GENETICS AND BIOCHEMISTRY OF THE LENS IN CELL CULTURE

要使晶状体正常运作，弹性和清晰度是最重要的。 重要性。这些特征高度依赖于 晶状体囊基膜和晶状体纤维细胞晶状体蛋白 细胞膜。已知用于合成和维护它们的系统 然而，要细腻，在许多疾病中往往会被破坏， 通常会导致白内障。我们打算继续我们的研究 正常晶状体囊膜基底膜和晶状体蛋白 晶状体上皮和纤维细胞，并研究这些蛋白质是如何 受白内障的影响。我们将建立正常人和人类的细胞系 白内障晶状体上皮细胞，以及适当的小鼠模型 单层组织培养。这将为我们提供大量的 直接生化和免疫学研究用生物合成产品 不需要苛刻的提取程序。通过利用体细胞 杂交，我们将研究基底膜的遗传控制 和晶体蛋白的生物合成，并进行染色体的基因图谱 负责蛋白质及其加工酶。此外，我们 将产生正常x病态和病态x病态的杂交后代 晶状体细胞研究在晶状体中起作用的机制和控制 晶体蛋白质的生物合成。这些数据将有助于确定真实的性质 并进一步帮助建立一种生物化学 在疾病状态下看到的症状的基础。 这些研究将提供有关生物化学和遗传学的信息。 调节晶状体蛋白，特别是基底膜和 晶体蛋白。正常基底膜和结晶蛋白的控制 细胞合成是正常生长发育的一个重要方面， 以及涉及了解这种控制如何工作的研究，以及 各种晶状体蛋白的染色体定位将有助于我们的 了解各种晶状体蛋白质中发生的错误将有助于 我们对各种遗传性和获得性错误的理解 晶状体紊乱，如白内障。

For proper functioning of the lens, elasticity and clarity are of paramount importance. These characteristics are highly dependent on the integrity of the lens capsule basement membrane and the lens fiber cell crystallins and cell membranes. The system for their synthesis and maintenance is known to be delicate, however, and can often be disrupted in many diseases, generally leading to cataracts. We intend to continue our studies of the normal lens capsule basement membrane and the crystallins produced by the lens epithelium and fiber cells, and investigate how these proteins are affected by cataracts. We will establish cell lines from human normal and cataractous lens epithelium, as well as with appropriate mouse models in monolayer tissue culture. This will provide us with large quantities of biosynthetic products for direct biochemical and immunological study without the need for harsh extraction procedures. By use of somatic cell hybridization, we will investigate the genetic control of basement membrane and crystallin biosynthesis, and carry out gene mapping of the chromosomes responsible for the proteins and their processing enzymes. Furthermore, we will produce hybrids between normal x diseased, and diseased x diseased lens cells to investigate the mechanisms and controls that function in the biosynthesis of lens proteins. These data will help define the true nature of the molecules of the lens and further help to establish a biochemical basis for symptoms seen in the disease states. These studies will provide information about the biochemical and genetic regulation of lens proteins, specifically basement membrane and crystallins. The control of normal basement membrane and crystallin synthesis by cells is an important aspect of normal growth and development, and studies involved with understanding how such controls work, as well as the chromosomal localizations of the various lens proteins, will aid in our understanding of errors that occur in various lens proteins, will aid in our understanding of errors that occur in various hereditary and acquired disorders of the lens, such as cataract.