Seehafer Sabrina S, Pearce David A
Center for Aging and Developmental Biology, Aab Institute of Biomedical Sciences, University of Rochester, School of Medicine and Dentistry, Rochester, NY 14642, USA.
Neurobiol Aging. 2006 Apr;27(4):576-88. doi: 10.1016/j.neurobiolaging.2005.12.006. Epub 2006 Feb 7.
Accumulation of intracellular autofluorescent material or "aging pigment" has been characterized as a normal aging event. Certain diseases also exhibit a similar accumulation of intracellular autofluorescent material. However, autofluorescent storage material associated with aging and disease has distinct characteristics. Lipofuscin is a common term for aging pigments, whereas ceroid is used to describe pathologically derived storage material, for example, in the neuronal ceroid lipofuscinoses (NCLs). NCLs are a family of neurodegenerative diseases that are characterized by an accumulation of autofluorescent storage material (ceroid) in the lysosome, which has been termed "lipofuscin-like". There have been many studies that describe this autofluorescent storage material, but what is it? Is this accumulation lipofuscin or ceroid? In this review we will try to answer the following questions: (1) What is lipofuscin and ceroid? (2) What contributes to the accumulation of this storage material in one or the other? (3) Does this material have an effect on cellular function? Studying parallels between the accumulation of lipofuscin and ceroid may provide insight into the biological relevance of these phenomena.
细胞内自发荧光物质或“老年色素”的积累被认为是一种正常的衰老现象。某些疾病也表现出类似的细胞内自发荧光物质积累。然而,与衰老和疾病相关的自发荧光储存物质具有不同的特征。脂褐素是老年色素的常用术语,而蜡样质则用于描述病理来源的储存物质,例如在神经元蜡样质脂褐质沉积症(NCLs)中。NCLs是一类神经退行性疾病,其特征是溶酶体中积累自发荧光储存物质(蜡样质),这种物质被称为“类脂褐素”。已经有许多研究描述了这种自发荧光储存物质,但它究竟是什么?这种积累是脂褐素还是蜡样质?在这篇综述中,我们将尝试回答以下问题:(1)什么是脂褐素和蜡样质?(2)是什么导致这种储存物质在其中一种或另一种中积累?(3)这种物质对细胞功能有影响吗?研究脂褐素和蜡样质积累之间的相似之处可能有助于深入了解这些现象的生物学意义。