Caughman S W, Krieg T, Timpl R, Hintner H, Katz S I
Dermatology Branch, National Cancer Institute, Bethesda, Maryland 20892.
J Invest Dermatol. 1987 Dec;89(6):547-50. doi: 10.1111/1523-1747.ep12461192.
The epidermal basement membrane zone comprises various biochemical constituents, some of which may be affected or involved in certain forms of mechanobullous diseases. Recently, nidogen and a low density form of heparan sulfate proteoglycan--two ubiquitous, noncollagenous components of basement membranes--were isolated and characterized, and affinity-purified antibodies to each component were prepared. These antibodies were used to study the distribution of both antigens in normal and diseased human skin. By immunofluorescence, both nidogen and heparan sulfate proteoglycan were linearly distributed along the basement membrane of the dermal-epidermal junction, adnexal structures, and blood vessels of normal human skin. On suction-induced blisters of normal skin, both antigens were found at the base of the blister, indicating that each was within or below the lamina lucida. By indirect immunoelectron microscopy, both antigens were ultrastructurally located within the lamina densa. The staining patterns for nidogen and heparan sulfate proteoglycan were examined in 11 patients with either junctional, dominant dystrophic, or recessive dystrophic epidermolysis bullosa, and were found to be not different from the patterns observed in normal skin.
表皮基底膜带由多种生化成分组成，其中一些成分可能会受到某些机械性大疱性疾病的影响或参与其中。最近，巢蛋白和一种低密度形式的硫酸乙酰肝素蛋白聚糖——基底膜中两种普遍存在的非胶原成分——被分离和鉴定，并制备了针对每种成分的亲和纯化抗体。这些抗体被用于研究这两种抗原在正常和患病人类皮肤中的分布。通过免疫荧光法，巢蛋白和硫酸乙酰肝素蛋白聚糖均沿正常人皮肤的真皮 - 表皮交界处、附属器结构和血管的基底膜呈线性分布。在正常皮肤的抽吸性水疱上，两种抗原均在水疱底部被发现，表明它们均位于透明层内或其下方。通过间接免疫电子显微镜检查，两种抗原在超微结构上均位于致密层内。对11例交界型、显性营养不良型或隐性营养不良型大疱性表皮松解症患者的巢蛋白和硫酸乙酰肝素蛋白聚糖的染色模式进行了检查，发现其与在正常皮肤中观察到的模式并无差异。
