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Mouse Anti-B4GAT1 Recombinant Antibody (2H6) (CBMAB-A0690-LY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
2H6
Antibody Isotype
IgG2b, κ
Application
ELISA

Basic Information

Immunogen
B4GAT1 (NP_006867.1, 316 a.a. ~ 415 a.a) partial recombinant protein with GST tag.
Specificity
Human
Antibody Isotype
IgG2b, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
None
Concentration
Batch dependent
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1
Introduction
This gene encodes a member of the beta-1,3-N-acetylglucosaminyltransferase family. This enzyme is a type II transmembrane protein. It is essential for the synthesis of poly-N-acetyllactosamine, a determinant for the blood group i antigen. [provided by RefSeq]
Entrez Gene ID
11041
UniProt ID
O43505
Alternative Names
B3GN-T1; B3GNT6; BETA3GNTI; iGAT; iGNT
Function
Beta-1,4-glucuronyltransferase involved in O-mannosylation of alpha-dystroglycan (DAG1). Transfers a glucuronic acid (GlcA) residue onto a xylose (Xyl) acceptor to produce the glucuronyl-beta-1,4-xylose-beta disaccharide primer, which is further elongated by LARGE1, during synthesis of phosphorylated O-mannosyl glycan (PubMed:25279699, PubMed:25279697).
Phosphorylated O-mannosyl glycan is a carbohydrate is a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity (PubMed:25279699, PubMed:25279697).
Required for axon guidance; via its function in O-mannosylation of alpha-dystroglycan (DAG1) (By similarity).
Biological Process
Keratan sulfate biosynthetic process Source: Reactome
Protein O-linked glycosylation Source: Reactome
Protein O-linked mannosylation Source: UniProtKB
Cellular Location
Golgi apparatus membrane. Localizes near the trans-Golgi apparatus.
Involvement in disease
Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A13 (MDDGA13): An autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies, eye malformations, profound mental retardation, and death usually in the first years of life. Included diseases are the more severe Walker-Warburg syndrome and the slightly less severe muscle-eye-brain disease.
Topology
Cytoplasmic: 1-8 aa
Helical: 9-36 aa
Lumenal: 37-415 aa
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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