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
娇喘嗯嗯~轻点啊视频福利 ,亚洲最大的熟女水蜜桃AV网站,水蜜桃AV无码专区亚洲AV麻豆
Rabbit Anti-SGSH/AP Conjugated antibody (bs-11756R-AP)
訂購(gòu)熱線:400-901-9800
訂購(gòu)郵箱:sales@bioss.com.cn
訂購(gòu)QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-11756R-AP
英文名稱 Rabbit Anti-SGSH/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標(biāo)記的磺氨基葡糖硫酸胺酶抗體
別    名 Heparan sulfate sulfatase; Heparan sulphate sulphatase; HSS; MPS 3A; MPS3 A; MPS3A; N sulfoglucosamine sulfohydrolase (sulfamidase); N-sulphoglucosamine sulphohydrolase; SFMD; SGSH; SPHM_HUMAN; Sulfoglucosamine sulfamidase; Sulphamidase; Sulphoglucosamine sulphamidase.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞骨架  細(xì)胞外基質(zhì)  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Rat,  (predicted: Human, Mouse, Dog, )
產(chǎn)品應(yīng)用 WB=1:50-200 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 Sulphamidase (301-388aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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:
Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.

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.

DISEASE:
Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival.

Similarity:
Belongs to the sulfatase family.

Database links:

Entrez Gene: 6448 Human

Omim: 605270 Human

SwissProt: P51688 Human

Unigene: 31074 Human



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 a6308.cn 北京博奧森生物技術(shù)有限公司
通過(guò)國(guó)際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號(hào): 00124Q34771R2M/1100
通過(guò)國(guó)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)
国产二区三区中文字幕 | 成人国产精品日本在线 | 久久久久久久成人午夜精品福利 | 男人天堂·手机版在线 | 婷婷五月综合缴情在线视频 | 日本一二三区在线视频 | 无码毛片内射白浆视频 | 国产精品手机在线无码援交 | 欧美日韩无套内射另类 | 亚洲国产成人精品女人久久久 | 久久久久久精品成人免费 | 亚洲熟妇av一区二区三区色堂 | 免费国产黄网站在线观看可以下载 | 两个人看的视频www高清 | 国产精品亚洲第一区在线 | 成全视频免费高清观看在线 | 一本色道久久综合亚洲精品加 | 一级做a爰片久久毛片免费陪 | 欧美日韩国产综合视频一区二区 | 国产91精品太粉嫩高中在线观看 | www.一级毛片 | 国产精品久久久久无毒 | 日韩成人精品视频免费专区 | 白丝乳交内射一二三区 | 5278欧美一区二区三区 | 一本色道久久88综合亚洲精品 | 女人被狂躁到爽动态gif图 | 最近中文字幕MV免费高清动漫 | JAPANESE少妇出轨内射 | 91在线一区二区 | 国产精品国产三级国产AV剧情 | 欧美高清狂热视频韩国影院 | 免费一级毛片在线观看 | 亚洲va国产日韩欧美精品 | 97se色在在线视频 | 久久成人无码国产免费播放 | 使劲快高潮了国语对白在线 | 亚洲无人区码一二三码区别图片 | 久久精品久久久精品美女 | 一级毛片免费在线播放 | 中文字幕看片在线a免费 |