The carboxyl-terminal domain of type VII collagen is present at the basement membrane in recessive dystrophic epidermolysis bullosa.
The carboxyl-terminal domain of type VII collagen is present at the basement membrane in recessive dystrophic epidermolysis bullosa.
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VII 型胶原蛋白的羧基末端结构域存在于隐性营养不良性大疱性表皮松解症的基底膜上。
DOI：
10.1111/1523-1747.ep12712167
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发表时间：
1989
期刊：
影响因子：
--
通讯作者：
Briggaman,RA
中科院分区：
文献类型：
--
作者：
Rusenko,KW;Gammon,WR;Fine,JD;Briggaman,RA
最近的研究表明，VII型胶原是锚定纤维的一种成分，提示隐性营养不良性大疱性表皮松解症中没有锚定纤维可能是由于VII型胶原的合成、分泌和沉积缺陷所致。最近的研究进一步支持了这一假设，研究表明，在大多数患者中，抗II型胶原的单抗不与基底膜发生反应。为了进一步研究，我们对12名患者的皮肤进行了电子显微镜、免疫组织学和免疫电子显微镜检查，使用了一种浓缩和纯化的针对III型胶原羧基末端结构域的单抗。虽然在电子显微镜下没有发现锚定纤维，但免疫组织学结果显示，11例患者的单抗与基底膜上的VII型胶原呈线性结合，但结合率较低。免疫电子显微镜下，致密层下可见抗VII型胶原抗体沉积减少。本研究结果表明，在12例隐性营养不良性大疱性表皮松解症患者中，有11例在基底膜区合成、分泌和沉积了VII型胶原的羧基末端结构域，提示VII型胶原的缺失可能是由于异常的VII型胶原沉积、正常的VII型胶原水平降低、VII型胶原结合成锚定纤维的缺陷，或者是VII型胶原的胶原域被破坏。
Recent studies showing that type VII collagen is a component of anchoring fibrils suggests that the absence of anchoring fibrils in recessive dystrophic epidermolysis bullosa may be due to defect in the synthesis, secretion, and deposition of type VII collagen. That hypothesis is further supported by recent studies suggesting that monoclonal antibodies to type VII collagen do not react with the basement membrane in most patients. To investigate further, we examined skin from 12 patients by electron microscopy and by immunohistology and immunoelectron microscopy using a concentrated and purified monoclonal antibody to the carboxy-terminal domain of Type VII collagen. Although anchoring fibrils were not detected by electron microscopy, the results of immunohistology showed definite, but reduced, binding of the monoclonal antibody to type VII collagen at the basement membrane in a linear pattern in 11 of 12 patients. By immunoelectron microscopy, reduced deposition of anti-type VII collagen antibody was detected beneath the lamina densa. The results of this study show that the carboxyl-terminal domain of type VII collagen is synthesized, secreted, and deposited at the basement membrane zone in 11 of 12 patients with recessive dystrophic epidermolysis bullosa and suggest that the absence of anchoring fibrils may be due either to deposition of abnormal type VII collagen, reduced levels of normal type VII collagen, defective assembly of type VII collagen into anchoring fibrils, or destruction of the collagenous domain of type VII collagen.