Anti-CHST6抗体The protein encoded by this gene is an enzyme that catalyzes the transfer of a sulfate group to the GlcNAc residues of keratan.

产品编号 yb-13937R
英文名称 Anti-CHST6抗体
中文名称 碳水化合物磺基转移酶6抗体
别    名 C GlcNAc6ST; C-GlcNAc6ST; Carbohydate sulfotransferase 6; Carbohydrate (N acetylglucosamine 6 O) sulfotransferase 6; Carbohydrate sulfotransferase 6; CHST6; CHST6_HUMAN; Corneal GlcNAc6-sulfotransferase; Corneal N acetylglucosamine 6 sulfotransferase; Corneal N-acetylglucosamine-6-O-sulfotransferase; Galactose N acetylglucosamine N acetylglucosamine 6 O sulfotransferase 4 beta; Galactose/N-acetylglucosamine/N-acetylglucosamine 6-O-sulfotransferase 4-beta; GlcNAc6ST 5; GlcNAc6ST-5; Gn6st-5; GST4 beta; GST4-beta; hCGn6ST; N-acetylglucosamine 6-O-sulfotransferase 5.
Anti-CHST6抗体  
说 明 书 0.1ml  0.2ml  
研究领域 神经生物学  细胞类型标志物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Dog, Cow, Horse, Sheep, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human CHST6
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
产品介绍 background:
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]

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.

Subcellular Location:
Golgi apparatus membrane.

Tissue Specificity:
Expressed in cornea. Mainly expressed in brain. Also expressed in spinal cord and trachea.

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.