强伦轩一级A片在线观看,中文字幕乱码人妻二区三区,鲁鲁狠狠狠7777一区二区,西西4444www无码精品
掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯(lián)系我們
1000部毛片A片免费视频,国产精品国产三级国产专区53,久久精品一区二区三区四区
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-Iduronate 2 sulfatase/PE-Cy7 Conjugated antibody (bs-15543R-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-15543R-PE-Cy7
英文名稱 Rabbit Anti-Iduronate 2 sulfatase/PE-Cy7 Conjugated antibody
中文名稱 PE-Cy7標記的艾杜糖-2-硫酸酯酶抗體
別    名 Alpha L iduronate sulfate sulfatase; Alpha-L-iduronate sulfate sulfatase; AW214631; Ids; IDS_HUMAN; Iduronate 2 sulfatase 14 kDa chain; Iduronate 2 sulfatase 42 kDa chain; Iduronate 2 sulfatase; Iduronate 2-sulfatase 14 kDa chain; Iduronate sulfatase; Idursulfase; MPS2; RP23-29M4.1; SIDS.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  免疫學  細胞類型標志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,  (predicted: Mouse, Rat, Dog, Cow, Sheep, )
產(chǎn)品應用 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 47kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Iduronate 2 sulfatase
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:
The protein encoded by this gene belongs to the sulfatase family, is localized to the lysosome, and is involved in lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease, mucopolysaccharidosis type II, also known as Hunter syndrome. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Aug 2013]

Function:
Required for the lysosomal degradation of heparan sulfate and dermatan sulfate.

Subunit:
Liver iduronate 2-sulfatase is composed of two major forms (A and B) which contain both a 42 kDa and a 14 kDa polypeptides.

Subcellular Location:
Lysosome.

Tissue Specificity:
Liver, kidney, lung, and placenta.

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:
Mucopolysaccharidosis 2 (MPS2) [MIM:309900]: An X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the sulfatase family.

Database links:

Entrez Gene: 3423 Human

Omim: 309900 Human

SwissProt: P22304 Human

Unigene: 460960 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 nmgps.com 北京博奧森生物技術有限公司
通過國際質量管理體系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
欧美精品二三四区人妻斩 | 成年人黄色视频免费观看 | EEUSS鲁片一区二区三区四虎 | 亚洲综合一区二区三区 | 91麻豆产精品久久久久久夏晴子 | 国产又黄又大又粗的视频 | 苍井空无码a片免费看 | 真实乱视频国产海角社区 | 亚洲老女人性生交视频在线 | 寡妇高潮一级毛片在线播放一小说 | 国内精品国产成人国产三级 | 97精品人妻一区二区三区 | 精品A片老女人免费看一区 国产麻豆一级黄色视频资源 | 亚欧人妻精品AV熟女人妻 | 免费毛片网站在线观看 | 四川丰满少妇A级无码 | 一区二区三区高清无码 | 国产精品一区二555 精品一区二区三区人妻 | 91无码人妻精品一区二区三区四 | 国产激情无码AV毛片多多 | 91 黑料 精品 国产 | 国产欧美一区二区色老头 | 国产毛多水多女人一级 | 91九色中文视频在线观看 | 中文字幕一区成人电影 | 久久国产劲爆∧v内射 | 91在线午夜福利精品 | 亚洲精品福利在线观看 | 久久久久久亚洲精品 | 高潮白浆美女自慰网站 | 成人做爰A片免费看视频 | 国产精品 同 | 波多野结衣无码在线 | 一区二区三区毛A片特级 | 少妇被大黑捧猛烈进出的 | 久久久久久成人精品视频网站 | 18禁影库永久免费 | 最近中文字幕在线播放中 | 青青草国产一区二区三区 | 日本视频免费在线观看 | 午夜肉体高潮免费毛片 |