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Plectin Rabbit pAb (bs-3640R)  
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產品編號 bs-3640R
英文名稱 Plectin Rabbit pAb
中文名稱 網蛋白抗體
別    名 EBS1; EBSO; HD1; Hemidesmosomal protein 1; PCN; PLEC1; PLEC1b; Plectin 1 plectin 1 intermediate filament binding protein 500kDa; Plectin 6; PLTN.  
研究領域 細胞生物  免疫學  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Cow,Dog,Horse)
產品應用 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 515 kDa
檢測分子量
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 Plectin: 4451-4550/4684 
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
產品介紹 Plectin interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the crosslinking and stabilization of cytoskeletal intermediate filaments network, but also in the regulation of their dynamics.

Function:
Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofibers integrity.

Subunit:
Homodimer or homotetramer. Interacts (via actin-binding domain) with Nesprin-3. Interacts (via CH 1 domain) with VIM (via rod region). Interacts with FER (By similarity). Interacts with COL17A1 (via N-terminus). Interacts (via N-terminus) with DST isoform 1 (via N-terminus).

Subcellular Location:
Cytoplasm, cytoskeleton. Cell junction, hemidesmosome.

Tissue Specificity:
Widely expressed with highest levels in muscle, heart, placenta and spinal cord.

Post-translational modifications:
Phosphorylated by CDK1; regulates dissociation from intermediate filaments during mitosis. Phosphorylated upon DNA damage, probably by ATM or ATR.

DISEASE:
Defects in PLEC are the cause of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:612138]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.

Similarity:
Belongs to the plakin or cytolinker family.
Contains 1 actin-binding domain.
Contains 2 CH (calponin-homology) domains.
Contains 33 plectin repeats.
Contains 4 spectrin repeats.

SWISS:
Q15149

Gene ID:
5339

Database links:

Entrez Gene: 5339 Human

Entrez Gene: 18810 Mouse

Entrez Gene: 64204 Rat

Omim: 601282 Human

SwissProt: Q15149 Human

SwissProt: Q9QXS1 Mouse

SwissProt: P30427 Rat

Unigene: 434248 Human

Unigene: 234912 Mouse

Unigene: 1085 Rat



網蛋白(plectin)在介導、維持、穩(wěn)定細胞骨架中起到關鍵作用,在微管、微絲和中間絲之間的連接中具有結構性功能。
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