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Rabbit Anti-SDHAF1/RBITC Conjugated antibody (bs-19603R-RBITC)
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說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-19603R-RBITC
英文名稱 Rabbit Anti-SDHAF1/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標(biāo)記的琥珀酸脫氫酶的組合因子1抗體
別    名 LYR motif containing 8; LYR motif-containing protein 8; LYRM8; mitochondrial; SDH assembly factor 1; SDHAF1; SDHF1_HUMAN; Succinate dehydrogenase assembly factor 1; Succinate dehydrogenase assembly factor 1 mitochondrial; Succinate dehydrogenase complex assembly factor 1.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  線粒體  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse,  (predicted: Rat, Dog, Pig, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 21kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SDHAF1
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
The succinate dehydrogenase (SDH) complex (or complex II) of the mitochondrial respiratory chain is composed of 4 individual subunits. The protein encoded by this gene resides in the mitochondria, and is essential for SDH assembly, but does not physically associate with the complex in vivo. Mutations in this gene are associated with SDH-defective infantile leukoencephalopathy (mitochondrial complex II deficiency).[provided by RefSeq, Mar 2010]

Function:
Plays an essential role in succinate dehydrogenase complex (SDH) assembly, a complex involved in complex II of the mitochondrial electron transport chain. Probably acts by participating in mitochondrial biosynthesis of iron-sulfur centers for complex II.

Subcellular Location:
Mitochondrion matrix.

Tissue Specificity:
Ubiquitously expressed.

DISEASE:
Mitochondrial complex II deficiency

Similarity:
Belongs to the complex I LYR family. SDHAF1 subfamily.

Database links:

Entrez Gene: 644096 Human

Entrez Gene: 68332 Mouse

Entrez Gene: 100912030 Rat

Entrez Gene: 499125 Rat

Omim: 612848 Human

SwissProt: A6NFY7 Human

SwissProt: Q3U276 Mouse

SwissProt: B0K036 Rat

Unigene: 356460 Human

Unigene: 6844 Mouse

Unigene: 3527 Rat



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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