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IVD Rabbit pAb (bs-17189R)  
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產品編號 bs-17189R
英文名稱 IVD Rabbit pAb
中文名稱 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.  
研究領域 腫瘤  細胞生物  神經生物學  信號轉導  新陳代謝  干擾素  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Mouse (predicted: Human,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 43 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human IVD: 201-300/423 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
P26440

Gene ID:
3712

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



產品圖片
25 ug total protein per lane of various lysates (see on figure) probed with IVD polyclonal antibody, unconjugated (bs-17189R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
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