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Rabbit Anti-IVD/BF488 Conjugated antibody (bs-17189R-BF488)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-17189R-BF488
英文名稱1 Rabbit Anti-IVD/BF488 Conjugated antibody
中文名稱 BF488標(biāo)記的異戊酰輔酶A脫氫酶抗體
別    名 ACAD2; FLJ12715; FLJ34849; Isovaleryl CoA dehydrogenase; Isovaleryl CoA dehydrogenase, mitochondrial; Isovaleryl Coenzyme A dehydrogenase; Isovaleryl-CoA dehydrogenase; IVD; IVD_HUMAN; mitochondrial.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  新陳代謝  干擾素  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 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 43kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human IVD
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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(chǎn)品介紹 background:
Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009]

Function:
Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 1/3.

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:243500]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.

Similarity:
Belongs to the acyl-CoA dehydrogenase family.

Database links:

Entrez Gene: 3712 Human

Entrez Gene: 510440 Cow

Entrez Gene: 56357 Mouse

Entrez Gene: 100156047 Pig

Entrez Gene: 24513 Rat

Omim: 607036 Human

SwissProt: Q3SZI8 Cow

SwissProt: P26440 Human

SwissProt: Q9JHI5 Mouse

SwissProt: P12007 Rat

Unigene: 513646 Human

Unigene: 6635 Mouse

Unigene: 147 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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