Accumulation of vitamin A-derived lipofuscin fluorophores in the retinal pigment epithelium (RPE) is a pathologic feature of recessive Stargardt macular dystrophy, a blinding disease caused by dysfunction or loss of the ABCA4 transporter in rods and cones. Age-related macular degeneration, a prevalent blinding disease of the elderly, is strongly associated with mutations in the genes for comple...

Polyclonal and monoclonal antibodies to human rhodopsin were used to identify and localize this principal glycoprotein of the photoreceptor outer segment discs on thin sections of human and monkey retinal pigment epithelium (RPE) and on immunoblots of RPE subcellular fractions following gel electrophoresis. Antiopsin was visualized with protein A-gold labeling by electron microscopy or peroxida...

The life history of melanin and lipofuscin granules of human retinal pigment epithelium (RPE) was studied in 30 human eyes spanning nine decades of life. Autofluorescent granules in the cytoplasm of eye over 30 years of age were shown, ultrastructurally and through lipid solvent extraction, to be lipofuscin granules. Sparse small fluorescent granules in infant eyes were secondary lysosomes cont...

Lipofuscin accumulation has been observed in a number of neurodegenerative diseases. We recently found that autofluorescent particles also occur in the aged human optic nerve. In this study we sought to determine the nature of these particles and their correlation with aging, age-related macular degeneration (AMD) and primary open angle glaucoma (POAG). Groups of eight optic nerves from patient...

The secretory acinar cells of parotid glands from rats of varying ages have been examined by electron microscopy to determine what age-related changes occur in these cells. The most prominent change noted in these cells is the progressive increase in the amount of lipofuscin granules with age. Lipofuscin granules are membrane-bound structures consisting of lipids, other subcomponents, and a mat...

PURPOSE Current standards and guidelines aimed at preventing retinal phototoxicity during intentional exposures do not specifically evaluate the contribution of endogenous photosensitizers. However, certain retinal diseases are characterized by abnormal accumulations of potential photosensitizers such as lipofuscin bisretinoids in the retinal pigment epithelium (RPE). We sought to determine the...

In the hard clam Eurhomalea exalbida, autofluorescent granules were detected in high concentrations in the connective tissue around the intestine and in other tissues. Autofluorescence combined with Sudan black B and PAS positive reactions suggested that these granules were lipofuscin-like. The concentration of this material in the connective tissue (CT) around the intestine was quantified by i...

BACKGROUND The progressive accumulation of misfolded and aggregated proteins in neurons is an accepted mechanism in aging. Overproduction of reactive oxygen species (ROS), referred to as oxidative stress, is currently believed to play a pivotal role in this process. Lipofuscin as a histological index of aging results from cross-links between oxidized proteins and lipids. Therefore, to attenuate...

Stargardt’s disease, also known as juvenile macular degeneration, occurs in approximately one in 10,000 people and results from genetic defects in the ABCA4 gene. The disease is characterized by premature accumulation of lipofuscin in the retinal pigment epithelium (RPE) of the eye and by vision loss. No cure or treatment is available. Although lipofuscin is considered a hallmark of Stargardt’s...

To determine whether portal lymphadenopathy in primary biliary cirrhosis is caused by deposition of lipofuscin pigment in sinus histiocytes and to compare primary biliary cirrhosis with other liver diseases a retrospective study on a consecutive series of 169 livers obtained at transplantation was carried out. There were grouped into eight diagnostic categories: primary biliary cirrhosis (n = 5...