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Rabbit Anti-ACADS/Biotin Conjugated antibody (bs-23057R-Bio)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-23057R-Bio
英文名稱 Rabbit Anti-ACADS/Biotin Conjugated antibody
中文名稱 生物素標記的酰基輔酶A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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
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 (predicted: Human, Mouse, Rat, Pig, Cow, )
產品應用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 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 45kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
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.
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.

Similarity:
Belongs to the acyl-CoA dehydrogenase family.

Database links:

Entrez Gene: 35 Human

Entrez Gene: 11409 Mouse

Entrez Gene: 64304 Rat

Omim: 606885 Human

SwissProt: P16219 Human

SwissProt: Q07417 Mouse

SwissProt: P15651 Rat

Unigene: 507076 Human

Unigene: 18759 Mouse

Unigene: 1167 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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