Impaired autophagic and mitochondrial functions are partially restored by ERT in Gaucher and Fabry diseases

The major cellular clearance pathway for organelle and unwanted proteins is the autophagy-lysosome pathway (ALP). Lysosomes not only house proteolytic enzymes, but also traffic organelles, sense nutrients, and repair mitochondria. Mitophagy is initiated by damaged mitochondria, which is ultimately d...

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Veröffentlicht in:	PloS one 2019-01, Vol.14 (1), p.e0210617-e0210617
Hauptverfasser:	Ivanova, Margarita M, Changsila, Erk, Iaonou, Chidima, Goker-Alpan, Ozlem
Format:	Artikel
Sprache:	eng
Schlagworte:	Abnormalities Accumulation Adolescent Adult Age Aged alpha-Galactosidase - therapeutic use Animal models Animals ATP Autophagy Autophagy - physiology Binding proteins Biology and Life Sciences Care and treatment Cell culture Cell cycle Cell death Cellular signal transduction Cellular stress response Child Development and progression Disease Diseases Energy shortages Enzyme Replacement Therapy - methods Enzyme therapy Enzymes Fabry Disease - drug therapy Fabry Disease - enzymology Fabry Disease - physiopathology Female Fibroblasts Galactosidase Gaucher Disease - drug therapy Gaucher Disease - enzymology Gaucher Disease - physiopathology Genetics Glucosylceramidase Glucosylceramidase - therapeutic use Humans Inborn errors of metabolism Inhibition Leukocytes (mononuclear) Leukocytes, Mononuclear - metabolism Localization Lysosomal enzymes Lysosomal storage diseases Lysosomes Lysosomes - metabolism Male Medicine and Health Sciences Metabolism Metabolites Middle Aged Mitochondria Mitophagy - physiology Mutation Nervous system Neurosciences Nutrients Organelles Patients Phagocytosis Physical Sciences Proteins Proteolysis Proteolytic enzymes Signal Transduction Substrates TOR protein Ubiquitin Vacuoles Young Adult
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