Pathology
A deficiency in any of the enzymes of this complex as well as an inhibition of the complex as a whole is responsible for maple syrup urine disease.
This enzyme is an autoantigen recognized in primary biliary cirrhosis, a form of acute liver failure. These antibodies appear to recognize oxidized protein that has resulted from inflammatory immune responses. Some of these inflammatory responses are explained by gluten sensitivity. Other mitochondrial autoantigens include pyruvate dehydrogenase and branched chain oxoglutarate dehydrogenase, which are antigens recognized by anti-mitochondrial antibodies.
Read more about this topic: Branched-chain Alpha-keto Acid Dehydrogenase Complex
Famous quotes containing the word pathology:
“It is often said that Poland is a country where there is anti-semitism and no Jews, which is pathology in its purest state.”
—Bronislaw Geremek (b. 1932)