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(hào)           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
国产在线观看不卡,强奸伦久久久久久久久久久特级av,两个人看的www视频中文字幕
Rabbit Anti-GALNS/Gold Conjugated antibody (bs-13269R-Gold)
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(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-13269R-Gold
英文名稱 Rabbit Anti-GALNS/Gold Conjugated antibody
中文名稱 膠體金標(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(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul(10nm  15nm  35nm
研究領(lǐng)域 細(xì)胞生物  信號(hào)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 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 55kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/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(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:
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(hào): 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(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
在线中文字幕亚洲第一 | 免费看撕开奶罩揉吮奶头视频 | 精品人妻aV中文字幕乱码 | jk黑色丝袜美腿老师啪啪 | 国产91色在线综合亚洲 | 一级a性色生活片久久无码火 | 亚洲欧美日韩中文加勒比 | 日本一级A片在线观看播放 久久精品中文字幕第一页国产精品 | 亚洲国产精品无码久久久久久曰 | 国产精品99久久久久久 | 国产成人精品三级在线 | 亚洲成AV人在线播放无码 | 九九综合VA免费看 | 亚洲一区精品在线 | 老熟女太熟了x98AV | 亚洲韩欧美第25集完整版 | 免费看**a一级毛片 国产精品无码AV片在线专区 | 中文字幕久久久久久久免费 | 欧美A∨一区二区三区久久黄 | 无码AV天堂亚洲内射夫妻 | 最近中文字幕高清字幕mv | 欧美被狂躁喷白浆精品 | 亚洲天天在线日亚洲洲精 | 韩日美无码精品无码 | 嫩小BBB揉BBB揉BBBB | 午夜视频在线观看国产 | 成年片色大黄全免费网站观看 | 亚洲综合精品伊人久久 | 99视频精品全部免费 | 性xxxxbbbb免费播放视频 | 中文日产乱幕九区无线码 | 久久国产精品99精国产 | 国产成a人亚洲精v品久久网 | 欧美两根一起进3p在线观看 | 日本不卡高字幕在线2019 | 婷婷色婷婷开心五月四房播播久久 | 午夜精品久久久久久不卡AV | 亚洲AV成人男人的天堂网 | 久久久久亚洲精品影视 | 久久久久久久久久久免费精品国产 | 无码射肉在线播放视频 |