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Rabbit Anti-MT-ATP6/Gold Conjugated antibody (bs-17865R-Gold)
訂購熱線:400-901-9800
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訂購QQ:  400-901-9800
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-17865R-Gold
英文名稱1 Rabbit Anti-MT-ATP6/Gold Conjugated antibody
中文名稱 膠體金標(biāo)記的ATP6蛋白抗體
別    名 ATP synthase subunit a; ATP6; ATP6_HUMAN; ATPASE6; F-ATPase protein 6; MT-ATP6; MTATP6  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul(10nm  15nm  35nm
研究領(lǐng)域 心血管  細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, )
產(chǎn)品應(yīng)用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 25kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Msx3
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產(chǎn)品介紹 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 cause 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]. 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.

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.
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