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>   首页   >   产品   >   一抗   >   其他   >   GMPPB Rabbit pAb   

GMPPB Rabbit pAb

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IHC-P, IHC-F, IF, E
Primary Accession Q9Y5P6
Other Accession Q9Y5P6
Host Rabbit
Clonality Polyclonal
Calculated MW 39834 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human GMPPB
Epitope Specificity 111-210/360
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the transferase hexapeptide repeat family.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions 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.
Additional Information
Gene ID 29925
Other Names LGMDR19; MDDGA14; MDDGB14; MDDGC14; E430010H19; RGD1560458; GMPPB_HUMAN; GMPPB; GDP-mannose pyrophosphorylase B; GTP-mannose-1-phosphate guanylyltransferase beta; 2.7.7.13; GMPPB_MOUSE; mannose-1-phosphate guanyltransferase beta
Dilution WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name GMPPB (HGNC:22932)
Function Catalytic subunit of the GMPPA-GMPPB mannose-1-phosphate guanylyltransferase complex (PubMed:33986552). Catalyzes the formation of GDP-mannose, an essential precursor of glycan moieties of glycoproteins and glycolipids (PubMed:33986552). Can catalyze the reverse reaction in vitro (PubMed:33986552). Together with GMPPA regulates GDP-alpha-D-mannose levels (PubMed:33986552).
Cellular Location Cytoplasm.
Tissue Location [Isoform 1]: Ubiquitously expressed, including in brain and skeletal muscle.
Research Areas
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