Anti-MT-ATP8 antibody
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概述
- 产品描述Mitochondrial membrane ATP synthase (F1F0 ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F1 - containing the extramembraneous catalytic core and F0 - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F1 is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Part of the complex F0 domain. Minor subunit located with subunit a in the membrane (By similarity). Defects in MT-ATP6 are a cause of infantile bilateral striatal necrosis. Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome. disease:Defects in MT-ATP6 are a cause of Leber hereditary optic neuropathy (LHON). LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. disease:Defects in MT-ATP6 are a cause of Leigh syndrome (LS).
- 产品名称Anti-MT-ATP8 antibody
- 种属反应性Human
- 验证应用IHC-P,ELISA
- 抗体类型兔多抗
- 免疫原Synthesized peptide derived from human MT-ATP8
- 偶联Non-conjugated
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性能
- 形态Liquid
- 浓度1 mg/mL.
- 存放说明Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles.
- 存储缓冲液Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 亚型IgG
- 纯化方式Immunogen affinity purified.
- 其它名称
- ATP synthase protein 8 (A6L) (F-ATPase subunit 8) antibody;
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