Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using POMGNT1 antibody at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit.Exposure time: 60s.)
Rabbit POMGNT1 Polyclonal Antibody | anti-POMGNT1 antibody
POMGNT1 Polyclonal Antibody
Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using POMGNT1 antibody at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit.Exposure time: 60s.)
Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using POMGNT1 antibody at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit.Exposure time: 60s.)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human prostate using POMGNT1 antibody at dilution of 1:100 (40x lens).)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human prostate using POMGNT1 antibody at dilution of 1:100 (40x lens).)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human liver injury using POMGNT1 antibody at dilution of 1:100 (40x lens).)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human liver injury using POMGNT1 antibody at dilution of 1:100 (40x lens).)
NCBI and Uniprot Product Information
Molecular Weight: 660
NCBI Description
This gene encodes a type II transmembrane protein that resides in the Golgi apparatus. It participates in O-mannosyl glycosylation and is specific for alpha linked terminal mannose. Mutations in this gene may be associated with muscle-eye-brain disease and several congenital muscular dystrophies. Alternatively spliced transcript variants that encode different protein isoforms have been described. [provided by RefSeq, Feb 2014]
Uniprot Description
POMGNT1: Participates in O-mannosyl glycosylation. May be responsible for the synthesis of the GlcNAc(beta1-2)Man(alpha1-)O- Ser/Thr moiety on alpha-dystroglycan and other O-mannosylated proteins. Is specific for alpha linked terminal mannose and does not have MGAT3, MGAT4, MGAT5, MGAT7 or MGAT8 activity. Defects in POMGNT1 are the cause of muscular dystrophy- dystroglycanopathy congenital with brain and eye anomalies type A3 (MDDGA3). MDDGA3 is an autosomal recessive disorder characterized by congenital muscular dystrophy, ocular abnormalities, cobblestone lissencephaly and cerebellar hypoplasia. MDDGA3 patients present severe congenital myopia, congenital glaucoma, pallor of the optic disks, retinal hypoplasia, mental retardation, hydrocephalus, abnormal electroencephalograms, generalized muscle weakness and myoclonic jerks. Defects in POMGNT1 are the cause of muscular dystrophy- dystroglycanopathy congenital with mental retardation type B3 (MDDGB3); also called muscular dystrophy congenital POMGNT1-related. MDDGB3 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with mental retardation and mild structural brain abnormalities. Clinical features include mental retardation, white matter changes, cerebellar cysts, pontine hypoplasia, myopia, optic atrophy, decreased alpha-dystroglycan on muscle biopsy and increased serum creatine kinase. Defects in POMGNT1 are the cause of muscular dystrophy- dystroglycanopathy limb-girdle type C3 (MDDGC3); also called muscular dystrophy-dystroglycanopathy limb-girdle POMGNT1- related. MDDGC3 is a rare form of limb-girdle muscular dystrophy with normal cognition. Muscle biopsy shows dystrophic changes with variable staining for glycosylated alpha-dystroglycan. Belongs to the glycosyltransferase 13 family.
Protein type: EC 2.4.1.-; Glycan Metabolism - O-mannosyl glycan biosynthesis; Membrane protein, integral; Transferase
Chromosomal Location of Human Ortholog: 1p34.1
Cellular Component: Golgi membrane; integral to membrane
Molecular Function: beta-1,3-galactosyl-O-glycosyl-glycoprotein beta-1,3-N-acetylglucosaminyltransferase activity; protein binding
Biological Process: protein amino acid O-linked glycosylation
Disease: Muscular Dystrophy-dystroglycanopathy (congenital With Brain And Eye Anomalies), Type A, 3; Muscular Dystrophy-dystroglycanopathy (congenital With Mental Retardation), Type B, 3; Muscular Dystrophy-dystroglycanopathy (limb-girdle), Type C, 3
Research Articles on POMGNT1
Similar Products
Product Notes
The POMGNT1 pomgnt1 (Catalog #AAA9129226) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The POMGNT1 Polyclonal Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's POMGNT1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC). WB: 1:500 - 1:2000, IHC: 1:50 - 1:100. Researchers should empirically determine the suitability of the POMGNT1 pomgnt1 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 "POMGNT1, 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
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
