半糖粒是细胞与细胞外基质之间的黏附结构。它们在将中间纤维锚定在细胞外基膜上发挥作用。在结构上,半脂质体有两种形式:I型和II型。I型半脂质体发育于分层上皮,如表皮。其主要成分包括细胞内连接蛋白果胶和BPAG1,粘附受体整合素α6β4和胶原型BP180/XVII。II型半脂质体作为I型半脂质体的简化形式出现在血管、雪旺细胞和消化道上皮中,仅由果胶和整合素α6β4组成。半糖体粘附受体通常与基底膜上的层粘连蛋白5相关。此外,层粘连蛋白5(其中层粘连蛋白2是一个亚基)通过VII型胶原蛋白与真皮层中的胶原纤维相连。半脂糖体相关蛋白的遗传缺失导致各种形式的大疱性表皮松解症,突出了它们在促进表皮和基底膜之间粘附的重要性。
角蛋白8通常与角蛋白18二聚,在简单的单层上皮细胞中形成中间纤维。角蛋白8在维持细胞结构完整性、信号转导和细胞分化中发挥作用。该基因突变导致隐源性肝硬化。与角蛋白19一起,有助于在横纹肌的肋部连接收缩器官和肌营养不良蛋白。
Cosmo Bio抗体,Anti Keratin, Type II Cytoskeletal 8 (Keratin-8) mAb (Clone RL273),CAC-NU-01-KE8
Application: IF, WB, IHC(f)
Clonality: Monoclonal
Host: Mouse
Purification: Supernatant
Reactivity: Rabbit, Human
Hemidesmosomes are adhesive structures between cells and the extracellular matrix. They play a role in anchoring intermediate fibers to the extracellular basement membrane. Structurally, hemidesmosomes occur in two forms: Type I and Type II. Type I hemidesmosomes develop in stratified epithelia such as the epidermis. Its main components include the intracellular linker proteins Plectin and BPAG1, the adhesion receptor integrin α6β4 and collagen type BP180/XVII. Type II hemidesmosomes occur in blood vessels, Schwann cells, and digestive tract epithelia as a simplified form of Type I hemidesmosomes, consisting only plectin and integrin α6β4. The hemidesmosomal adhesion receptor is normally associated with Laminin 5 in the basement membrane. Furthermore, Laminin 5 (of which Laminin gamma 2 is a subunit) is linked to collagen fibers in the dermis via type VII collagen. Genetic deletion of hemidesmosome-related proteins causes various forms of epidermolysis bullosa, highlighting their importance in promoting adhesion between the epidermis and the basement membrane.
Keratin 8 typically dimerizes with Keratin 18 to form an intermediate filament in simple single-layered epithelial cells. Keratin 8 plays a role in maintaining cellular structural integrity and also functions in signal transduction and cellular differentiation. Mutations in this gene cause cryptogenic cirrhosis.Together with Keratin 19, helps to link the contractile apparatus to dystrophin at the costameres of striated muscle.