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HIBADH Rabbit pAb (bs-15483R)  
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產品編號 bs-15483R
英文名稱 HIBADH Rabbit pAb
中文名稱 3-羥基異丁酸脫氫酶抗體
別    名 3 hydroxy 2 methylpropanoate:NAD(+) oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN.  
研究領域 細胞生物  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,Mouse (predicted: Rat,Sheep,Cow,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 32 kDa
檢測分子量
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HIBADH: 251-336/336 
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 HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.

Subcellular Location:
Mitochondrion.

Tissue Specificity:
Detected in skin fibroblasts.

Similarity:
Belongs to the 3-hydroxyisobutyrate dehydrogenase family.

SWISS:
P31937

Gene ID:
11112

Database links:

Entrez Gene: 11112 Human

Entrez Gene: 58875 Mouse

Entrez Gene: 63938 Rat

Omim: 608475 Human

SwissProt: P31937 Human

SwissProt: Q99L13 Mouse

SwissProt: P29266 Rat

Unigene: 406758 Human

Unigene: 286458 Mouse

Unigene: 73 Rat



產品圖片
Sample: 193T(Human) Cell Lysate at 30 ug MCF-7(Human) Cell Lysate at 30 ug HepG2(Human) Cell Lysate at 30 ug Kidney (Mouse) Lysate at 40 ug Primary: Anti- HIBADH (bs-15483R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 32 kD Observed band size: 30 kD
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