See also
Caseous necrosis
Coagulative necrosis
Liquefactive necrosis
Myospherulosis
Necrosis

References
External links

Fat Necrosis of the breast
Pyruvate carboxylase deficiency is an inherited disorder that causes lactic acid to accumulate in the blood. High levels of these substances can damage the bodys organs and tissues, particularly in the nervous system. Pyruvate carboxylase deficiency is a rare condition, with an estimated incidence of 1 in 250,000 births worldwide. Type A of the disease appears to be much more common in some Algonkian Indian tribes in eastern Canada, while the type B disease is more present in European populations. Signs and symptoms
Pyruvate carboxylase deficiency causes lactic acidosis and hyperammonaemia. Lactic acidosis may then lead to liver failure, hepatomegaly, reduced ketone body synthesis, and demyelination of neurons. Genetics
Pyruvate carboxylase deficiency is caused by mutations in the PC gene. The PC gene provides instructions for making an enzyme called pyruvate carboxylase. This condition is inherited in an autosomal recessive pattern, which means two copies of the gene must be inherited for the disorder to be present. The parents of an individual with an autosomal recessive disorder are carriers of one copy of the altered gene, but do not show signs and symptoms of the disorder. Mechanism
Pyruvate carboxylase is active in mitochondria in cells. It is involved in the generation of glucose through gluconeogenesis. By generating oxaloacetate, it catalyses an important anaplerotic reaction that maintains the citric acid cycle to generate energy by aerobic respiration. Pyruvate carboxylase also plays a role in the formation of the myelin sheath that surrounds certain nerve cells, and the production of neurotransmitters for communication between neurons.