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ACADL Rabbit pAb (bs-5015R)  
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產品編號 bs-5015R
英文名稱 ACADL Rabbit pAb
中文名稱 ?;o酶A脫氫酶長鏈抗體
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.  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 (predicted: Human,Mouse,Rat)
產品應用 WB=1:500-2000,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 44 kDa
檢測分子量
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACADL: 201-300/430 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

Similarity:
Belongs to the acyl-CoA dehydrogenase family.

SWISS:
P28330

Gene ID:
33

Database links:

Entrez Gene: 33 Human

Entrez Gene: 614508 Cow

Entrez Gene: 11363 Mouse

Entrez Gene: 396931 Pig

Entrez Gene: 25287 Rat

Omim: 609576 Human

SwissProt: P28330 Human

SwissProt: P51174 Mouse

SwissProt: P79274 Pig

SwissProt: P15650 Rat

Unigene: 471277 Human

Unigene: 2445 Mouse

Unigene: 174 Rat



產品圖片
Sample: Lane 1: Human Jurkat cell lysates Lane 2: Human HeLa cell lysates Lane 3: Human HepG2 cell lysates Primary: Anti-ACADL (bs-5015R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 44 kDa Observed band size: 48 kDa
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