OFD1: One gene, several disorders

N Pezzella, G Bove, R Tammaro… - American Journal of …, 2022 - Wiley Online Library
The OFD1 protein is necessary for the formation of primary cilia and left–right asymmetry
establishment but additional functions have also been ascribed to this multitask protein …

Pathophysiology of primary cilia: signaling and proteostasis regulation

E Senatore, R Iannucci, F Chiuso… - Frontiers in Cell and …, 2022 - frontiersin.org
Primary cilia are microtubule-based, non-motile sensory organelles present in most types of
growth-arrested eukaryotic cells. They are transduction hubs that receive and transmit …

Fibroblast growth factor 21 (FGF21) alleviates senescence, apoptosis, and extracellular matrix degradation in osteoarthritis via the SIRT1-mTOR signaling pathway

H Lu, C Jia, D Wu, H Jin, Z Lin, J Pan, X Li… - Cell Death & …, 2021 - nature.com
Osteoarthritis (OA) is a complex condition that involves both apoptosis and senescence and
currently cannot be cured. Fibroblast growth factor 21 (FGF21), known for its role as a potent …

NEK9 regulates primary cilia formation by acting as a selective autophagy adaptor for MYH9/myosin IIA

Y Yamamoto, H Chino, S Tsukamoto, KL Ode… - Nature …, 2021 - nature.com
Autophagy regulates primary cilia formation, but the underlying mechanism is not fully
understood. In this study, we identify NIMA-related kinase 9 (NEK9) as a GABARAPs …

Tumor treating fields suppression of ciliogenesis enhances temozolomide toxicity

P Shi, J Tian, BS Ulm, JC Mallinger… - Frontiers in …, 2022 - frontiersin.org
Tumor Treating Fields (TTFields) are low-intensity, alternating intermediate-frequency (200
kHz) electrical fields that extend survival of glioblastoma patients receiving maintenance …

Repurposing drugs in autophagy for the treatment of cancer: From bench to bedside

F Bu, J Zhang, W Shuai, J Liu, Q Sun, L Ouyang - Drug Discovery Today, 2022 - Elsevier
Highlights•Targeting autophagy has become a hot tumor-therapeutic strategy.•The drug
repurposing strategy is emerging as a good option to discover new treatments.•Many FDA …

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 …

Cilia and cancer: From molecular genetics to therapeutic strategies

P Carotenuto, SA Gradilone, B Franco - Genes, 2023 - mdpi.com
Cilia are microtubule-based organelles that project from the cell surface with motility or
sensory functions. Primary cilia work as antennae to sense and transduce extracellular …

Targeting the MITF/APAF-1 axis as salvage therapy for MAPK inhibitors in resistant melanoma

P Carotenuto, A Romano, A Barbato, P Quadrano… - Cell Reports, 2022 - cell.com
Melanoma is a deadly form of cancer characterized by remarkable therapy resistance.
Analyzing the transcriptome of MAPK inhibitor sensitive-and resistant-melanoma, we …

Proteome balance in ciliopathies: the OFD1 protein example

M Morleo, N Pezzella, B Franco - Trends in Molecular Medicine, 2023 - cell.com
The balance of protein synthesis and degradation is finely regulated and influences cellular
homeostasis and biological processes (eg, embryonic development and neuronal plasticity) …