Lipofuscin

Lipofuscin, also written lipofuscin and often referred to as age pigment, is a yellow-brown cross-linked aggregate consisting of oxidized protein ( 30-58 %) and lipid clusters ( 19-51 %), which is as well particularly in cardiac muscle, hepatocytes and neurons in the retinal pigment epithelium (RPE ), accumulates a layer of specific cells in the back of the eye with time. Lipofuscin is found primarily in the Telolysosomen ( residual bodies ), vacuoles from the fusion of lysosomes with phagosomes or organelles.

It is microscopic in the field of the nucleus visible as a dark field. With age, this compacted endogenous pigment and discolors the skin in some places brownish. Lipofuscin is formed as not usable and degradable waste product by oxidative stress in proteins (protein oxidation) and lipids ( lipid peroxidation). It is stored, inter alia, by the cardiac muscle cells. The pigment is also the subject of aging research.

When the clinical picture of neuronal ceroid lipofuscinosis an overwhelming accumulation of lipofuscin is noted in nerve cells of the brain, which is considered as a cause of nerve cell sunsets. It is a neurodegenerative disorder. In type 3 of this disease (Crohn's Batten ) but the majority of accumulation consists of a lipophilic subunit of ATP synthase.

In age-related macular degeneration ( AMD) with increasing age there is a nearly linear accumulation of lipofuscin in the RPE cells. Excessive accumulation of lipofuscin in the RPE leads to an impairment of the function and viability of the cells of the RPE, and eventually their death. The progressive loss ever larger areas of the RPE ( " geographic atrophy " ) causes the death of himself overlying photoreceptors. Recently, it was found that a new drug ( Remofuscin © ) lipofuscin could be removed from the RPE cells from monkeys and humans.