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Rabbit Anti-SDHA/Gold Conjugated antibody (bs-3970R-Gold)
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-3970R-Gold
英文名稱 Rabbit Anti-SDHA/Gold Conjugated antibody
中文名稱 膠體金標(biāo)記的琥珀酸脫氫酶復(fù)合體亞基A抗體
別    名 mitochondrial; DHSA_HUMAN; Flavoprotein subunit of complex II; Fp; SDH 2; SDH2; SDHA; SDHF; Succinate dehydrogenase [ubiquinone] flavoprotein subunit; Succinate dehydrogenase [ubiquinone] flavoprotein subunit mitochondrial; Succinate dehydrogenase complex flavoprotein subunit precursor; Succinate dehydrogenase complex subunit A; Succinate Dehydrogenase Complex subunit A Flavoprotein.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領(lǐng)域 腫瘤  細胞生物  免疫學(xué)  信號轉(zhuǎn)導(dǎo)  細胞凋亡  轉(zhuǎn)錄調(diào)節(jié)因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse,  (predicted: Rat, )
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 70kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SDHA
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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:
In aerobic respiration reactions, succinate dehydrogenase (SDH) catalyzes the oxidation of succinate and ubiquinone to fumarate and ubiquinol. Four subunits comprise the SDH protein complex: a flavochrome subunit (SDHA), an iron-sulfur protein (SDHB), and two membrane-bound subunits (SDHC and SDHD) anchored to the inner mitochondrial membrane. Mutations to these subunits cause mitochondrial dysfunction, corresponding to several distinct disorders. Mutations in the membrane bound components may cause hereditary paraganglioma, while SDHA mutations are associated with juvenile encephalopathy as well as Leigh Syndrome, a severe neurological disorder. Inactivating mutations in SDHB correlate with inherited, but not necessarily sporadic, cases of pheochromocytoma.

Function:
Defects in SDHA are a cause of mitochondrial complex II deficiency (MT-C2D). A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome.

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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