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MT-ATP6
ATP synthase Fo subunit 6 (or subunit/chain A) (human mitochondrial gene name ATP6) is a subunit of Fo complex of transmembrane F-type ATP synthase. The MT-ATP6 gene provides information for making a protein that is essential for normal mitochondrial function. The MT-ATP6 protein forms one part (subunit) of a large enzyme called ATP synthase. This enzyme, which is also known as complex V, is responsible for the final step of oxidative phosphorylation. Specifically, one segment of ATP synthase allows positively charged particles, called protons, to flow across a specialized membrane inside mitochondria. Another segment of the enzyme uses the energy created by this proton flow to convert a molecule called adenosine diphosphate (ADP) to ATP. Mutations in the MT-ATP6 gene have been found in approximately 10 to 20 percent of people with Leigh syndrome. Leigh syndrome is a progressive brain disorder that usually appears in infancy or early childhood. Affected children may experience delayed development, muscle weakness, problems with movement, or difficulty breathing.〔(【引用サイトリンク】 work = Genetics Home Resource )〕
== Structure ==
The MT-ATP6 gene, located in mitochondrial DNA, is 681 base pairs in length.〔(【引用サイトリンク】 url = http://www.ncbi.nlm.nih.gov/gene/4508 )〕 The MT-ATP6 protein weighs 24.8 kDa and is composed of 226 amino acids.〔(【引用サイトリンク】 work = Cardiac Organellar Protein Atlas Knowledgebase (COPaKB) )〕 The protein is a subunit of the F1Fo ATPase, also known as Complex V, which consists of 14 nuclear and 2 mitochondrial -encoded subunits. As an A subunit, MT-ATP6 is contained within the non-catalytic, transmembrane Fo portion of the complex.〔 The nomenclature of the enzyme has a long history. The F1 fraction derives its name from the term "Fraction 1" and Fo (written as a subscript letter "o", not "zero") derives its name from being the binding fraction for oligomycin, a type of naturally-derived antibiotic that is able to inhibit the Fo unit of ATP synthase. The Fo region of ATP synthase is a proton pore that is embedded in the mitochondrial membrane. It consists of three main subunits A, B, and C, and (in humans) six additional subunits, d, e, f, g, F6, and 8 (or A6L). 3D structure of E. coli homologue of this subunit was modeled based on electron microscopy data (chain M of ). It forms a transmembrane 4-α-bundle.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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