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>   首页   >   产品   >   一抗   >   其他   >   POMGNT1 Antibody (monoclonal) (M07)   

POMGNT1 Antibody (monoclonal) (M07)

Mouse monoclonal antibody raised against a partial recombinant POMGNT1.

     
  • 1 - POMGNT1 Antibody (monoclonal) (M07) AT3382a
    Antibody Reactive Against Recombinant Protein.Western Blot detection against Immunogen (36.52 KDa) .
  • 2 - POMGNT1 Antibody (monoclonal) (M07) AT3382a
    Immunoperoxidase of monoclonal antibody to POMGNT1 on formalin-fixed paraffin-embedded human liver. [antibody concentration 3 ug/ml]
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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
Primary Accession Q8WZA1
Other Accession NM_017739
Reactivity Human
Host mouse
Clonality monoclonal
Isotype IgG2a Kappa
Clone Names 6C12
Calculated MW 75252 Da
Additional Information
Gene ID 55624
Other Names Protein O-linked-mannose beta-1, 2-N-acetylglucosaminyltransferase 1, POMGnT1, 241-, UDP-GlcNAc:alpha-D-mannoside beta-1, 2-N-acetylglucosaminyltransferase I2, GnT I2, POMGNT1, MGAT12
Target/Specificity POMGNT1 (NP_060209, 221 a.a. ~ 318 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Dilution WB~~1:500~1000
IHC~~1:100~500
Format Clear, colorless solution in phosphate buffered saline, pH 7.2 .
StorageStore at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
PrecautionsPOMGNT1 Antibody (monoclonal) (M07) is for research use only and not for use in diagnostic or therapeutic procedures.

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

Research Areas

BACKGROUND

The protein encoded by this gene is a type II transmembrane protein that resides in the golgi. It participates in O-mannosyl glycosylation, and is specific for alpha linked terminal mannose. Mutations in this gene are associated with muscle-eye-brain (MEB) disease. Alternatively spliced transcript variants have been found for this gene.

REFERENCES

Congenital muscular dystrophies with defective glycosylation of dystroglycan: a population study. Mercuri E, et al. Neurology, 2009 May 26. PMID 19299310.Novel POMGnT1 mutations define broader phenotypic spectrum of muscle-eye-brain disease. Hehr U, et al. Neurogenetics, 2007 Nov. PMID 17906881.Severe muscle-eye-brain disease is associated with a homozygous mutation in the POMGnT1 gene. Teber S, et al. Eur J Paediatr Neurol, 2008 Mar. PMID 17881266.Refining genotype phenotype correlations in muscular dystrophies with defective glycosylation of dystroglycan. Godfrey C, et al. Brain, 2007 Oct. PMID 17878207.Protein O-mannosyltransferase activities in lymphoblasts from patients with alpha-dystroglycanopathies. Manya H, et al. Neuromuscul Disord, 2008 Jan. PMID 17869517.

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