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>   首页   >   产品   >   一抗   >   代谢   >   PMM2 Antibody (monoclonal) (M01)   

PMM2 Antibody (monoclonal) (M01)

Mouse monoclonal antibody raised against a partial recombinant PMM2.

     
  • 1 - PMM2 Antibody (monoclonal) (M01) AT3355a
    Antibody Reactive Against Recombinant Protein.Western Blot detection against Immunogen (32.89 KDa) .
  • 1 - PMM2 Antibody (monoclonal) (M01) AT3355a
    Western Blot analysis of PMM2 expression in transfected 293T cell line by PMM2 monoclonal antibody (M01), clone 2E9.

    Lane 1: PMM2 transfected lysate(28.1 KDa).
    Lane 2: Non-transfected lysate.
  • 10 - PMM2 Antibody (monoclonal) (M01) AT3355a
    Detection limit for recombinant GST tagged PMM2 is 0.1 ng/ml as a capture antibody.
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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, E
Primary Accession O15305
Other Accession NM_000303
Reactivity Human
Host mouse
Clonality monoclonal
Isotype IgG2b Kappa
Clone Names 2E10
Calculated MW 28082 Da
Additional Information
Gene ID 5373
Other Names Phosphomannomutase 2, PMM 2, PMM2
Target/Specificity PMM2 (NP_000294, 47 a.a. ~ 111 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Dilution WB~~1:500~1000
E~~N/A
Format Clear, colorless solution in phosphate buffered saline, pH 7.2 .
StorageStore at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
PrecautionsPMM2 Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures.
Research Areas

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

BACKGROUND

The protein encoded by this gene catalyzes the isomerization of mannose 6-phosphate to mannose 1-phosphate, which is a precursor to GDP-mannose necessary for the synthesis of dolichol-P-oligosaccharides. Mutations in this gene have been shown to cause defects in glycoprotein biosynthesis, which manifests as carbohydrate-deficient glycoprotein syndrome type I.

REFERENCES

Functional analysis of three splicing mutations identified in the PMM2 gene: toward a new therapy for congenital disorder of glycosylation type Ia. Vega AI, et al. Hum Mutat, 2009 May. PMID 19235233.Primary skeletal dysplasia as a major manifesting feature in an infant with congenital disorder of glycosylation type Ia. Coman D, et al. Am J Med Genet A, 2008 Feb 1. PMID 18203160.Cerebellar ataxia and congenital disorder of glycosylation Ia (CDG-Ia) with normal routine CDG screening. Vermeer S, et al. J Neurol, 2007 Oct. PMID 17694350.Congenital disorder of glycosylation type 1a: three siblings with a mild neurological phenotype. Coman D, et al. J Clin Neurosci, 2007 Jul. PMID 17451957.Characterization of two unusual truncating PMM2 mutations in two CDG-Ia patients. Schollen E, et al. Mol Genet Metab, 2007 Apr. PMID 17307006.

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