Researchers Identify a Potential Path Against Inherited Neurological Disease (IMAGE)
Caption
Researchers at the Icahn School of Medicine at Mount Sinai and NCATS identified compounds that reversed the effects of several neurodegenerative diseases called lysosomal storage disorders (LSDs) in patient cells and mice. LSDs are characterized by genetic defects that prevent the cell’s lysosomes from breaking down and recycling fats, sugars and proteins, which can accumulate in the liver and brain. This can cause a malfunction in the energy-producing mitochondria. The compounds increased the activity of TRAP1, a protein that helps the mitochondria work properly. Above, lipid-filled lysosomes (red) from Niemann-Pick disease type C1 cells are normalized when mitochondrial TRAP1 (green) is activated.
Credit
Ioannou Lab, Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai
Usage Restrictions
Image may be used only within the context of this news.
License
Original content