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Rabbit Anti-Nesprin 1/Gold Conjugated antibody (bs-19207R-Gold)
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(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-19207R-Gold
英文名稱 Rabbit Anti-Nesprin 1/Gold Conjugated antibody
中文名稱 膠體金標記的突觸核膜蛋白1抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 心血管  細胞生物  神經(jīng)生物學  細胞分化  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Sheep, )
產(chǎn)品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 1010kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Nesprin 1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產(chǎn)品介紹 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.
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