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
免费无码专区毛片高潮喷水,国产精品久久久久久久久齐齐
Rabbit Anti-GALNS/PE-Cy7 Conjugated antibody (bs-13269R-PE-Cy7)
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-PE-Cy7
英文名稱 Rabbit Anti-GALNS/PE-Cy7 Conjugated antibody
中文名稱 PE-Cy7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 ICC=1:50-200 IF=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
51视频精品全部免费的意义在哪里 | 久久成人永久免费播放 | 女人高潮喷浆毛片视频 | 久久久国产精品综合无人区 | 日韩内射美女人妻一区二区三区 | 亚洲乱码国产一区三区 | 狠狠躁日日躁夜夜躁2022麻豆 | 亚洲国产在线精品一区在 | 欧美白人极品性喷潮 | 奇米成人影院777欧美极品 | 蜜臀久久久久精品久久久 | 水蜜桃AV无码专区亚洲AV麻豆 | 久热香蕉在线爽青青 | 国内精品久久久久久久试看 | 日本精品一区二区三区在线 | 午夜视频在线观看国产 | 少妇无码太爽了不卡视频在线看 | 国产高清中文手机在线观看 | 琪琪精品视频在线观看 | 在线观看免费人成视频色 | 麻豆国产AV尤物网站尤物 | 最近的2019中文字幕国语HD | 国产日产欧产精品精品浪潮 | 天美传媒TMY0013在线观看 | 欧美激情视频在线观看一区二区三区 | 国产伦一区二区三区四区 | 又大又爽又硬的曰皮视频 | 向日葵视频APP秋葵榴莲在线 | 国产69精品久久久久无码小说 | 欧美又大又粗又硬BBBBB小说 | 久久综合给合久久狠狠狠97色 | 18禁无遮挡羞羞污污污污网站 | 97亚洲熟妇自偷自拍另类图片 | 好爽插到我子宫了高清在线 | 成人网站www污污污网站 | 忍不住亲子性行为中文字幕 | 欧美第一区重口sm虐性奴视频 | 欧美高清视频www夜色资源网 | 无码一区18禁3D | www.99在线 | 99国产欧美久久久精品 |