亚洲制服欧美另类-午夜激情av电影-日本高清中文字幕一区二区三区-中国欧美日韩一区二区三区-欧洲亚洲日本韩国-成人欧美激情一区二区-亚洲偷偷自拍高清

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
人妻欧美精品AAAAAAAAA片,成年免费视频播放网站推荐
Rabbit Anti-GALNS/HRP Conjugated antibody (bs-13269R-HRP)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-13269R-HRP
英文名稱 Rabbit Anti-GALNS/HRP Conjugated antibody
中文名稱 辣根過氧化物酶標(biāo)記的硫酸軟骨素裂解酶抗體
別    名 Chondroitinase; Chondroitinsulfatase; FLJ00319; FLJ17434; FLJ42844; FLJ98217; Galactosamine (N acetyl) 6 sulfate sulfatase; Galactose 6 sulfate sulfatase; GALNAC6S; GalNAc6S sulfatase; GAS; mFLJ00319; MPS4A; N acetylgalactosamine 6 sulfatase; N acetylgalactosamine 6 sulfate sulfatase; GALNS_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  信號轉(zhuǎn)導(dǎo)  泛素  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用 WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 55kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GALNS
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
Chondroitinase is a 522 amino acid protein that localizes to the lysosome and functions as an exohydrolase that is essential for the degradation of glycosaminoglycans, keratan sulfate and chondroitin 6-sulfate. Using calcium as a cofactor, Chondroitinase, which exists as a disulfide linked oligomer, catalyzes the hydrolysis of the 6-sulfate group on target substrates. Defects in the gene encoding Chondroitinase are the cause of mucopolysaccharidosis type 4A (MPS4A), an autosomal recessive lysosomal storage disease that is characterized by the intracellular accumulation of keratan sulfate and chondroitin-6-sulfate and is associated with dental anomalies, short stature and, in some cases, death in the second or third decade of life.

Function:
GALNS (N-acetylgalactosamine-6-sulfatase) is a lysosomal exohydrolase which catalyzes the hydrolysis of the 6-sulfate groups of the N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate and of the D-galactose 6-sulfate units of keratan sulfate. Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder.

Subunit:
Oligomer of disulfide linked 40- and 15 kDa polypeptides.

Subcellular Location:
Lysosome.

Post-translational modifications:
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity (By similarity).

DISEASE:
Defects in GALNS are the cause of mucopolysaccharidosis type 4A (MPS4A) [MIM:253000]; also known as Morquio A syndrome. MPS4A is a form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life.

Similarity:
Belongs to the sulfatase family.

Database links:
UniProtKB/Swiss-Prot: P34059.1

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權(quán)所有 2004-2026 www.a6308.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
久久刺激cijilu福利72 | 国产在线一区观看 | 国产又猛又黄又爽 | 国产成人无码AⅤ片在线 | 久久久这里只有免费精品2018 | AV蜜臀在线网站 | 精品国产90后在线观看 | 把手戳进美女尿口里动态图 | 成年网站免费视频A在线双飞 | 国产精品福利在线观看免费不卡 | 国内精品久久久久久网站 | 国产一卡 二卡三卡四卡无卡乱码视频 | 亚洲日本香蕉观看观视频 | 成年网站免费人性视频a站 老司机在线精品视频网站 亚洲色熟女图激情另类图区 | 久久免费看少妇级毛片蜜臀 | 国产又粗又黄又爽的大片 | 精品久久久久久亚洲精品 | 夜夜爱成人免费网站 | 成熟丰满女人免费视频 | 免费无码又爽又刺激高潮久久 | 绿意盎然侄子小钢炮的成长经历 | 好吊妞无码中文字幕在线视频 | 免费国产美女爽到喷出水来视频 | 2019午夜福利不卡片在线 | 精品一区二区三区高清免费观看 | 久久青青草原精品国产软件 | 看亚洲A级一级毛片,亚洲AV片 | 国产女人十八毛片A级毛片 综合欧美亚洲首页在线播放 | 放荡的小峓子2中文字幕 | 91在线一区二区 | 亚洲中文字幕乱码免费播放 | 伊人99综合精品视频 | 欧美野性肉体狂欢大派对 | 久久久久精品国产未满十八禁 | 一区二区三区精品视频 | 日韩欧美一区二区三区免费看 | 国产精品991TV制片厂在线观看 | 50岁老熟女露脸25分钟91 | 麻豆精品一区二正一三区 | 爆乳护士一区二区三区在线播放 | 成人影片麻豆国产影片免费观看 |