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Rabbit Anti-Nesprin 2  antibody (bs-19208R)  
~~~促銷代碼KT202411~~~
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產(chǎn)品編號 bs-19208R
英文名稱 Rabbit Anti-Nesprin 2  antibody
中文名稱 突觸核膜蛋白2抗體
別    名 Nesprin2; Nesprin-2; DKFZP434H2235; DKFZp686E01115; DKFZp686H1931; FLJ11014; FLJ43727; FLJ45710; FLJ46790; KIAA1011; Nesprin-2; Nesprin2; NUA; NUANCE; Nuclear envelope spectrin repeat protein 2; Nucleus and actin connecting element; Nucleus and actin connecting element protein; Protein NUANCE; Spectrin repeat containing nuclear envelope 2; Synaptic nuclear envelope protein 2; Synaptic nuclei expressed gene 2; SYNE 2; Syne-2; SYNE2; SYNE2_HUMAN.  
研究領(lǐng)域 心血管  細胞生物  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 796kDa
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Nesprin 2: 3801-3900/6885 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 The protein encoded by this gene is a nuclear outer membrane protein that binds cytoplasmic F-actin. This binding tethers the nucleus to the cytoskeleton and aids in the maintenance of the structural integrity of the nucleus. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009]

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. Specifically, SYNE2 and SUN2 assemble in arrays of transmembrane actin-associated nuclear (TAN) lines which are bound to F-actin cables and couple the nucleus to retrograde actin flow during actin-dependent nuclear movement. Required for centrosome migration to the apical cell surface during early ciliogenesis.

Subcellular Location:
Nucleus outer membrane. Sarcoplasmic reticulum membrane. Cell membrane. Cytoplasm > cytoskeleton. Mitochondrion. Nucleus > nucleoplasm. Different isoform patterns are found in the different compartments of the cell. The isoforms having the C-terminal transmembrane span can be found in several organellar membranes like the nuclear envelope, the sarcoplasmic reticulum of myoblasts, or the lamellipodia and focal adhesions at the cell membrane. The largest part of the outer nuclear membrane-associated protein is cytoplasmic, while its C-terminal part is associated with the nuclear envelope, most probably the outer nuclear membrane. Remains associated with the nuclear envelope during its breakdown in mitotic cells. Shorter solubles isoforms can be found in the cytoplasm and within the nucleus.

Tissue Specificity:
Widely expressed, with higher level in kidney, adult and fetal liver, stomach and placenta. Weakly expressed in skeletal muscle and brain. Isoform 5 is highly expressed in pancreas, skeletal muscle and heart.

DISEASE:
Defects in SYNE2 are the cause of Emery-Dreifuss muscular dystrophy type 5 (EDMD5) [MIM:612999]. 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 1 KASH domain. Contains 9 spectrin repeats.

SWISS:
Q8WXH0

Gene ID:
23224

Database links:

Entrez Gene: 23224 Human

Omim: 608442 Human

SwissProt: Q8WXH0 Human

Unigene: 525392 Human

Unigene: 729020 Human



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