mm1313亚洲精品,欧美俄罗斯40老熟妇,欧美日韩在线观看视频在线,亚洲欧美国产激情综合在线

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  人才招聘  關于我們  聯(lián)系我們
日韩欧美中文字幕在线一二三区,欧美精品久久人妻水a激情
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-NAGLU/Biotin Conjugated antibody (bs-19002R-Bio)
訂購熱線:400-901-9800
訂購郵箱:sales@www.p2b3.cn
訂購QQ:  400-901-9800
技術支持:techsupport@www.p2b3.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-19002R-Bio
英文名稱1 Rabbit Anti-NAGLU/Biotin Conjugated antibody
中文名稱 生物素標記的N-乙?;?α葡萄糖苷酶抗體
別    名 Alpha N acetylglucosaminidase; alpha N acetylglucosaminidase, lysosomal; Alpha-N-acetylglucosaminidase 77 kDa form; ANAG; ANAG_HUMAN; MPS IIIB; MPS3B; N acetyl alpha glucosaminidase; N acetylglucosaminidase, alpha; N-acetyl-alpha-glucosaminidase; NAG; NAGLU; UFHSD 1; UFHSD; UFHSD1.  
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  神經(jīng)生物學  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Dog, Pig, Horse, Rabbit, Sheep, )
產品應用 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 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 80kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NAGLU
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.
產品介紹 background:
This gene encodes an enzyme that degrades heparan sulfate by hydrolysis of terminal N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides. Defects in this gene are the cause of mucopolysaccharidosis type IIIB (MPS-IIIB), also known as Sanfilippo syndrome B. This disease is characterized by the lysosomal accumulation and urinary excretion of heparan sulfate. [provided by RefSeq, Jul 2008]

Function:
Involved in the degradation of heparan sulfate.

Subcellular Location:
Lysosome.

Tissue Specificity:
Liver, ovary, peripheral blood leukocytes, testis, prostate, spleen, colon, lung, placenta and kidney.

DISEASE:
Defects in NAGLU are the cause of mucopolysaccharidosis type 3B (MPS3B) [MIM:252920]; also known as Sanfilippo syndrome B. MPS3B is a 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.

Database links:

Entrez Gene: 4669 Human

Entrez Gene: 789125 Cow

Entrez Gene: 27419 Mouse

Omim: 609701 Human

SwissProt: P54802 Human

Unigene: 50727 Human

Unigene: 95452 Mouse



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 www.www.p2b3.cn 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
平乐县| 平定县| 海南省| 舟曲县| 石河子市| 城市| 白玉县| 湾仔区| 响水县| 永丰县| 腾冲县| 册亨县| 和林格尔县| 昆山市| 抚州市| 奇台县| 南昌县| 天峻县| 榆社县| 陆丰市| 沐川县| 班玛县| 海阳市| 弥勒县| 当雄县| 若羌县| 武安市| 广元市| 离岛区| 昌都县| 莫力| 凤冈县| 大名县| 田阳县| 浮山县| 桃江县| 池州市| 六枝特区| 临沂市| 曲沃县| 道真|