
产品介绍 评论(0)
物种
Cynomolgus, Rhesus macaque分子别名
PCN, PLTN, Plectin-1, PLEC1Accession
Rhesus macaque :F7ABN9, Cynomolgus: XP_065375884.1表达序列
Rhesus macaque :Ala4555-Ala4862 with His Tag at the N-Terminus
Cynomolgus:Ala4394-Ala4701 with His Tag at the N-Terminus表达宿主
HEK293分子量
40-60kDa (Reducing)
纯度
>90% by SDS-PAGE标记
Unconjugated标签
His Tag性状
Lyophilized Powder缓冲体系
PBS, pH7.4, 5% trehalose
溶解方法
Reconstitute at 0.1-1 mg/ml according to the size in ultrapure water after rapid centrifugation.
储存条件
· 12 months from date of receipt, lyophilized powder stored at -20 to -80℃.
· 3 months, -20 to -80℃ under sterile conditions after reconstitution.
· 1 week, 2 to 8℃ under sterile conditions after reconstitution.
· Please avoid repeated freeze-thaw cycles.文献引用
1.Natsuga K, Nishie W, Akiyama M, Nakamura H, Shinkuma S, McMillan JR, Nagasaki A, Has C, Ouchi T, Ishiko A, Hirako Y, Owaribe K, Sawamura D, Bruckner-Tuderman L, Shimizu H. Plectin expression patterns determine two distinct subtypes of epidermolysis bullosa simplex. Hum Mutat. 2010 Mar;31(3):308-16.
2.Tu WT, Chen PC, Hou PC, Huang HY, Wang JY, Chao SC, Lee JY, McGrath JA, Natsuga K, Hsu CK. Plectin Missense Mutation p.Leu319Pro in the Pathogenesis of Autosomal Recessive Epidermolysis Bullosa Simplex. Acta Derm Venereol. 2020 Aug 18;100(15):adv00242.
Plectin is a large cytolinker protein composed of an N-terminal actin-binding domain, a central rod-shaped α-helical coiled-coil region, and a C-terminal intermediate filament-binding domain, with multiple alternatively spliced isoforms enabling diverse subcellular localization; its functional core lies in bridging microfilaments, intermediate filaments, and microtubule networks to maintain cellular mechanical stability, while also participating in hemidesmosome assembly, myofibril integrity maintenance, and signal transduction regulation. Clinically, PLEC gene mutations cause epidermolysis bullosa simplex (EBS) and its variant subtypes with muscular dystrophy, pyloric atresia, or cardiomyopathy, collectively termed "plectinopathies," with pathological mechanisms involving dermal-epidermal junction disruption, desmin aggregation in muscle fibers, and mitochondrial dysfunction, while recent therapeutic strategies such as chemical chaperone approaches have provided new directions for treatment.
电泳
1μg (R: reducing condition, N:non-reducing condition).







评论(0)