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Rabbit Anti-CHST6/Gold Conjugated antibody (bs-13937R-Gold)
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說(shuō) 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-13937R-Gold
英文名稱 Rabbit Anti-CHST6/Gold Conjugated antibody
中文名稱 膠體金標(biāo)記的碳水化合物磺基轉(zhuǎn)移酶6抗體
別    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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul(10nm  15nm  35nm
研究領(lǐng)域 神經(jīng)生物學(xué)  細(xì)胞類型標(biāo)志物  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,  (predicted: Dog, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CHST6
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產(chǎn)品介紹 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.

Similarity:
Belongs to the sulfotransferase 1 family. Gal/GlcNAc/GalNAc subfamily.

Database links:

Entrez Gene: 4166 Human

Omim: 605294 Human

SwissProt: Q9GZX3 Human

Unigene: 655622 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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