PC : pyruvate carboxylase


Description

The PC (pyruvate carboxylase) is a protein-coding gene located on chromosome 11.

The PC gene provides instructions for making pyruvate carboxylase, an enzyme crucial for energy production within cells. This enzyme operates in mitochondria, converting pyruvate to oxaloacetate, a vital step in various cellular processes. In the liver and kidneys, it initiates gluconeogenesis, the generation of glucose, the body's main energy source. This reaction also occurs in the pancreas, regulating insulin secretion, which controls blood glucose levels. In fat-storing tissue, pyruvate carboxylase participates in fat formation (lipogenesis). It's also essential in the nervous system, replenishing building blocks for neurotransmitters and contributing to myelin formation, the protective covering of nerve cells.

Pyruvate carboxylase catalyzes a two-step reaction. First, it carboxylates covalently attached biotin in an ATP-dependent manner. Second, it transfers the carboxyl group to pyruvate. This enzyme plays a key role in glucose synthesis in the liver and kidneys, and lipid synthesis in adipose tissue, liver, and brain.

PC is also known as PCB.

Associated Diseases


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