Monitoring and measuring autophagy
SR Yoshii, N Mizushima - International journal of molecular sciences, 2017 - mdpi.com
Autophagy is a cytoplasmic degradation system, which is important for starvation adaptation
and cellular quality control. Recent advances in understanding autophagy highlight its …
and cellular quality control. Recent advances in understanding autophagy highlight its …
Fabry disease
DP Germain - Orphanet journal of rare diseases, 2010 - Springer
Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid
metabolism due to deficient or absent lysosomal α-galactosidase A activity. FD is pan-ethnic …
metabolism due to deficient or absent lysosomal α-galactosidase A activity. FD is pan-ethnic …
Autophagy failure in Alzheimer's disease—locating the primary defect
Autophagy, the major degradative pathway for organelles and long-lived proteins, is
essential for the survival of neurons. Mounting evidence has implicated defective autophagy …
essential for the survival of neurons. Mounting evidence has implicated defective autophagy …
Autophagy in lysosomal storage disorders
Lysosomes are ubiquitous intracellular organelles that have an acidic internal pH, and play
crucial roles in cellular clearance. Numerous functions depend on normal lysosomes …
crucial roles in cellular clearance. Numerous functions depend on normal lysosomes …
[HTML][HTML] Autophagy, lipophagy and lysosomal lipid storage disorders
Autophagy is a catabolic process with an essential function in the maintenance of cellular
and tissue homeostasis. It is primarily recognised for its role in the degradation of …
and tissue homeostasis. It is primarily recognised for its role in the degradation of …
MitophAging: mitophagy in aging and disease
Maintaining mitochondrial health is emerging as a keystone in aging and associated
diseases. The selective degradation of mitochondria by mitophagy is of particular …
diseases. The selective degradation of mitochondria by mitophagy is of particular …
Fabry disease: Mechanism and therapeutics strategies
Fabry disease is a monogenic disease characterized by a deficiency or loss of the α-
galactosidase A (GLA). The resulting impairment in lysosomal GLA enzymatic activity leads …
galactosidase A (GLA). The resulting impairment in lysosomal GLA enzymatic activity leads …
Fabry disease: molecular basis, pathophysiology, diagnostics and potential therapeutic directions
Fabry disease (FD) is a lysosomal storage disorder (LSD) characterized by the deficiency of
α-galactosidase A (α-GalA) and the consequent accumulation of toxic metabolites such as …
α-galactosidase A (α-GalA) and the consequent accumulation of toxic metabolites such as …
Accumulation of α-synuclein mediates podocyte injury in Fabry nephropathy
F Braun, A Abed, D Sellung, M Rogg… - The Journal of …, 2023 - Am Soc Clin Investig
Current therapies for Fabry disease are based on reversing intracellular accumulation of
globotriaosylceramide (Gb3) by enzyme replacement therapy (ERT) or chaperone-mediated …
globotriaosylceramide (Gb3) by enzyme replacement therapy (ERT) or chaperone-mediated …
Emerging role of autophagy in kidney function, diseases and aging
Autophagy is a highly conserved process that degrades cellular long-lived proteins and
organelles. Accumulating evidence indicates that autophagy plays a critical role in kidney …
organelles. Accumulating evidence indicates that autophagy plays a critical role in kidney …