Lien Y H, Fu J, Rucker R B, Scheck M, Abbott U, Stern R
Department of Pathology, University of California, San Francisco.
Biochim Biophys Acta. 1990 Jun 20;1034(3):318-25. doi: 10.1016/0304-4165(90)90058-5.
Connective tissue matrix components were investigated using skin fibroblasts from normal or inbred scoliotic lines of chickens. Specifically, the fibroblasts were obtained from either an isogenic line or a backcross, derived by crossing the isogenic line with a pure line of scoliotic birds. From the backcross, both affected (35-45%) and non-affected (55-65%) progeny were produced. The affected birds had spinal curves greater than 20 degrees. Several abnormalities of connective tissue were observed when cells from scoliotic chicks were grown in culture: increased collagen extractability, decreased aggregatability of proteoglycans under associative conditions and lower than normal levels of hyaluronic acid. There was also less collagen deposited in the cell layer with proportionately increased amounts of collagen secreted into the culture media by cells from scoliotic versus normal chick fibroblasts. Values for collagen matrix stability, as estimated by extractability and net deposition, were intermediate for cells from the backcrossed, but non-affected, birds. Moreover, hyaluronidase, an enzyme that degrades hyaluronic acid, was abnormally elevated in the fibroblast cultures from scoliotic chicks. It is proposed that the increase in hyaluronidase contributes to the abnormalities observed in extracellular matrix components and may be a factor in the expression of scoliosis in susceptible birds.
利用来自正常鸡或近交脊柱侧凸品系鸡的皮肤成纤维细胞，对结缔组织基质成分进行了研究。具体而言，成纤维细胞取自同基因品系或回交后代，回交后代是通过将同基因品系与脊柱侧凸纯系鸟类杂交获得的。在回交后代中，产生了受影响（35 - 45%）和未受影响（55 - 65%）的子代。受影响的鸟类脊柱弯曲度大于20度。当培养脊柱侧凸雏鸡的细胞时，观察到结缔组织的几种异常情况：胶原蛋白可提取性增加、在缔合条件下蛋白聚糖的聚集性降低以及透明质酸水平低于正常。与正常雏鸡成纤维细胞相比，脊柱侧凸雏鸡的细胞分泌到培养基中的胶原蛋白量成比例增加，而细胞层中沉积的胶原蛋白较少。通过可提取性和净沉积估计的胶原蛋白基质稳定性值，对于回交但未受影响的鸟类的细胞来说处于中间水平。此外，在脊柱侧凸雏鸡的成纤维细胞培养物中，降解透明质酸的酶——透明质酸酶异常升高。有人提出，透明质酸酶的增加导致了在细胞外基质成分中观察到的异常情况，并且可能是易感鸟类脊柱侧凸表达的一个因素。
