Mouse Anti-B3GAT3 Recombinant Antibody (CBYY-0080) (CBMAB-0080-YY)

Name: Mouse Anti-B3GAT3 Recombinant Antibody (CBYY-0080)
SKU: CBMAB-0080-YY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

CBYY-0080

Application

ELISA, WB, IHC-P, IF, IP

Immunogen

Amino acids 323-334 at the C-terminus of GlcAT-I of human origin.

Specificity

Human, Mouse, Rat

Antibody Isotype

IgM, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:100-1:1,000
IP	1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)	1:50-1:500
IHC-P	1:50-1:500
ELISA	1:100-1:1,000

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 freeze/thaw cycles.

More Infomation

Target

Full Name

beta-1,3-glucuronyltransferase 3 (glucuronosyltransferase I)

Introduction

The protein encoded by this gene is a member of the glucuronyltransferase gene family, enzymes that exhibit strict acceptor specificity, recognizing nonreducing terminal sugars and their anomeric linkages. This gene product catalyzes the formation of the glycosaminoglycan-protein linkage by way of a glucuronyl transfer reaction in the final step of the biosynthesis of the linkage region of proteoglycans. A pseudogene of this gene has been identified on chromosome 3.

Entrez Gene ID

26229

UniProt ID

O94766

Alternative Names

Beta-1,3-Glucuronyltransferase 3; Galactosylgalactosylxylosylprotein 3-Beta-Glucuronosyltransferase 3; Beta-1,3-Glucuronyltransferase 3 (Glucuronosyltransferase I); UDP-GlcUA:Gal Beta-1,3-Gal-R Glucuronyltransferase; Glucuronosyltransferase I; EC 2.4.1.135;

Function

Glycosaminoglycans biosynthesis (PubMed:25893793).
Involved in forming the linkage tetrasaccharide present in heparan sulfate and chondroitin sulfate. Transfers a glucuronic acid moiety from the uridine diphosphate-glucuronic acid (UDP-GlcUA) to the common linkage region trisaccharide Gal-beta-1,3-Gal-beta-1,4-Xyl covalently bound to a Ser residue at the glycosaminylglycan attachment site of proteoglycans. Can also play a role in the biosynthesis of l2/HNK-1 carbohydrate epitope on glycoproteins. Shows strict specificity for Gal-beta-1,3-Gal-beta-1,4-Xyl, exhibiting negligible incorporation into other galactoside substrates including Galbeta1-3Gal beta1-O-benzyl, Galbeta1-4GlcNAc and Galbeta1-4Glc. Stimulates 2-phosphoxylose phosphatase activity of PXYLP1 in presence of uridine diphosphate-glucuronic acid (UDP-GlcUA) during completion of linkage region formation (PubMed:24425863).

Biological Process

Carbohydrate metabolic process Source: GO_Central
Chondroitin sulfate proteoglycan biosynthetic process Source: MGI
Dermatan sulfate proteoglycan biosynthetic process Source: MGI
Glycosaminoglycan biosynthetic process Source: UniProtKB
Glycosaminoglycan metabolic process Source: Reactome
Heparan sulfate proteoglycan biosynthetic process Source: MGI
Positive regulation of catalytic activity Source: UniProtKB
Positive regulation of intracellular protein transport Source: UniProtKB
Protein glycosylation Source: UniProtKB-UniPathway

Cellular Location

Golgi apparatus membrane; Cis-Golgi network

Involvement in disease

Multiple joint dislocations, short stature, and craniofacial dysmorphism with or without congenital heart defects (JDSCD): An autosomal recessive disease characterized by dysmorphic facies, bilateral dislocations of the elbows, hips, and knees, clubfeet, and short stature, as well as cardiovascular defects.

Topology

Cytoplasmic: 1-7 aa
Helical: 8-28 aa
Lumenal: 29-335 aa

PTM

N-glycosylated.

Colman, M., Van Damme, T., Steichen-Gersdorf, E., Laccone, F., Nampoothiri, S., Syx, D., ... & Malfait, F. (2019). The clinical and mutational spectrum of B3GAT3 linkeropathy: two case reports and literature review. Orphanet journal of rare diseases, 14(1), 1-10.

Yauy, K., Mau-Them, F. T., Willems, M., Coubes, C., Blanchet, P., Herlin, C., ... & Genevieve, D. (2018). B3GAT3-related disorder with craniosynostosis and bone fragility due to a unique mutation. Genetics in Medicine, 20(2), 269-274.

Bloor, S., Giri, D., Didi, M., & Senniappan, S. (2017). Novel splicing mutation in B3GAT3 associated with short stature, GH deficiency, Hypoglycaemia, developmental delay, and multiple congenital anomalies. Case reports in genetics, 2017.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

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

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