Mouse Anti-ALG6 Recombinant Antibody (2G11) (CBMAB-A0302-LY)

The product is antibody recognizes ALG6. The antibody 2G11 immunoassay techniques such as: WB, ELISA.
See all ALG6 antibodies

Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

2G11

Antibody Isotype

IgG2a, κ

Application

WB, ELISA, IF

Basic Information

Immunogen

ALG6 (NP_037471, 25 a.a. ~ 114 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG2a, κ

Clonality

Monoclonal

Application Notes

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

Application	Note
IF(ICC)	10 µg/ml

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

asparagine-linked glycosylation 6, alpha-1,3-glucosyltransferase homolog (S. cerevisiae)

Introduction

This gene encodes a member of the ALG6/ALG8 glucosyltransferase family. The encoded protein catalyzes the addition of the first glucose residue to the growing lipid-linked oligosaccharide precursor of N-linked glycosylation. Mutations in this gene are associated with congenital disorders of glycosylation type Ic. [provided by RefSeq]

Entrez Gene ID

29929

UniProt ID

Q9Y672

Function

Adds the first glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation. Transfers glucose from dolichyl phosphate glucose (Dol-P-Glc) onto the lipid-linked oligosaccharide Man9GlcNAc2-PP-Dol.

Biological Process

Dolichol-linked oligosaccharide biosynthetic process Source: Reactome
Oligosaccharide-lipid intermediate biosynthetic process Source: GO_Central
Protein N-linked glycosylation Source: MGI

Cellular Location

Endoplasmic reticulum membrane

Involvement in disease

Congenital disorder of glycosylation 1C (CDG1C): A form of congenital disorder of glycosylation, a multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions.

References

Bloch, J. S., Pesciullesi, G., Boilevin, J., Nosol, K., Irobalieva, R. N., Darbre, T., ... & Locher, K. P. (2020). Structure and mechanism of the ER-based glucosyltransferase ALG6. Nature, 579(7799), 443-447.

Starosta, R. T., Tarnowski, J., e Vairo, F. P., Raymond, K., Preston, G., & Morava, E. (2020). Bosch-Boonstra-Schaaf optic atrophy syndrome (BBSOAS) initially diagnosed as ALG6-CDG: Functional evidence for benignity of the ALG6 c. 391T> C (p. Tyr131His) variant and further expanding the BBSOAS phenotype. European journal of medical genetics, 63(7), 103941.

Zhou, B., Zhao, Y. C., Liu, H., Luo, S., Amos, C. I., Lee, J. E., ... & Wei, Q. (2020). Novel Genetic Variants of ALG6 and GALNTL4 of the Glycosylation Pathway Predict Cutaneous Melanoma-Specific Survival. Cancers, 12(2), 288.

van Veen, S., Martin, S., Van den Haute, C., Benoy, V., Lyons, J., Vanhoutte, R., ... & Vangheluwe, P. (2020). Structure and mechanism of the ER-based glucosyltransferase ALG6.

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Protocols

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

Associated Products

Isotype control

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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