Immunohistochemistry (IHC)
(Immunohistochemical staining of paraffin-embedded U87 xenograft using PMM2 antibody at a dilution of 1:100)
Rabbit PMM2 Polyclonal Antibody | anti-PMM2 antibody
PMM2 Antibody
WB: 1:500-1:3000
Immunohistochemistry (IHC)
(Immunohistochemical staining of paraffin-embedded U87 xenograft using PMM2 antibody at a dilution of 1:100)
Immunohistochemistry (IHC)
(Immunohistochemical staining of paraffin-embedded U87 xenograft using PMM2 antibody at a dilution of 1:100)
Western Blot (WB)
(Western blot analysis of 30 ug of whole cell lysate (A: Hela; B: Hep G2) using a 12% SDS PAGE gel and PMM2 antibody at a dilution of 1:1000)
Western Blot (WB)
(Western blot analysis of 30 ug of whole cell lysate (A: Hela; B: Hep G2) using a 12% SDS PAGE gel and PMM2 antibody at a dilution of 1:1000)
NCBI and Uniprot Product Information
NCBI Description
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. [provided by RefSeq, Jul 2008]
Uniprot Description
PMM2: Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions. Defects in PMM2 are the cause of congenital disorder of glycosylation type 1A (CDG1A); also known as carbohydrate-deficient glycoprotein syndrome type Ia (CDGS1A) or Jaeken syndrome. Congenital disorders of glycosylation are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1A is an autosomal recessive disorder characterized by a severe encephalopathy with axial hypotonia, abnormal eye movement, and pronounced psychomotor retardation, as well as peripheral neuropathy, cerebellar hypoplasia, and retinitis pigmentosa. Patients show a peculiar distribution of subcutaneous fat, nipple retraction, and hypogonadism. Belongs to the eukaryotic PMM family.
Protein type: Carbohydrate Metabolism - amino sugar and nucleotide sugar; Isomerase; EC 5.4.2.8; Carbohydrate Metabolism - fructose and mannose
Chromosomal Location of Human Ortholog: 16p13
Cellular Component: cell soma; cytosol
Molecular Function: phosphomannomutase activity
Biological Process: mannose biosynthetic process; cellular protein metabolic process; dolichol-linked oligosaccharide biosynthetic process; protein amino acid glycosylation; GDP-mannose biosynthetic process; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification
Disease: Congenital Disorder Of Glycosylation, Type Ia
Research Articles on PMM2
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Product Notes
The PMM2 pmm2 (Catalog #AAA5312447) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's PMM2 can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC) Paraffin, Western Blot (WB). IHC-P: 1:100-1:500 WB: 1:500-1:3000. Researchers should empirically determine the suitability of the PMM2 pmm2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PMM2, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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