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Publication : Melanoregulin (MREG) modulates lysosome function in pigment epithelial cells.

First Author  Damek-Poprawa M Year  2009
Journal  J Biol Chem Volume  284
Issue  16 Pages  10877-89
PubMed ID  19240024 Mgi Jnum  J:149250
Mgi Id  MGI:3848102 Doi  10.1074/jbc.M808857200
Citation  Damek-Poprawa M, et al. (2009) Melanoregulin (MREG) modulates lysosome function in pigment epithelial cells. J Biol Chem 284(16):10877-89
abstractText  Melanoregulin (MREG), the product of the Mreg(dsu) gene, is a small highly charged protein, hypothesized to play a role in organelle biogenesis due to its effect on pigmentation in dilute, ashen, and leaden mutant mice. Here we provide evidence that MREG is required in lysosome-dependent phagosome degradation. In the Mreg(-/-) mouse, we show that loss of MREG function results in phagosome accumulation due to delayed degradation of engulfed material. Over time, the Mreg(-/-) mouse retinal pigment epithelial cells accumulate the lipofuscin component, A2E. MREG-deficient human and mouse retinal pigment epithelial cells exhibit diminished activity of the lysosomal hydrolase, cathepsin D, due to defective processing. Moreover, MREG localizes to small intracellular vesicles and associates with the endosomal phosphoinositide, phosphatidylinositol 3,5-biphosphate. Collectively, these studies suggest that MREG is required for lysosome maturation and support a role for MREG in intracellular trafficking.
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