產品編號 yb-4048R
英文名稱 Anti-ACADS抗體
中文名稱 酰基*脫氫酶短鏈抗體
別    名 ACAD3; Acyl Coenzyme A dehydrogenase, C2 to C3 short chain; Acyl-CoA dehydrogenase, C2 to C3 short chain; Acyl-CoA dehydrogenase, short chain; Acyl-Coenzyme A dehydrogenase, short chain; AI196007; Bcd-1; Bcd1; Butyryl CoA dehydrogenase; EC 1.3.99.2; SCAD; Short chain acyl CoA dehydrogenase; Short-chain specific acyl-CoA dehydrogenase, mitochondrial; Unsaturated acyl CoA reductase; ACADS_HUMAN.
Anti-ACADS抗體   
說 明 書 0.2ml  
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
交叉反應 Human, Mouse, Rat, Chicken, 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 45kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACADS
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:
ACADS is a homotetramer mitochondrial flavoprotein, which is a member of the acyl CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta oxidation pathway. Mutations in this gene have been associated with Short Chain Acyl CoA Dehydrogenase Deficiency.

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD) [MIM:201470]. It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults.

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