Mitochondrial dysfunction in lung ageing and disease

SM Cloonan, K Kim, P Esteves, T Trian… - European Respiratory …, 2020 - Eur Respiratory Soc
Mitochondrial biology has seen a surge in popularity in the past 5 years, with the emergence
of numerous new avenues of exciting mitochondria-related research including …

A change of heart: new roles for cilia in cardiac development and disease

L Djenoune, K Berg, M Brueckner… - Nature Reviews Cardiology, 2022 - nature.com
Although cardiac abnormalities have been observed in a growing class of human disorders
caused by defective primary cilia, the function of cilia in the heart remains an underexplored …

ORP1L mediated PI (4) P signaling at ER-lysosome-mitochondrion three-way contact contributes to mitochondrial division

M Boutry, PK Kim - Nature Communications, 2021 - nature.com
Mitochondrial division is not an autonomous event but involves multiple organelles,
including the endoplasmic reticulum (ER) and lysosomes. Whereas the ER drives the …

Mitophagy in cardiovascular diseases

G Morciano, S Patergnani, M Bonora, G Pedriali… - Journal of clinical …, 2020 - mdpi.com
Cardiovascular diseases are one of the leading causes of death. Increasing evidence has
shown that pharmacological or genetic targeting of mitochondria can ameliorate each stage …

PGC-1α in disease: recent renal insights into a versatile metabolic regulator

JM Chambers, RA Wingert - Cells, 2020 - mdpi.com
Peroxisome proliferator-activated receptor gamma co-activator 1 alpha (PGC-1α) is perhaps
best known as a master regulator of mitochondrial biogenesis and function. However, by …

[HTML][HTML] Mitochondrial dysfunction compromises ciliary homeostasis in astrocytes

O Ignatenko, S Malinen, S Rybas, H Vihinen… - Journal of Cell …, 2022 - rupress.org
Astrocytes, often considered as secondary responders to neurodegeneration, are emerging
as primary drivers of brain disease. Here we show that mitochondrial DNA depletion in …

Primary cilia formation requires the Leigh syndrome–associated mitochondrial protein NDUFAF2

CH Lo, Z Liu, S Chen, F Lin, AR Berneshawi… - The Journal of Clinical …, 2024 - jci.org
Mitochondria-related neurodegenerative diseases have been implicated in the disruption of
primary cilia function. Mutation in an intrinsic mitochondrial complex I component NDUFAF2 …

Understanding laterality disorders and the left-right organizer: Insights from zebrafish

K Forrest, AC Barricella, SA Pohar… - Frontiers in Cell and …, 2022 - frontiersin.org
Vital internal organs display a left-right (LR) asymmetric arrangement that is established
during embryonic development. Disruption of this LR asymmetry—or laterality—can result in …

Crosstalk between cilia and autophagy: implication for human diseases

M Morleo, HLA Vieira, P Pennekamp, A Palma… - Autophagy, 2023 - Taylor & Francis
Macroautophagy/autophagy is a self-degradative process necessary for cells to maintain
their energy balance during development and in response to nutrient deprivation …

CLPP depletion causes diplotene arrest; underlying testis mitochondrial dysfunction occurs with accumulation of perrault proteins ERAL1, PEO1, and HARS2

J Key, S Gispert, L Koornneef, E Sleddens-Linkels… - Cells, 2022 - mdpi.com
Human Perrault syndrome (PRLTS) is autosomal, recessively inherited, and characterized
by ovarian insufficiency with hearing loss. Among the genetic causes are mutations of matrix …