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Mouse Anti-LARGE1 Recombinant Antibody (LARGE-02) (CBMAB-L0694-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to Large Xylosyl- And Glucuronyltransferase 1 (LARGE1). The antibody can be used for immunoassay techniques, such as FC.
See all LARGE1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
LARGE-02
Antibody Isotype
IgG2b
Application
FC

Basic Information

Specificity
Human
Antibody Isotype
IgG2b
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!]

Buffer
PBS, pH 7.4
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
LARGE xylosyl- and glucuronyltransferase 1
Introduction
LARGE1 is a member of the N-acetylglucosaminyltransferase gene family. LARGE1 may carry out the synthesis of glycoprotein and glycosphingolipid sugar chains. LARGE1 may also be involved in the addition of a repeated disaccharide unit. LARGE1 is the glycotransferase that adds the final xylose and glucuronic acid to alpha-dystroglycan and thereby allows alpha-dystroglycan to bind ligands including laminin 211 and neurexin. Mutations in this gene cause several forms of congenital muscular dystrophy characterized by cognitive disability and abnormal glycosylation of alpha-dystroglycan. Alternative splicing of this gene results in multiple transcript variants that encode the same protein.
Entrez Gene ID
9215
UniProt ID
O95461
Alternative Names
LARGE; MDC1D; MDDGA6; MDDGB6
Function
Bifunctional glycosyltransferase with both alpha-1,3-xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the maturation of alpha-dystroglycan (DAG1) by glycosylation leading to DAG1 binding to laminin G-like domain-containing extracellular proteins with high affinity (PubMed:22223806, PubMed:15752776, PubMed:15661757, PubMed:25279699, PubMed:25279697, PubMed:23125099, PubMed:21987822).
Elongates the glucuronyl-beta-1,4-xylose-beta disaccharide primer structure initiated by AC B4GAT1 by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide (PubMed:22223806, PubMed:25279699, PubMed:25279697, PubMed:25138275, PubMed:32975514, PubMed:23125099).
Requires the phosphorylation of core M3 (O-mannosyl trisaccharide) by POMK to elongate the glucuronyl-beta-1,4-xylose-beta disaccharide primer (PubMed:21987822).
Plays a key role in skeletal muscle function and regeneration (By similarity).
Biological Process
Glycoprotein biosynthetic processManual Assertion Based On ExperimentTAS:UniProtKB
Glycosphingolipid biosynthetic processManual Assertion Based On ExperimentTAS:UniProtKB
Muscle cell cellular homeostasisISS:UniProtKB
N-acetylglucosamine metabolic processManual Assertion Based On ExperimentTAS:ProtInc
Protein glycosylationManual Assertion Based On ExperimentIMP:UniProtKB
Protein O-linked glycosylationTAS:Reactome
Protein O-linked mannosylationManual Assertion Based On ExperimentIDA:UniProtKB
Skeletal muscle organ developmentISS:UniProtKB
Skeletal muscle tissue regenerationISS:UniProtKB
Cellular Location
Golgi apparatus membrane
Involvement in disease
Muscular dystrophy-dystroglycanopathy congenital with impaired intellectual development B6 (MDDGB6):
A congenital muscular dystrophy associated with profound mental retardation, white matter changes and structural brain abnormalities. Skeletal muscle biopsies show reduced immunolabeling of alpha-dystroglycan.
Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A6 (MDDGA6):
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: 1-10
Helical: 11-31
Lumenal: 32-756

Okuma, H., Hord, J. M., Chandel, I., Venzke, D., Anderson, M. E., Walimbe, A. S., ... & Campbell, K. P. (2023). N-terminal domain on dystroglycan enables LARGE1 to extend matriglycan on α-dystroglycan and prevents muscular dystrophy. Elife, 12, e82811.

Katz, M., & Diskin, R. (2022). Structural basis for matriglycan synthesis by the LARGE1 dual glycosyltransferase. Plos one, 17(12), e0278713.

Joseph, S., Schnicker, N. J., Xu, Z., Yang, T., Hopkins, J., Watkins, M., ... & Campbell, K. P. (2022). Structure and mechanism of LARGE1 matriglycan polymerase. bioRxiv, 2022-05.

Yonekawa, T., Rauckhorst, A. J., El-Hattab, S., Cuellar, M. A., Venzke, D., Anderson, M. E., ... & Campbell, K. P. (2022). Large1 gene transfer in older myd mice with severe muscular dystrophy restores muscle function and greatly improves survival. Science advances, 8(21), eabn0379.

Inamori, K. I. (2022). Enzyme assay of protein-O-mannosyl glycan glycosyltransferases (LARGE1/2). Glycoscience Protocols (GlycoPODv2)[Internet].

Liu, Y., Huang, S., Kuang, M., Wang, H., & Xie, Q. (2021). High LARGE1 expression may predict benefit from adjuvant chemotherapy in resected non-small-cell lung cancer. Pharmacogenomics and Personalized Medicine, 87-99.

Lyu, J., Wang, D., Duan, P., Liu, Y., Huang, K., Zeng, D., ... & Li, Y. (2020). Control of grain size and weight by the GSK2-LARGE1/OML4 pathway in rice. The Plant Cell, 32(6), 1905-1918.

Walimbe, A. S., Okuma, H., Joseph, S., Yang, T., Yonekawa, T., Hord, J. M., ... & Campbell, K. P. (2020). POMK regulates dystroglycan function via LARGE1-mediated elongation of matriglycan. Elife, 9, e61388.

Righino, B., Bozzi, M., Pirolli, D., Sciandra, F., Bigotti, M. G., Brancaccio, A., & De Rosa, M. C. (2020). Identification and modeling of a GT-A fold in the α-dystroglycan glycosylating enzyme LARGE1. Journal of Chemical Information and Modeling, 60(6), 3145-3156.

Beltrán, D., Anderson, M. E., Bharathy, N., Settelmeyer, T. P., Svalina, M. N., Bajwa, Z., ... & Campbell, K. P. (2019). Exogenous expression of the glycosyltransferase LARGE1 restores α-dystroglycan matriglycan and laminin binding in rhabdomyosarcoma. Skeletal Muscle, 9, 1-10.

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

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