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Rabbit Anti-IVD/Gold Conjugated antibody (bs-17189R-Gold)
訂購熱線:400-901-9800
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-17189R-Gold
英文名稱 Rabbit Anti-IVD/Gold Conjugated antibody
中文名稱 膠體金標記的異戊酰輔酶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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
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
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
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 0.4mg/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 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產品介紹 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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