来一水AV@lysav|亚洲AV无码片VR一区二区三区 |国产亚洲精久久久久久无码|视色4se成人午夜精品久久

掃碼關(guān)注公眾號           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  人才招聘  關(guān)于我們  聯(lián)系我們
欧美中文字幕一区在线|亚洲狠狠婷婷综合久久久久图片|无码午夜看片爽爽在线视频
Rabbit Anti-SUMF1/BF594 Conjugated antibody (bs-12366R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@73327.net
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@73327.net
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-12366R-BF594
英文名稱1 Rabbit Anti-SUMF1/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的硫酸酯酶修飾因子1抗體
別    名 MGC150436; AAPA3037; C alpha formylglycine generating enzyme 1; C-alpha-formylglycine-generating enzyme 1; FGE; FGly generating enzyme; MGC131853; Sulfatase modifying factor 1 [Precursor]; Sulfatase-modifying factor 1; SUMF1; SUMF1_HUMAN; UNQ3037.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  發(fā)育生物學(xué)  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  干細(xì)胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng)
產(chǎn)品應(yīng)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 37kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SUMF1
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:
SUMF1 is a 374 amino acid alternatively spliced protein that localizes to the lumen of the endoplasmic reticulum and belongs to the sulfatase-modifying factor family. Expressed ubiquitously with highest expression in liver, kidney and pancreas, SUMF1 exists as either a monomer, a homodimer or a heterodimer (with SUMF2) and functions to oxidize sulfatase cysteine residues to an active FGIy residue, thereby playing an important role in sulfatase activity. Defects in the gene encoding SUMF1 are the cause of multiple sulfatase deficiency (MSD), a heterogeneous disorder characterized by metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.

Function:
Using molecular oxygen and an unidentified reducing agent, oxidizes a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also called C(alpha)-formylglycine. Known substrates include GALNS, ARSA, STS and ARSE.

Subunit:
Monomer, homodimer and heterodimer with SUMF2.

Subcellular Location:
Endoplasmic reticulum lumen.

Tissue Specificity:
Ubiquitous. Highly expressed in kidney, pancreas and liver. Detected at lower levels in leukocytes, lung, placenta, small intestine, skeletal muscle and heart.

Post-translational modifications:
N-glycosylated. Contains high-mannose-type oligosaccharides.

DISEASE:
Defects in SUMF1 are the cause of multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Inheritance is autosomal recessive.

Similarity:
Belongs to the sulfatase-modifying factor family.

Database links:
UniProtKB/Swiss-Prot: Q8NBK3.3

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.73327.net 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: 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: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
欧美日韩精品一区二区在线播放| 日本精品网站在线观看| 国产精品玖玖玖在线| 亚欧洲精品在线视频免费观看 | 亚洲国产成人精品女人久久久| 免费看久久精品视频| 99精品视频在线观看免费| 小草影院| 中文字幕亚洲无线码在线一区| 精品黑人一区二区三区| 疯狂的肥岳交换130部小短片| 亚洲欧美激情啪啪啪| 国产特级毛片A片WWW| 久久精品国产经典免费电影| 超大胆丝袜人妻无码系列| 最近韩国日本免费高清观看 | 无码JK粉嫩小泬在线观看欧美| 精品免费久久久久久| 夜夜嗨老熟女av一区二区三区| 亚洲高清自有码中文字在线观看| 老司机午夜免费精品视频| 中国丰满人妻VIDEOSHD| 亚洲色坉15| 99精品久久久久久久婷婷| 女同久久精品国产99国产精品| 人与禽性视频77777| 婷婷综合久久中文字幕蜜桃三电影 | 国产小呦泬泬99精品| 将军与娇妻各种做高H| 亚洲精品久久久久久一区二区| 亚洲精品一区三区三区在线观看| 亚洲熟妇AV一区二区三区漫画| 无码人妻一区二区三区在线| 人妻中文字幕无码专区| 亚洲中文字幕在线19页| 日韩av一区二区三区精品| 欧美久久久久久精选大尺度| 国产精品尤物在线免费观看 | 久久久中日AB精品综合| 久久99精品久久久久久琪琪| 亚洲综合在线视频一区二区三区|