ATP6蛋白抗体
规格：1mg/1ml
英文名： MT-ATP6
别名： ATP synthase subunit a; ATP6; ATP6_HUMAN; ATPASE6; F-ATPase protein 6; MT-ATP6; MTATP6
分子量： 25kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Msx3
交叉反应：Human,
细胞定位：细胞核 细胞膜
ATP6蛋白抗体产品介绍：background: Mitochondrial membrane ATP synthase (F(1)F(0) 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, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Key component of the proton channel; it may play a direct role in the translocation of protons across the membrane. Subcellular Location: Mitochondrion inner membrane. DISEASE: Defects in MT-ATP6 are the causeATP6蛋白抗体 of neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP) [MIM:551500]. Defects in MT-ATP6 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. 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. Defects in MT-ATP6 are a cause of Leigh syndrome (LS) [MIM:256000]. ATP6蛋白抗体LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in MT-ATP6 are a cause of mitochondrial infantile bilateral striatal necrosis (MIBSN) [MIM:500003]. Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome. Similarity: Belongs to the ATPase A chain family. Gene ID: 4508 Database links: Entrez Gene: 4508 Human Omim: 516060 Human SwissProt: P00846 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
ATP6蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：心血管 细胞生物 **学 神经生物学 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid