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Mouse Anti-CHST6 Recombinant Antibody (CBWJC-2708) (CBMAB-C3801WJ)

This product is a Mouse antibody that recognizes CHST6. This antibody CBWJC-2708 can be used for immunoassay techniques such as: WB.
See all CHST6 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Dog, Monkey, Mouse
Clone
CBWJC-2708
Antibody Isotype
IgG1
Application
WB

Basic Information

Immunogen
Human recombinant protein fragment corresponding to aa202-395 of human CHST6 (NP_067628) produced in E.coli
Specificity
Human, Dog, Monkey, Mouse
Antibody Isotype
IgG1
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
Buffer
PBS (PH 7.3), 1% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
1 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.

Target

Full Name
Carbohydrate Sulfotransferase 6
Introduction
The protein encoded by this gene is an enzyme that catalyzes the transfer of a sulfate group to the GlcNAc residues of keratan. Keratan sulfate helps maintain corneal transparency. Defects in this gene are a cause of macular corneal dystrophy (MCD). [provided by RefSeq, Jan 2010]
Entrez Gene ID
Human4166
Mouse116070211
Dog489707
Monkey711570
UniProt ID
HumanQ9GZX3
MonkeyF6S4Z1
Alternative Names
Carbohydrate Sulfotransferase 6; Galactose/N-Acetylglucosamine/N-Acetylglucosamine 6-O-Sulfotransferase 4-Beta; Carbohydrate (N-Acetylglucosamine 6-O) Sulfotransferase 6; Corneal N-Acetylglucosamine-6-O-Sulfotransferase; N-Acetylglucosamine 6-O-Sulfotransferase 5; GlcNAc6ST-5; C-GlcNAc6ST; GST4-Beta; Gn6st-5;
Function
Sulfotransferase that utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as sulfonate donor to catalyze the transfer of sulfate to position 6 of non-reducing N-acetylglucosamine (GlcNAc) residues of keratan. Mediates sulfation of keratan in cornea. Keratan sulfate plays a central role in maintaining corneal transparency. Acts on the non-reducing terminal GlcNAc of short and long carbohydrate substrates that have poly-N-acetyllactosamine structures.
Biological Process
Carbohydrate metabolic process Source: UniProtKB-KW
Keratan sulfate biosynthetic process Source: UniProtKB
N-acetylglucosamine metabolic process Source: UniProtKB
Sulfur compound metabolic process Source: UniProtKB
Cellular Location
Golgi apparatus membrane
Involvement in disease
Macular dystrophy, corneal (MCD):
The disease is caused by variants affecting the gene represented in this entry. CHST6 homozygous missense mutations have been observed in patients with macular corneal dystrophy type I, while type II patients show a large deletion and replacement in the upstream region of CHST6. The only missense mutation for type II is Cys-50, which is heterozygous with a replacement in the upstream region on the other allele of CHST6. An ocular disease characterized by bilateral, progressive corneal opacification, and reduced corneal sensitivity. Onset occurs in the first decade, usually between ages 5 and 9. Painful attacks with photophobia, foreign body sensations, and recurrent erosions occur in most patients. The disease is due to deposition of an unsulfated keratan sulfate both within the intracellular space (within the keratocytes and endothelial cells) and in the extracellular corneal stroma. Macular corneal dystrophy is divided into the clinically indistinguishable types I, IA, and II based on analysis of the normally sulfated, or antigenic, keratan sulfate levels in serum and immunohistochemical evaluation of the cornea. Patients with types I and IA macular corneal dystrophy have undetectable serum levels of antigenic keratan sulfate, whereas those with type II macular corneal dystrophy have normal or low levels, depending on the population examined.
Topology
Cytoplasmic: 1-5
Helical: 6-26
Lumenal: 27-395

Hao, X. D., Liu, Y. N., Hu, S. H., Pan, X. J., & Chen, P. (2022). Association of macular corneal dystrophy with excessive cell senescence and apoptosis induced by the novel mutant CHST6. Experimental Eye Research, 214, 108862.

Huang, Y., Yuan, L., Cao, Y., Tang, R., Xu, H., Tang, Z., & Deng, H. (2021). Novel compound heterozygous mutations in the CHST6 gene cause macular corneal dystrophy in a Han Chinese family. Annals of Translational Medicine, 9(8).

Li, D., Tian, L., Wang, X., & Chen, M. (2021). Macular corneal dystrophy related to novel mutations of CHST6 in a Chinese family and clinical observation after penetrating keratoplasty. BMC Medical Genomics, 14(1), 1-9.

Safari, I., Baradaran-Rafii, A., Issazadeh-Navikas, S., & Elahi, E. (2020). CHST6 mutations identified in Iranian MCD patients and CHST6 mutations reported worldwide identify targets for gene editing approaches including the CRISPR/Cas system. International Ophthalmology, 40, 2223-2235.

Zhang, J., Wu, D., Li, Y., Fan, Y., Dai, Y., & Xu, J. (2019). A comprehensive evaluation of 181 reported CHST6 variants in patients with macular corneal dystrophy. Aging (Albany NY), 11(3), 1019.

Jing, Y., Zhou, Y., Wang, C., Liu, J., Guo, Y., Mao, S., ... & Chen, J. (2019). Establishment of a non-integrate iPS cell line CSUASOi002-A, from urine-derived cells of a female patient with macular corneal dystrophy carrying compound heterozygous CHST6 mutations. Stem cell research, 41, 101598.

Murugan, D., Prajna, N. V., Devi, L., & Sundaresan, P. (2017). Genetic Analysis of CHST6 Gene in Indian Families with Macular Corneal Dystrophy.

Wang, L., Tang, X., Lv, X., Sun, E., Wu, D., Wang, C., & Liu, P. (2017). CHST6 mutation screening and endoplasmatic reticulum stress in macular corneal dystrophy. Oncotarget, 8(56), 96301.

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

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