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 m.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
中文字幕人妻无码精品一区二区 | 亚洲AV无码乱码棈品熟妇 | 熟女俱乐部专干老妇女 | 影音先锋av在线资源 | 日本黄色视频免费看 | 苍井空一级婬片A片免费观看 | 多毛人妻日韩精品无码专区加勒比亚 | 天天综合,91综合永久 | 乱码一区二区三区四区精品蜜桃久久 | 成人A片产无码免费视频奶头鸭度 | JK白丝自慰一区二区免费 | 波多野结衣无码伦理片 | 中文字幕曰本髙清无码 | 18禁污黄网站在线播放 | 国产少妇性乱高潮国产传媒 | 欧美人与禽乱婬A片 | 日日摸人人爽av熟女 | 美女搡BBB又爽又猛又黄www | 扒开腿挺进肉嫩小泬18禁 | 姝姝窝人体www聚色窝 | 爆乳熟妇一区二区三区爆乳视频 | 少妇高潮灌满白浆毛片免费看 | 久久久久久久久va豆产国 | 黄色视频在线免费播放 | 精产品99永久免费网页版 | 精品久久AVA片免费播放 | 91精品国产一区二区三竹菊影视 | 17c久久精品国产亚洲 | 亚洲精品久久久久毛片A级绿茶 | 韩国黑寡妇一级毛片 | 国产传媒免费在线观看 | 国产伦精品一区二区三区88AV | 全免费A级毛片免费看网站招嫖 | 亚洲精品无码毛片久道具明星 | AA片在线观看视频在线播放 | 国产秘 精品一区二区三区免费 | 国产无人区一区二区三区四区 | 肉丝祙做爰在线播放无码 | 三亚三黄三色AAA毛片 | 中文丝袜字幕在线播放 | 国产又黄又粗在线观看 |