MitophAging: mitophagy in aging and disease

D Bakula, M Scheibye-Knudsen - Frontiers in cell and developmental …, 2020 - frontiersin.org
Maintaining mitochondrial health is emerging as a keystone in aging and associated
diseases. The selective degradation of mitochondria by mitophagy is of particular …

The Bis (monoacylglycero)-phosphate Hypothesis: From Lysosomal Function to Therapeutic Avenues

UN Medoh, M Abu-Remaileh - Annual Review of Biochemistry, 2024 - annualreviews.org
Lysosomes catabolize and recycle lipids and other biological molecules to maintain cellular
homeostasis in diverse nutrient environments. Lysosomal lipid catabolism relies on the …

Mouse models of inherited retinal degeneration with photoreceptor cell loss

GB Collin, N Gogna, B Chang, N Damkham, J Pinkney… - Cells, 2020 - mdpi.com
Inherited retinal degeneration (RD) leads to the impairment or loss of vision in millions of
individuals worldwide, most frequently due to the loss of photoreceptor (PR) cells. Animal …

KCTD7 mutations impair the trafficking of lysosomal enzymes through CLN5 accumulation to cause neuronal ceroid lipofuscinoses

Y Wang, X Cao, P Liu, W Zeng, R Peng, Q Shi… - Science …, 2022 - science.org
Lysosomes are central organelles for cellular degradation and energy metabolism.
Neuronal ceroid lipofuscinoses (NCLs) are a group of the most common neurodegenerative …

Homozygous GRN mutations: new phenotypes and new insights into pathological and molecular mechanisms

V Huin, M Barbier, A Bottani, JA Lobrinus, F Clot… - Brain, 2020 - academic.oup.com
Homozygous mutations in the progranulin gene (GRN) are associated with neuronal ceroid
lipofuscinosis 11 (CLN11), a rare lysosomal-storage disorder characterized by cerebellar …

Lysosomal positioning diseases: beyond substrate storage

G Scerra, V De Pasquale, M Scarcella… - Open …, 2022 - royalsocietypublishing.org
Lysosomal storage diseases (LSDs) comprise a group of inherited monogenic disorders
characterized by lysosomal dysfunctions due to undegraded substrate accumulation. They …

Lipophagy and lipolysis status in lipid storage and lipid metabolism diseases

A Kloska, M Węsierska, M Malinowska… - International journal of …, 2020 - mdpi.com
This review discusses how lipophagy and cytosolic lipolysis degrade cellular lipids, as well
as how these pathway ys communicate, how they affect lipid metabolism and energy …

[HTML][HTML] Pathomechanisms in the neuronal ceroid lipofuscinoses

HR Nelvagal, J Lange, K Takahashi… - … et Biophysica Acta (BBA …, 2020 - Elsevier
The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative
lysosomal storage disorders (LSDs), traditionally grouped together based on shared clinical …

Genetic disruption of WASHC4 drives endo-lysosomal dysfunction and cognitive-movement impairments in mice and humans

JL Courtland, TWA Bradshaw, G Waitt, EJ Soderblom… - Elife, 2021 - elifesciences.org
Mutation of the Wiskott–Aldrich syndrome protein and SCAR homology (WASH) complex
subunit, SWIP, is implicated in human intellectual disability, but the cellular etiology of this …

[HTML][HTML] Intracellular pH–Advantages and pitfalls of surface-enhanced Raman scattering and fluorescence microscopy–A review

A Jaworska, K Malek, A Kudelski - … Acta Part A: Molecular and Biomolecular …, 2021 - Elsevier
The value of pH in various parts of protoplasm can affect nearly all aspects of cell functions.
Therefore, the determination of intracellular acid-base features is required in many areas of …