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Rabbit Anti-GALT/AP Conjugated antibody (bs-3984R-AP)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-3984R-AP
英文名称 Rabbit Anti-GALT/AP Conjugated antibody
中文名称 碱性磷酸酶(AP)标记的磷酸半乳糖尿苷酸转移酶1抗体
别    名 Gal 1 P uridylyltransferase; Galactose 1 phosphate uridyl transferase; Galactose 1 phosphate uridylyltransferase; UDP glucose hexose 1 phosphate uridylyltransferase; GALT_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  免疫学  激酶和磷酸酶  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Cow, )
产品应用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 43kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GALT
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 background:
GALT (Galactose 1 phosphate uridyl transferase) catalyzes the second step of the Leloir pathway of galactose metabolism, namely the conversion of UDP glucose + galactose 1 phosphate to glucose 1 phosphate + UDP galactose. The absence of this enzyme results in classic galactosemia in humans and can be fatal in the newborn period if lactose is not removed from the diet. The pathophysiology of galactosemia has not been clearly defined.

Subunit:
Homodimer (Probable).

DISEASE:
Defects in GALT are the cause of galactosemia (GALCT) [MIM:230400]. Galactosemia is an inherited disorder of galactose metabolism that causes jaundice, cataracts, and mental retardation.

Similarity:
Belongs to the galactose-1-phosphate uridylyltransferase type 1 family.

Database links:
UniProtKB/Swiss-Prot: P07902.3

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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