GDP甘露糖焦磷酸化酶B抗体

Quantity size:

0.2ml

Concentration:

1mg/ml  GDP甘露糖焦磷酸化酶B抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

GMPPB is a 360 amino acid protein that belongs to the transferase hexapeptide repeat family and is involved in protein modification pathways. Functioning as a GDP-mannose pyrophosphorylase, GMPPB enzymatically catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose and a free phosphate, a reaction that is involved in the production of  GDP甘露糖焦磷酸化酶B抗体N-linked oligosaccharides. Defects in the gene encoding GMPPB that cause errors in the glycosylation pathway may lead to congenital disorders of glycosylation (CDG). CDGs are multisystemic diseases that often involve both the central and peripheral nervous systems and are often characterized by endocrine and coagulation disorders. GMPPB is expressed as two isoforms due to alternative splicing events.

 GDP甘露糖焦磷酸化酶B抗体Also known as:

GDP mannose pyrophosphorylase B; GDP-mannose pyrophosphorylase B; GMPPB; GMPPB_HUMAN; GTP-mannose-1-phosphate guanylyltransferase beta; KIAA1851; Mannose 1 phosphate guanylyltransferase; Mannose-1-phosphate guanyltransferase beta.

Specificity:

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Rabbit Polyclonal IgG,  GDP甘露糖焦磷酸化酶B抗体affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human GMPPB.

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Predicted Molecular Weight: 40kDa.

 GDP甘露糖焦磷酸化酶B抗体Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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