Mitochondrial antigens

Autoantibodies to mitochondrial antigens, detectable by IIF on rat kidney sections, are primarily associated with patients with primary biliary cirrhosis. The major antigen is reported to be a component of the pyruvate dehydrogenase enzyme complex. The antibodies belong to the IgG, IgA and IgM classes. The mitochondrial antibodies stain the cytoplasm of cells from different organs, such as thyroid or kidney, that varies with the mitochondrial content of the cells. Distal tubules of kidney and gastric parietal cells show a bright, uniform, cytoplasmic fluorescence, whereas renal proximal tubules show a characteristic coarse, granular, duller staining.

Mitochondrial antibodies are detected in 87-98% of patients with primary biliary cirrhosis. They are also observed in 25-28% of patients with active chronic hepatitis and in 25-30% of patients with cryptogenic cirrhosis. They are only seldom observed in patients with extrahepatic biliary tract obstruction and occur very rarely in normal subjects.

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