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Mouse Anti-EXTL3 (AA 52-919) Recombinant Antibody (CBFYE-1422) (CBMAB-E2047-FY)

This product is mouse antibody that recognizes EXTL3. The antibody CBFYE-1422 can be used for immunoassay techniques such as: WB, IP.
See all EXTL3 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYE-1422
Antibody Isotype
IgG2a
Application
WB, IP

Basic Information

Immunogen
Mouse myeloma cell line NS0-derived recombinant human Exostosin-like 3/EXTL3, Thr52-Ile919
Specificity
Human
Antibody Isotype
IgG2a
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
Lyophilized
Buffer
PBS, trehalose
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 52-919

Target

Full Name
Exostosin Like Glycosyltransferase 3
Introduction
This gene encodes a single-pass membrane protein which functions as a glycosyltransferase. The encoded protein catalyzes the transfer of N-acetylglucosamine to glycosaminoglycan chains. This reaction is important in heparin and heparan sulfate synthesis. Alternative splicing results in the multiple transcript variants.
Entrez Gene ID
2137
UniProt ID
O43909
Alternative Names
Exostosin Like Glycosyltransferase 3; Glucuronyl-Galactosyl-Proteoglycan 4-Alpha-N-Acetylglucosaminyltransferase; Hereditary Multiple Exostoses Gene Isolog; Putative Tumor Suppressor Protein EXTL3; Exostoses (Multiple)-Like 3; EXTL1L; EXTR1; Multiple Exostosis-Like Protein 3; Exostosin Tumor-Like 3; EXT-Related Protein 1; Exostosin-Like 3
Research Area
Glycosyltransferase which regulates the biosynthesis of heparan sulfate (HS). Important for both skeletal development and hematopoiesis, through the formation of HS proteoglycans (HSPGs) (PubMed:28132690, PubMed:28148688).

Required for the function of REG3A in regulating keratinocyte proliferation and differentiation (PubMed:22727489).
Biological Process
Heparan sulfate proteoglycan biosynthetic process Source: UniProtKB
Positive regulation of cell growth Source: Ensembl
Protein glycosylation Source: InterPro
Cellular Location
Golgi apparatus; Endoplasmic reticulum membrane
Involvement in disease
Immunoskeletal dysplasia with neurodevelopmental abnormalities (ISDNA):
An autosomal recessive disorder characterized by variable skeletal abnormalities and neurodevelopmental defects. Neurologic manifestations include intellectual disability and motor delay. Some patients manifest hypotonia and seizures. Skeletal features include disproportionate short stature, cervical malformations, epiphyseal and metaphyseal dysplasia, and rarely premature craniosynostosis with progressive microcephaly. Severe combined immunodeficiency with a complete absence of T cells is observed in some patients.
Topology
Cytoplasmic: 1-30
Helical: 31-51
Lumenal: 52-919

Chang, P., Chen, S., Chang, X., Zhu, J., Tang, Q., & Ma, L. (2022). EXTL3 could serve as a potential biomarker of prognosis and immunotherapy for prostate cancer and its potential mechanisms. European Journal of Medical Research, 27(1), 1-14.

Wilson, L. F. L., Dendooven, T., Hardwick, S. W., Echevarría-Poza, A., Tryfona, T., Krogh, K. B. R. M., ... & Dupree, P. (2022). The structure of EXTL3 helps to explain the different roles of bi-domain exostosins in heparan sulfate synthesis. Nature Communications, 13(1), 1-15.

Marques, C., Poças, J., Gomes, C., Faria-Ramos, I., Reis, C. A., Vivès, R. R., & Magalhães, A. (2022). Glycosyltransferases EXTL2 and EXTL3 cellular balance dictates Heparan Sulfate biosynthesis and shapes gastric cancer cell motility and invasion. Journal of Biological Chemistry, 102546.

Bajaj, S., Satoskar, P., Nair, A., Sheth, F., Sheth, J., & Sheth, H. (2022). An ultra-rare case of immunoskeletal dysplasia with neurodevelopmental abnormalities in an Indian patient with homozygous c. 953C> T variant in EXTL3 gene: a case report. BMC pediatrics, 22(1), 1-9.

Dupree, P., Dendooven, T., Hardwick, S., Tryfona, T., Chirgadze, D., & Luisi, B. (2022). The structure of EXTL3 helps to explain the different roles of bi-domain exostosins in heparan sulfate synthesis.

Akalın, A., Taskiran, E. Z., Şimşek‐Kiper, P. Ö., Utine, E., Alanay, Y., Özçelik, U., & Boduroğlu, K. (2021). Spondyloepimetaphyseal dysplasia EXTL3‐deficient type: Long‐term follow‐up and review of the literature. American Journal of Medical Genetics Part A, 185(10), 3104-3110.

Zhang, H., Corredor, A. L. G., Messina-Pacheco, J., Li, Q., Zogopoulos, G., Kaddour, N., ... & Gao, Z. H. (2021). REG3A/REG3B promotes acinar to ductal metaplasia through binding to EXTL3 and activating the RAS-RAF-MEK-ERK signaling pathway. Communications biology, 4(1), 1-13.

Yamada, S. (2020). Specific functions of Exostosin-like 3 (EXTL3) gene products. Cellular & molecular biology letters, 25(1), 1-12.

Aints, A., Mölder, S., & Salumets, A. (2019). EXTL3-interacting endometriosis-specific serum factors induce colony formation of endometrial stromal cells. Scientific reports, 9(1), 1-10.

Awad, W., Kjellström, S., Svensson Birkedal, G., Mani, K., & Logan, D. T. (2018). Structural and biophysical characterization of human EXTL3: domain organization, glycosylation, and solution structure. Biochemistry, 57(7), 1166-1177.

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

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