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Mouse Anti-B3GLCT Recombinant Antibody (EG329) (CBMAB-EN366-LY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
EG329
Antibody Isotype
IgG2a, κ
Application
ELISA, WB, IF, IP

Basic Information

Immunogen
Amino acids 394-498 mapping at the C-terminus of β-1,3-Gal-TL of human origin.
Specificity
Human
Antibody Isotype
IgG2a, κ
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:100-1:1,000
IP1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)1:50-1:500
ELISA1:100-1:1,000

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

Format
Liquid
Buffer
PBS, 0.1% gelatin
Preservative
< 0.1% sodium azide
Concentration
0.2 mg/ml
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
Beta 3-Glucosyltransferase
Introduction
The protein encoded by this gene is a beta-1,3-glucosyltransferase that transfers glucose to O-linked fucosylglycans on thrombospondin type-1 repeats (TSRs) of several proteins. The encoded protein is a type II membrane protein. Defects in this gene are a cause of Peters-plus syndrome (PPS).[provided by RefSeq, Mar 2009]
Entrez Gene ID
145173
UniProt ID
Q6Y288
Alternative Names
Beta 3-Glucosyltransferase; Beta 1,3-Galactosyltransferase-Like; Beta-1,3-Glucosyltransferase; Beta3Glc-T; B3GALTL; B3GTL; UDP-GAL:Beta-GlcNAc Beta-1,3-Galactosyltransferase-Like;
Function
O-glucosyltransferase that transfers glucose toward fucose with a beta-1,3 linkage. Specifically glucosylates O-linked fucosylglycan on TSP type-1 domains of proteins, thereby contributing to elongation of O-fucosylglycan.
Biological Process
Fucose metabolic process Source: UniProtKB-KW
Protein O-linked fucosylation Source: Reactome
Cellular Location
Endoplasmic reticulum membrane
Involvement in disease
Peters-plus syndrome (PTRPLS): An autosomal recessive disorder characterized by anterior eye-chamber abnormalities, disproportionate short stature, developmental delay, characteristic craniofacial features, cleft lip and/or palate.
Topology
Cytoplasmic: 1-6 aa
Helical: 7-27 aa
Lumenal: 28-498 aa
More Infomation

Zhang, A., Venkat, A., Taujale, R., Mull, J. L., Ito, A., Kannan, N., & Haltiwanger, R. S. (2021). Peters plus syndrome mutations affect the function and stability of human β1, 3-glucosyltransferase. Journal of Biological Chemistry, 100843.

Thu, C. T., Chung, J. Y., Dhawan, D., Vaiana, C. A., & Mahal, L. K. (2021). High-Throughput miRFluR Platform Identifies miRNA Regulating B3GLCT That Predict Peters’ Plus Syndrome Phenotype, Supporting the miRNA Proxy Hypothesis. ACS Chemical Biology.

Thu, C. T., Chung, J. Y., Dhawan, D., Vaiana, C. A., & Mahal, L. K. (2021). High-throughput analysis of B3GLCT regulation predicts phenotype of Peters' Plus Syndrome in line with the miRNA Proxy Hypothesis. bioRxiv.

Neupane, S., Goto, J., Berardinelli, S. J., Ito, A., Haltiwanger, R. S., & Holdener, B. C. (2021). Hydrocephalus in mouse B3glct mutants is likely caused by defects in multiple B3GLCT substrates in ependymal cells and subcommissural organ. Glycobiology.

Totoń-Żurańska, J., Kapusta, P., Rybak-Krzyszkowska, M., Lorenc, K., Machlowska, J., Skalniak, A., ... & Wołkow, P. P. (2019). Contribution of a Novel B3GLCT Variant to Peters Plus Syndrome Discovered by a Combination of Next-Generation Sequencing and Automated Text Mining. International journal of molecular sciences, 20(23), 6006.

Holdener, B. C., Percival, C. J., Grady, R. C., Cameron, D. C., Berardinelli, S. J., Zhang, A., ... & Haltiwanger, R. S. (2019). ADAMTS9 and ADAMTS20 are differentially affected by loss of B3GLCT in mouse model of Peters plus syndrome. Human molecular genetics, 28(24), 4053-4066.

Weh, E., Takeuchi, H., Muheisen, S., Haltiwanger, R. S., & Semina, E. V. (2017). Functional characterization of zebrafish orthologs of the human Beta 3-Glucosyltransferase B3GLCT gene mutated in Peters Plus Syndrome. PloS one, 12(9), e0184903.

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For research use only. Not intended for any clinical use.

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