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Rabbit Anti-ACADM/BF488 Conjugated antibody (bs-4047R-BF488)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-4047R-BF488
英文名稱 Rabbit Anti-ACADM/BF488 Conjugated antibody
中文名稱 BF488標記的酰基輔酶A脫氫酶中鏈抗體
別    名 mitochondrial antibody; ACAD 1; ACAD1; Acyl coenzyme A dehydrogenase; Acyl coenzyme A dehydrogenase C 4 to C 12 straight chain; MCAD; MCADH; Medium chain acyl CoA dehydrogenase; Medium chain fatty acyl CoA dehydrogenase; Medium chain specific acyl CoA dehydrogenase; Medium chain specific acyl CoA dehydrogenase mitochondrial; FLJ18227; FLJ93013; FLJ99884; ACADM_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, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 47kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACADM
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:
ACADM protein is a medium chain specific (C4 to C12 straight chain) acyl Coenzyme A dehydrogenase. The enzyme catalyzes the initial step of the mitochondrial fatty acid beta oxidation pathway. ACADM expression is induced during periods of fasting, when reliance on fatty acids for energy is increased. Clinical phenotypes are associated with ACADM hereditary deficiency.

Function:
This enzyme is specific for acyl chain lengths of 4 to 16.

Subunit:
Homotetramer. Interacts with the heterodimeric electron transfer flavoprotein ETF.

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Acyl-CoA dehydrogenase medium-chain deficiency (ACADMD) [MIM:201450]: An inborn error of mitochondrial fatty acid beta-oxidation which causes fasting hypoglycemia, hepatic dysfunction and encephalopathy, often resulting in death in infancy. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the acyl-CoA dehydrogenase family.

Database links:

Entrez Gene: 505968 Cow

Entrez Gene: 34 Human

Entrez Gene: 11364 Mouse

Entrez Gene: 24158 Rat

Omim: 607008 Human

SwissProt: Q3SZB4 Cow

SwissProt: P11310 Human

SwissProt: P45952 Mouse

SwissProt: P08503 Rat

Unigene: 445040 Human

Unigene: 10530 Mouse

Unigene: 6302 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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