Product Name	GMPPB
Chinese Name	GDP甘露糖焦磷酸化酶B抗体
Alias	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.
Research Area	Cell biology  Signal transduction  Epigenetics
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	40kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human GMPPB: 111-210/360
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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 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. Subcellular Location: Belongs to the transferase hexapeptide repeat family. Similarity: Belongs to the transferase hexapeptide repeat family. SWISS: Q9Y5P6 Gene ID: 29925 Database links: Entrez Gene: 29925 Human Entrez Gene: 331026 Mouse Entrez Gene: 363145 Rat SwissProt: Q9Y5P6 Human SwissProt: Q8BTZ7 Mouse Unigene: 567488 Human Unigene: 22554 Mouse Unigene: 102187 Rat

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