Liu Aihua, Lin Yanhua, Terry Ryan, Nelson Kelly, Bernstein Paul S
Department of Ophthalmology and Visual Sciences, 65 Mario Capecchi Drive, Moran Eye Center, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
Clin Lipidol. 2011;6(5):593-613. doi: 10.2217/clp.11.41.
Macular degeneration is a progressive, bilateral eye disorder that damages the macula of the human eye. The most common form of macular degeneration is age-related macular degeneration (AMD), which is the leading cause of irreversible blindness in people older than 50 years in developed countries. Autosomal dominant Stargardt disease-3 (STGD3) is an inherited macular dystrophy that has clinical features similar to dry AMD, but occurs at a much earlier age. It is caused by a mutation in the elongation of very-long-chain fatty acids-like 4 () gene, which is responsible for encoding the elongase enzyme that converts shorter chain fatty acids into C-C very long-chain polyunsaturated fatty acids (VLCPUFAs, total number of carbons ≥24). Diets rich in long-chain polyunsaturated fatty acids (LCPUFAs) have inverse associations with the progression of AMD and STGD3, and a deficiency in retinal LCPUFAs and VLCPUFAs has been detected in AMD retinas and STGD3 animal models. This article systematically summarizes the roles of LCPUFAs and VLCPUFAs in AMD and STGD3, and discusses future research directions.
黄斑变性是一种进行性双侧眼部疾病，会损害人眼的黄斑。最常见的黄斑变性形式是年龄相关性黄斑变性（AMD），它是发达国家50岁以上人群不可逆失明的主要原因。常染色体显性遗传性Stargardt病3型（STGD3）是一种遗传性黄斑营养不良，其临床特征与干性AMD相似，但发病年龄要早得多。它是由超长链脂肪酸样4（）基因的突变引起的，该基因负责编码将短链脂肪酸转化为碳碳超长链多不饱和脂肪酸（VLCPUFAs，碳总数≥24）的延长酶。富含长链多不饱和脂肪酸（LCPUFAs）的饮食与AMD和STGD3的进展呈负相关，并且在AMD视网膜和STGD3动物模型中已检测到视网膜LCPUFAs和VLCPUFAs缺乏。本文系统总结了LCPUFAs和VLCPUFAs在AMD和STGD3中的作用，并讨论了未来的研究方向。
