產品編號 yb-5018R
英文名稱 Anti-ACADVL抗體
中文名稱 酰基*脫氫酶很長鏈抗體
別    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.
Anti-ACADVL抗體   
說 明 書 0.2ml  
研究領域 腫瘤  心血管  細胞生物  免疫學  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, 
產品應用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 66kDa
細胞定位 細胞漿 細胞膜 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACADVL
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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.

PubMed PubMed
產品介紹 background:
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.

Database links:
UniProtKB/Swiss-Prot: P49748.1

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.