochondrial (SLC25A3) is an integral membrane protein primarily responsible for the transport of phosphate ions from the cytosol into the mitochondrial matrix. This transport is necessary for the ATP (adenosine triphosphate) production, a key process in cellular energy metabolism.

Structure

The SLC25A3 protein is a multi-pass membrane protein, meaning it crosses the mitochondrial membrane multiple times. It consists of three similar domains, each containing two transmembrane segments. The protein's structure allows it to bind to phosphate ions and transport them across the mitochondrial membrane.

Clinical significance

Mutations in the SLC25A3 gene can lead to a deficiency in the phosphate carrier protein, mitochondrial. This deficiency can cause a variety of medical conditions, including mitochondrial diseases and cardiomyopathy, a disease of the heart muscle.

See also

• Solute carrier family
• Mitochondrial disease
• Cardiomyopathy