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Rabbit Anti-ACADL/AF350 Conjugated antibody (bs-10150R-AF350)
订购热线:400-901-9800
订购邮箱:[email protected]
订购传真:010-58129612
技术支持:[email protected]
说 明 书:100ul  
100ul/2980.00元
大包装/询价
产品编号bs-10150R-AF350
英文名称Anti-ACADL/AF350
中文名称AF350标记的酰基辅酶A脱氢酶长链抗体
别    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.  
规格价格100ul/2980元购买        大包装/询价
说 明 书100ul  
研究领域肿瘤  细胞生物  免疫学  转录调节因子  线粒体  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, 
产品应用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    状Lyophilized or Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ACADL
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存条件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.
产品介绍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.

Similarity:
Belongs to the acyl-CoA dehydrogenase family.

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



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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