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FITC標(biāo)記的酰基*脫氫酶長(zhǎng)鏈抗體

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更新時(shí)間：2018-05-08 21:59:24瀏覽次數(shù)：325次

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商鋪產(chǎn)品：9811條

所在地區(qū)：上海上海

聯(lián)系人：馬經(jīng)理 （銷售專員）

產(chǎn)品簡(jiǎn)介

FITC標(biāo)記的酰基*脫氫酶長(zhǎng)鏈抗體產(chǎn)品介紹：This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

詳細(xì)介紹

英文名稱Anti-ACADL/FITC
中文名稱FITC標(biāo)記的酰基*脫氫酶長(zhǎng)鏈抗體
別    名mitochondrial; ACAD4; ACADL; ACADL_HUMAN; Acyl Coenzyme A dehydrogenase long chain; FLJ94052; LCAD; Long chain acyl CoA dehydrogenase; Long-chain specific acyl-CoA dehydrogenase.
說(shuō) 明 書(shū)100ul  
研究領(lǐng)域腫瘤  細(xì)胞生物  免疫學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  線粒體  
抗體來(lái)源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Human, Mouse, Rat, 
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44kDa
性    狀Lyophilized or Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ACADL
亞    型IgG
純化方法affinity purified by Protein A
儲(chǔ) 存 液Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
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 protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq].

Subunit:
Homotetramer.

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting.


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