MPC1

MPC1 (Mitochondrial Pyruvate Carrier 1) is a protein that in humans is encoded by the MPC1 gene. It is a key component of the mitochondrial pyruvate carrier (MPC) complex, which is responsible for the transport of pyruvate into the mitochondria.

Function

MPC1, along with its partner protein MPC2, forms the mitochondrial pyruvate carrier complex. This complex is located in the inner mitochondrial membrane and facilitates the transport of pyruvate from the cytosol into the mitochondrial matrix. Once inside the mitochondria, pyruvate can be used in the citric acid cycle (also known as the Krebs cycle) to generate adenosine triphosphate (ATP), the cell's main source of energy.

Clinical significance

Mutations in the MPC1 gene can lead to a deficiency in the MPC complex, which can result in a variety of metabolic disorders. These include lactic acidosis, hyperpyruvatemia, and Leigh syndrome, a severe neurological disorder.

Research

Research into the function and regulation of the MPC complex, and the role of MPC1 in particular, is ongoing. This research has potential implications for understanding and treating a range of diseases, including cancer, diabetes, and neurodegenerative diseases.

See also

• MPC2
• Mitochondrial pyruvate carrier
• Citric acid cycle
• Leigh syndrome