Plotegher 2017 Trends Mol Med: Difference between revisions
No edit summary |
No edit summary ย |
||
Line 6: | Line 6: | ||
|journal=Trends Mol Med | |journal=Trends Mol Med | ||
|abstract=Lysosomal storage disorders (LSDs) are rare inherited debilitating and often fatal disorders. Caused by mutations affecting lysosomal proteins, LSDs are characterized by the accumulation of undegraded material in lysosomes and by lysosomal dysfunction. Although LSDs are multisystemic diseases, the majority display neurologic symptoms and neurodegeneration. Only recently has a role emerged for mitochondrial dysfunction in the pathophysiology of LSDs, suggesting an impact of lysosomal dysfunction on mitochondria. Moreover, mitochondrial damage may also cause lysosomal dysfunction, further supporting the activity of common signaling pathways and crosstalk between the two organelles. In this review we explore the mechanisms linking lysosomal and mitochondrial dysfunction to assess whether specific mitochondrial pathways represent a new therapeutic frontier in the management of LSDs. | |abstract=Lysosomal storage disorders (LSDs) are rare inherited debilitating and often fatal disorders. Caused by mutations affecting lysosomal proteins, LSDs are characterized by the accumulation of undegraded material in lysosomes and by lysosomal dysfunction. Although LSDs are multisystemic diseases, the majority display neurologic symptoms and neurodegeneration. Only recently has a role emerged for mitochondrial dysfunction in the pathophysiology of LSDs, suggesting an impact of lysosomal dysfunction on mitochondria. Moreover, mitochondrial damage may also cause lysosomal dysfunction, further supporting the activity of common signaling pathways and crosstalk between the two organelles. In this review we explore the mechanisms linking lysosomal and mitochondrial dysfunction to assess whether specific mitochondrial pathways represent a new therapeutic frontier in the management of LSDs. | ||
|keywords=Review | |||
|editor=[[Kandolf G]] | |editor=[[Kandolf G]] | ||
|mipnetlab=UK London Duchen MR | |mipnetlab=UK London Duchen MR | ||
Line 14: | Line 15: | ||
|injuries=Oxidative stress;RONS | |injuries=Oxidative stress;RONS | ||
|preparations=Intact cells, Isolated mitochondria | |preparations=Intact cells, Isolated mitochondria | ||
}} | }} |
Latest revision as of 13:45, 28 November 2017
Plotegher N, Duchen MR (2017) Mitochondrial dysfunction and neurodegeneration in lysosomal storage disorders. Trends Mol Med 23:116-34. |
Plotegher N, Duchen MR (2017) Trends Mol Med
Abstract: Lysosomal storage disorders (LSDs) are rare inherited debilitating and often fatal disorders. Caused by mutations affecting lysosomal proteins, LSDs are characterized by the accumulation of undegraded material in lysosomes and by lysosomal dysfunction. Although LSDs are multisystemic diseases, the majority display neurologic symptoms and neurodegeneration. Only recently has a role emerged for mitochondrial dysfunction in the pathophysiology of LSDs, suggesting an impact of lysosomal dysfunction on mitochondria. Moreover, mitochondrial damage may also cause lysosomal dysfunction, further supporting the activity of common signaling pathways and crosstalk between the two organelles. In this review we explore the mechanisms linking lysosomal and mitochondrial dysfunction to assess whether specific mitochondrial pathways represent a new therapeutic frontier in the management of LSDs. โข Keywords: Review โข Bioblast editor: Kandolf G โข O2k-Network Lab: UK London Duchen MR
Labels: MiParea: Respiration
Pathology: Inherited, Neurodegenerative
Stress:Oxidative stress;RONS
Preparation: Intact cells, Isolated mitochondria