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ACADVL Rabbit pAb (bs-5018R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-5018R
英文名称 ACADVL Rabbit pAb
中文名称 酰基辅酶A脱氢酶很长链抗体
别    名 ACAD 6; ACAD6; ACADV_HUMAN; Acadvl; Acyl CoA dehydrogenase very long chain; Acyl Coenzyme A dehydrogenase very long chain; LCACD; mitochondrial; Very long chain specific acyl CoA dehydrogenase; Very long chain specific acyl CoA dehydrogenase mitochondrial; Very long-chain specific acyl-CoA dehydrogenase; VLCAD.  
研究领域 肿瘤  心血管  细胞生物  免疫学  信号转导  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Rat (predicted: Human,Mouse,Rabbit,Pig,Cow,Dog,Horse)
产品应用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 66 kDa
检测分子量
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACADVL: 251-350/655 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 ACADVL (acyl-Coenzyme A dehydrogenase, very long chain) catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. It is specific to esters of long-chain and very long chain fatty acids such as palmitoyl-CoA and stearoyl-CoA. Deficiencies in ACADVL are associated with reduced myocardial fatty acid beta-oxidation and cardiomyopathy.

Function:
Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.

Subunit:
Homodimer.

Subcellular Location:
Mitochondrion inner membrane.

DISEASE:
Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:201475]. ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and 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:
P49748

Gene ID:
37

Database links:

Entrez Gene: 282130 Cow

Entrez Gene: 37 Human

Entrez Gene: 11370 Mouse

Entrez Gene: 25363 Rat

Omim: 609575 Human

SwissProt: P49748 Human

SwissProt: P50544 Mouse

SwissProt: P45953 Rat

Unigene: 437178 Human

Unigene: 18630 Mouse

Unigene: 33319 Rat



产品图片
Paraformaldehyde-fixed, paraffin embedded (rat adrenal gland); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ACADVL) Polyclonal Antibody, Unconjugated (bs-5018R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (rat liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ACADVL) Polyclonal Antibody, Unconjugated (bs-5018R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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