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(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  關(guān)于我們  聯(lián)系我們
日本在线不卡一区二区三区,国内精品视频久久久,久热久久在线观看精品视频
Rabbit Anti-BSCL2/Cy5.5 Conjugated antibody (bs-6311R-Cy5.5)
訂購熱線:400-901-9800
訂購郵箱:sales@m.p2b3.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@m.p2b3.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-6311R-Cy5.5
英文名稱 Rabbit Anti-BSCL2/Cy5.5 Conjugated antibody
中文名稱 Cy5.5標(biāo)記的先天性脂肪代謝障礙蛋白2抗體(常染色體顯性遺傳痙攣性截癱17)
別    名 Bernardinelli Seip congenital lipodystrophy 2; Bernardinelli Seip congenital lipodystrophy type 2 protein; Bernardinelli-Seip congenital lipodystrophy type 2 protein; BSCL 2; BSCL2; BSCL2_HUMAN; GNG3LG; HMN 5; HMN5; MGC4694; Seipin; Spastic paraplegia 17 (autosomal dominant); Spastic paraplegia 17 (Silver syndrome); Spastic paraplegia 17; Spastic paraplegia with amyotrophy of hands and feet (Silver syndrome); Spastic paraplegia with amyotrophy of hands and feet; SPG 17; SPG17.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 心血管  細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  細(xì)胞類型標(biāo)志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BSCL2/SPG17
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:
Defects in BSCL2 are the cause of congenital generalized lipodystrophy type 2 (CGL2) . Congenital generalized lipodystrophy is an autosomal recessive disorder characterized by a near absence of adipose tissue, extreme insulin resistance, hypertriglyceridemia, hepatic steatosis and early onset of diabetes.
Defects in BSCL2 are the cause of spastic paraplegia type 17 (SPG17) ; also known as Silver spastic paraplegia syndrome. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG17 is characterized by prominent amyotrophy of the hand muscles, the presence of mild to severe pyramidal tract signs, and spastic paraplegia.
SPG17 is a motor neuron disease overlapping with distal spinal muscular atrophy type 5. Defects in BSCL2 are a cause of distal hereditary motor neuropathy type 5 (HMN5); also known aS distal hereditary motor neuropathy type V (DSMAV). HMN5 is an autosomal dominant disorder characterized by degeneration of motor nerve fibers, predominantly in limb distal regions.

Function:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

Tissue Specificity:
Highest expression in brain and testis.

DISEASE:
Defects in BSCL2 are the cause of congenital generalized lipodystrophy type 2 (CGL2) [MIM:269700]. Congenital generalized lipodystrophy is an autosomal recessive disorder characterized by a near absence of adipose tissue, extreme insulin resistance, hypertriglyceridemia, hepatic steatosis and early onset of diabetes.
Defects in BSCL2 are the cause of spastic paraplegia type 17 (SPG17) [MIM:270685]; also known as Silver spastic paraplegia syndrome. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG17 is characterized by prominent amyotrophy of the hand muscles, the presence of mild to severe pyramidal tract signs, and spastic paraplegia. SPG17 is a motor neuron disease overlapping with distal spinal muscular atrophy type 5.
Defects in BSCL2 are a cause of distal hereditary motor neuropathy type 5 (HMN5) [MIM:600794]; also known aS distal hereditary motor neuropathy type V (DSMAV). HMN5 is an autosomal dominant disorder characterized by degeneration of motor nerve fibers, predominantly in limb distal regions.

Similarity:
Belongs to the seipin family.

Database links:
UniProtKB/Swiss-Prot: Q96G97.3

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.m.p2b3.cn 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
找个日韩操逼的看看| 国产熟女露脸普通话对白| 久久久久精品无码专区喝奶| 韩国无玛黄片毛片| 巨屌抽插舔阴视频| 国产黄片在线免费看| 男生狂操女生污视频| 日韩视频在线网页| 国产又粗又猛又色又免费| 日本免费一区二区在线| 一区二区三区av精品| 亚洲精品精品精国产| 国产精品一区二区在线观看91| 久久精品国产自清天天线| 日本男人捅女人机机| 欧美日韩久久一区二区三区| 中文字幕av一区二区三区蜜桃| 亚洲欧洲综合成人综合网| 精品久久久久中文字幕人| 亚欧日韩国产在线| 插欧美美女逼逼逼逼| 操女人b直播软件| 精品的极品美女一区二区三区| 亚洲欧美日韩中文v在线| 女人被男人躁爽色欲国产| 亚洲国产区男人本色| 国产高清在线观看一区二区三区| 天天爽夜夜爽夜夜爽一区| 美女骚逼黄色18禁| 欧美日韩在线成人| 久久久久黑人强伦姧人妻| 久操视频中文字幕在线观看| 日韩午夜资源在线观看| 大香蕉中码手机在线视频| 无码毛片一区二区本码视频| 精彩欧美一区二区三区| 毛片日产av一区二区三区四区| 老司机免费福利午夜入口| 亚洲Av无码专区一区二区三区| 日韩美女叉B视频| 操的我的逼逼好爽好多水|