Product datasheet
Anti-CHST6 antibody ab154332
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Overview
Product name
Anti-CHST6 antibody
Description
Rabbit polyclonal to CHST6
Tested applications
WB
Species reactivity
Reacts with: Human
Predicted to work with: Cow
Immunogen
Recombinant fragment, corresponding to a region within amino acids 57-340 of Human CHST6
(Uniprot ID: Q9GZX3).
Positive control
HeLa, 293T, A431, H1299, HepG2, MOLT4 and Raji whole cell lysates.
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage buffer
pH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 0.75% Glycine, 1.21% Tris, 10% Glycerol
Purity
Immunogen affinity purified
Clonality
Polyclonal
Isotype
IgG
Applications
Our Abpromise guarantee covers the use of ab154332 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application
WB
Abreviews
Notes
1/500 - 1/3000. Predicted molecular weight: 44 kDa.
Target
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)
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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.
Tissue specificity
Expressed in cornea. Mainly expressed in brain. Also expressed in spinal cord and trachea.
Involvement in disease
Defects in CHST6 are the cause of macular dystrophy, corneal, 1 (MCDC1) [MIM:217800]. 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.
Note=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.
Sequence similarities
Belongs to the sulfotransferase 1 family. Gal/GlcNAc/GalNAc subfamily.
Cellular localization
Golgi apparatus membrane.
Anti-CHST6 antibody images
Anti-CHST6 antibody (ab154332) at 1/1000
dilution + HeLa whole cell lysate. at 30 µg
Predicted band size : 44 kDa
Western blot - Anti-CHST6 antibody (ab154332)
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