Mitochondrial and Cell Death Mechanisms in XXXX .

@entity1 @entity19 @entity191 @entity195 @entity179 @entity746 @entity528
@entity195 ( @entity195 ), @entity191 ( @entity191 ) and @entity746 ( @entity746 ) are the most common @entity1 adult-onset @entity179 . They are characterized by prominent age-related @entity179 in selectively vulnerable neural systems. Some forms of @entity195 , @entity191 , and @entity746 are inherited, and genes causing these diseases have been identified. Nevertheless, the mechanisms of the neuronal cell death are unresolved. Morphological, biochemical, genetic, as well as cell and animal model studies reveal that mitochondria could have roles in this @entity179 . The functions and properties of mitochondria might render subsets of selectively vulnerable neurons intrinsically susceptible to cellular aging and stress and overlying genetic variations, triggering @entity179 according to a cell death matrix theory. In @entity195 , alterations in enzymes involved in oxidative phosphorylation, oxidative damage, and mitochondrial binding of Ab and amyloid precursor protein have been reported. In @entity191 , mutations in putative mitochondrial proteins have been identified and mitochondrial DNA mutations have been found in neurons in the substantia nigra. In @entity746 , changes occur in mitochondrial respiratory chain enzymes and mitochondrial cell death proteins. Transgenic @entity19 models of @entity1 @entity179 are beginning to reveal possible principles governing the biology of selective neuronal vulnerability that implicate mitochondria and the mitochondrial permeability transition pore. This review summarizes how mitochondrial pathobiology might contribute to @entity528 in @entity195 , @entity191 , and @entity746 and could serve as a target for drug therapy.
