Pyruvate Carboxylase

Metabolic Role:

Pyruvate carboxylase is a key enzyme in the TCA cycle and gluconeogenesis. It catalyzes the reaction which turns pyruvate into oxaloacetate, the starting point of the TCA cycle, which is then converted into citrate, the starting point of gluconeogenesis.

Medical Conditions associated with Pyruvate Carboxylase:

Not enough pyruvate carboxylase in the body leads to the conditions known as pyruvate carboxylase deficiency (PCD). This condition begins in neonatal development, and those afflicted generally do not live longer than 6 months. The fatality and sudden onset of this condition is consistent with its central role in metabolism; but still leaves questions as to why this condition allows for significant amounts of growth before becoming fatal. There are no known treatments for this condition, but it is extremely rare, effecting 1 in 250,000 infants.

Source: http://www.emedicine.com/PED/topic1967.htm

Location of Pyruvate Carboxylase:

Pyruvate carboxylase is located mainly in the mitochondria of cells, which is hardly surprising, given its central role in metabolism.

Structure of Pyruvate Carboxylase:

Image courtesy of http://www.biochemj.org/bj/340/0001/bj3400001f02.jpg.

More stuff added later…..