扫码关注公众号           扫码咨询技术支持           扫码咨询技术服务
  
客服热线:400-901-9800  客服QQ:4009019800  技术答疑  技术支持  质量反馈  人才招聘  关于我们  联系我们
产品中心-北京博奥森生物技术有限公司
首页 > 产品中心 > 标记一抗 > 产品信息
Rabbit Anti-Nesprin 1/PE Conjugated antibody (bs-19207R-PE)
~~~促销,代码KT202502A~~~
~~~促销,代码KT202502B~~~
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
技术支持:techsupport@bioss.com.cn
说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-19207R-PE
英文名称 Rabbit Anti-Nesprin 1/PE Conjugated antibody
中文名称 PE标记的突触核膜蛋白1抗体
别    名 CPG2; Enaptin; Myne-1; MYNE1; Myocyte nuclear envelope protein 1; Nesprin-1; Nuclear envelope spectrin repeat protein 1; SCAR8; Synaptic nuclear envelope protein 1; Syne-1; SYNE1; SYNE1_HUMAN; SYNE1B.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 心血管  细胞生物  神经生物学  细胞分化  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Sheep, )
产品应用 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 1010kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Nesprin 1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 background:
This gene encodes a spectrin repeat containing protein expressed in skeletal and smooth muscle, and peripheral blood lymphocytes, that localizes to the nuclear membrane. Mutations in this gene have been associated with autosomal recessive spinocerebellar ataxia 8, also referred to as autosomal recessive cerebellar ataxia type 1 or recessive ataxia of Beauce. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]

Function:
Multi-isomeric modular protein which forms a linking network between organelles and the actin cytoskeleton to maintain the subcellular spatial organization. Component of SUN-protein-containing multivariate complexes also called LINC complexes which link the nucleoskeleton and cytoskeleton by providing versatile outer nuclear membrane attachment sites for cytoskeletal filaments. Involved in the maintenance of nuclear organization and structural integrity. Connects nuclei to the cytoskeleton by interacting with the nuclear envelope and with F-actin in the cytoplasm. Required for centrosome migration to the apical cell surface during early ciliogenesis.

Subcellular Location:
Nucleus outer membrane. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere. The largest part of the protein is cytoplasmic, while its C-terminal part is associated with the nuclear envelope, most probably the outer nuclear membrane. In skeletal and smooth muscles, a significant amount is found in the sarcomeres.

Tissue Specificity:
Widely expressed. Highly expressed in skeletal and smooth muscles, heart, spleen, and peripheral blood leukocytes.

DISEASE:
Defects in SYNE1 are the cause of spinocerebellar ataxia autosomal recessive type 8 (SCAR8) [MIM:610743]; also known as autosomal recessive cerebellar ataxia type 1 (ARCA1) or recessive ataxia of Beauce. Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCAR8 is an autosomal recessive form.
Defects in SYNE1 are the cause of Emery-Dreifuss muscular dystrophy type 4 (EDMD4) [MIM:612998]. A degenerative myopathy characterized by weakness and atrophy of muscle without involvement of the nervous system, early contractures of the elbows, Achilles tendons and spine, and cardiomyopathy associated with cardiac conduction defects.

Similarity:
Belongs to the nesprin family.
Contains 1 actin-binding domain.
Contains 2 CH (calponin-homology) domains.
Contains 12 HAT repeats.
Contains 1 KASH domain.
Contains 31 spectrin repeats.

Database links:

Entrez Gene: 23345 Human

Entrez Gene: 64009 Mouse

Entrez Gene: 499010 Rat

Omim: 608441 Human

SwissProt: Q8NF91 Human

SwissProt: Q6ZWR6 Mouse

Unigene: 12967 Human

Unigene: 331626 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版权所有 2004-2026 www.bioss.com.cn 北京博奥森生物技术有限公司
通过国际质量管理体系ISO 9001:2015 GB/T 19001-2016    证书编号: 00124Q34771R2M/1100
通过国际医疗器械-质量管理体系ISO 13485:2016 GB/T 42061-2022    证书编号: CQC24QY10047R0M/1100
京ICP备05066980号-1         京公网安备110107000727号