英文名称
Plectin Recombinant Rabbit mAb
英文别名
EBS1; EBS5A; EBS5B; EBS5C; EBS5D; EBSMD; EBSND; EBSO; EBSOG; EBSPA; HD1; LGMD2Q; LGMDR17; PCN; PLEC1; PLEC1b; PLTN; PLEC_HUMAN; PLEC; Hemidesmosomal protein 1 (HD1); Plectin-1; PLEC_MOUSE; Plectin-6; PLEC_RAT; plectin; plectin 1, intermediate filament binding protein, 500kD; epidermolysis bullosa simplex 1 (Ogna); plectin 1, intermediate filament binding protein 500kDa
免疫原
A synthesized peptide derived from human Plectin: 2-61
纯化方法
affinity purified by Protein A
储存液
10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.
保存条件
Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍
网蛋白(plectin)在介导、维持、稳定细胞骨架中起到关键作用,在微管、微丝和中间丝之间的连接中具有结构性功能。
背景资料
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 myofiber integrity.
亚基
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).
亚细胞定位
Cytoplasm, cytoskeleton. Cell junction, hemidesmosome.
组织特异性
Widely expressed with highest levels in muscle, heart, placenta and spinal cord.
翻译后修饰
Phosphorylated by CDK1; regulates dissociation from intermediate filaments during mitosis. Phosphorylated upon DNA damage, probably by ATM or ATR.
疾病
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.
相似性
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.
功能
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.