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Mouse Anti-GYS2 Recombinant Antibody (8H3) (CBMAB-G5896-LY)

This product is antibody recognizes GYS2. The antibody 8H3 immunoassay techniques such as: ELISA, WB.
See all GYS2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
8H3
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

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.

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

Format
Liquid
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
glycogen synthase 2
Introduction
The protein encoded by this gene, liver glycogen synthase, catalyzes the rate-limiting step in the synthesis of glycogen - the transfer of a glucose molecule from UDP-glucose to a terminal branch of the glycogen molecule. Mutations in this gene cause glycogen storage disease type 0 (GSD-0) - a rare type of early childhood fasting hypoglycemia with decreased liver glycogen content. [provided by RefSeq, Dec 2009]
Entrez Gene ID
2998
UniProt ID
P54840
Alternative Names
Glycogen Synthase 2; EC 2.4.1.11; Glycogen Synthase 2 (Liver);
Function
Transfers the glycosyl residue from UDP-Glc to the non-reducing end of alpha-1,4-glucan.
Biological Process
Generation of precursor metabolites and energy Source: ProtInc
Glycogen biosynthetic process Source: UniProtKB
Response to glucose Source: UniProtKB
Cellular Location
Cortical actin cytoskeleton; Cytoskeleton; Cytosol; Cell cortex; Cytoplasm; Ectoplasm
Involvement in disease
Glycogen storage disease 0 (GSD0):
A metabolic disorder characterized by fasting hypoglycemia presenting in infancy or early childhood, high blood ketones and low alanine and lactate concentrations. Although feeding relieves symptoms, it often results in postprandial hyperglycemia and hyperlactatemia.
PTM
Primed phosphorylation at Ser-657 (site 5) by CSNK2A1 and CSNK2A2 is required for inhibitory phosphorylation at Ser-641 (site 3a), Ser-645 (site 3b), Ser-649 (site 3c) and Ser-653 (site 4) by GSK3A an GSK3B. Dephosphorylation at Ser-641 and Ser-645 by PP1 activates the enzyme (By similarity). Phosphorylation at Ser-8 is not required for interaction with GYG1 (By similarity). Interaction with GYG1 does not regulate the phosphorylation at Ser-8 and Ser-641 (By similarity).

Zhang, X., Yin, H., Zhang, X., Jiang, X., Liu, Y., Zhang, H., ... & Zhang, R. (2022). N6-methyladenosine modification governs liver glycogenesis by stabilizing the glycogen synthase 2 mRNA. Nature Communications, 13(1), 7038.

Iijima, H., Ago, Y., Fujiki, R., Takayanagi, T., & Kubota, M. (2021). Novel GYS2 mutations in a Japanese patient with glycogen storage disease type 0a. Molecular Genetics and Metabolism Reports, 26, 100702.

Tao, J., Zhang, Y., Wang, D., Jiang, X., Liu, Y., Chen, N., & Li, S. (2021). Liver type glycogen synthase (GYS2) and phosphorylase (PYGL) in largemouth bass (Micropterus salmoides): Cloning, characterization, and their transcriptional response to dietary carbohydrate content and resveratrol inclusion. Aquaculture Reports, 21, 100968.

Sigen, A., Wu, H., Wang, X., Wang, X., Yang, J., Xia, L., & Xia, Y. (2021). Value of glycogen synthase 2 in intrahepatic cholangiocarcinoma prognosis assessment and its influence on the activity of cancer cells. Bioengineered, 12(2), 12167.

Kamenets, E. A., Gusarova, E. A., Milovanova, N. V., Itkis, Y. S., Strokova, T. V., Melikyan, M. A., ... & Zakharova, E. Y. (2020). Hepatic glycogen synthase (GYS2) deficiency: seven novel patients and seven novel variants. JIMD reports, 53(1), 39-44.

Arko, J. J., Debeljak, M., Tansek, M. Z., Battelino, T., & Groselj, U. (2020). A patient with glycogen storage disease type 0 and a novel sequence variant in GYS2: a case report and literature review. Journal of International Medical Research, 48(8), 0300060520936857.

Hacıhamdioğlu, B., Özgürhan, G., Çaran, B., Meydan-Aksanlı, E., & Keskin, E. (2018). Glycogen storage disease type 0 due to a novel frameshift mutation in glycogen synthase 2 (GYS2) gene in a child presenting with fasting hypoglycemia and postprandial hyperglycemia. The Turkish Journal of Pediatrics, 60(5), 581-583.

Flannery, C. A., Choe, G. H., Cooke, K. M., Fleming, A. G., Radford, C. C., Kodaman, P. H., ... & Taylor, H. S. (2018). Insulin regulates glycogen synthesis in human endometrial glands through increased GYS2. The Journal of Clinical Endocrinology & Metabolism, 103(8), 2843-2850.

Pursell, N., Gierut, J., Zhou, W., Dills, M., Diwanji, R., Gjorgjieva, M., ... & Lai, C. (2018). Inhibition of glycogen synthase II with RNAi prevents liver injury in mouse models of glycogen storage diseases. Molecular Therapy, 26(7), 1771-1782.

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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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