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HGD Rabbit pAb (bs-15472R)  
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產品編號 bs-15472R
英文名稱 HGD Rabbit pAb
中文名稱 尿黑酸氧化酶抗體
別    名 2-dioxygenase; AKU; FLJ94126; hgd; HGD_HUMAN; HGO; Homogentisate 1 2 dioxygenase; Homogentisate 1; Homogentisate oxidase; Homogentisate oxygenase; Homogentisic acid oxidase; Homogentisicase.  
研究領域 信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Mouse (predicted: Human,Rat,Rabbit)
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 50 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 HGD: 351-445/445 
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 HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyze the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterized by urine that turns dark on standing and alkalinization, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.

Tissue Specificity:
Highest expression in the prostate, small intestine, colon, kidney and liver.

DISEASE:
Alkaptonuria (AKU) [MIM:203500]: An autosomal recessive error of metabolism characterized by an increase in the level of homogentisic acid. The clinical manifestations are urine that turns dark on standing and alkalinization, black ochronotic pigmentation of cartilage and collagenous tissues, and spine arthritis. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the homogentisate dioxygenase family.

SWISS:
Q93099

Gene ID:
3081

Database links:

Entrez Gene: 3081 Human

Omim: 607474 Human

SwissProt: Q93099 Human

Unigene: 368254 Human



產品圖片
Sample: Kidney (Mouse) Lysate at 40 ug Primary: Anti- HGD (bs-15472R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 50 kD Observed band size: 50 kD
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