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。
BPAG1是一种230 kda的定位于分层上皮半脂质体的细胞质蛋白，是慢性自身免疫性皮肤病大疱性类天疱疮(BP)的主要抗原蛋白之一。该抗BPAG1单克隆抗体(克隆279)是针对牛BPAG1培养的，可用于包括人和小鼠在内的多种动物的western blotting和免疫荧光染色(IF)。
Cosmo Bio抗体,Anti Dystonin (BPAG1/BP230) mAb (Clone 279),CAC-NU-01-BP1
Application: IF, WB, IHC(f)
Clonality: Monoclonal
Host: Mouse
Purification: Supernatant
Reactivity: Porcine, Rabbit, Bovine, Rat, 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.
BPAG1 is a 230-kDa cytoplasmic protein localized to hemidesmosomes of stratified epithelia and is one of the major antigenic proteins of bullous pemphigoid (BP), a chronic autoimmune skin disease. This anti-BPAG1 monoclonal antibody (clone 279) was raised against bovine BPAG1 and can be used in western blotting and immunofluorescent staining (IF) in multiple animal species, including humans and mice.