[HTML][HTML] Prohibitins: a critical role in mitochondrial functions and implication in diseases

A Signorile, G Sgaramella, F Bellomo, D De Rasmo - Cells, 2019 - mdpi.com
Prohibitin 1 (PHB1) and prohibitin 2 (PHB2) are proteins that are ubiquitously expressed,
and are present in the nucleus, cytosol, and mitochondria. Depending on the cellular …

[HTML][HTML] Mechanisms of mitochondrial dysfunction in lysosomal storage disorders: a review

KM Stepien, F Roncaroli, N Turton… - Journal of Clinical …, 2020 - mdpi.com
Mitochondrial dysfunction is emerging as an important contributory factor to the
pathophysiology of lysosomal storage disorders (LSDs). The cause of mitochondrial …

Mitochondrial dysfunction in kidney tubulopathies

CA Hoogstraten, JG Hoenderop… - Annual Review of …, 2024 - annualreviews.org
Mitochondria play a key role in kidney physiology and pathology. They produce ATP to fuel
energy-demanding water and solute reabsorption processes along the nephron. Moreover …

Mitochondria, oxidative stress, cAMP signalling and apoptosis: a crossroads in lymphocytes of multiple sclerosis, a possible role of nutraceutics

A Signorile, A Ferretta, M Ruggieri, D Paolicelli… - Antioxidants, 2020 - mdpi.com
Multiple sclerosis (MS) is a complex inflammatory and neurodegenerative chronic disease
that involves the immune and central nervous systems (CNS). The pathogenesis involves …

Nlrp2 deletion ameliorates kidney damage in a mouse model of cystinosis

MN Rossi, V Matteo, F Diomedi-Camassei… - Frontiers in …, 2024 - frontiersin.org
Cystinosis is a rare autosomal recessive disorder caused by mutations in the CTNS gene
that encodes cystinosin, a ubiquitous lysosomal cystine/H+ antiporter. The hallmark of the …

Potential mechanisms of aortic medial degeneration promoted by co-exposure to microplastics and lead

X **e, K Wang, X Shen, S Wang, S Yuan, B Li… - Journal of Hazardous …, 2024 - Elsevier
Microplastics (MPs) have attracted widespread attention because they can lead to combined
toxicity by adsorbing heavy metals from the environment. Exposure to lead (Pb), a frequently …

Macrophage polarization impacts tunneling nanotube formation and intercellular organelle trafficking

S Goodman, S Naphade, M Khan, J Sharma… - Scientific reports, 2019 - nature.com
Tunneling nanotubes (TNTs) are cellular extensions enabling cytosol-to-cytosol intercellular
interaction between numerous cell types including macrophages. Previous studies of …

Molecular mechanisms and treatment options of nephropathic cystinosis

A Jamalpoor, A Othman, EN Levtchenko… - Trends in Molecular …, 2021 - cell.com
Nephropathic cystinosis is a severe, monogenic systemic disorder that presents early in life
and leads to progressive organ damage, particularly affecting the kidneys. It is caused by …

[HTML][HTML] Urine-derived epithelial cells as models for genetic kidney diseases

T Bondue, FO Arcolino, KRP Veys, OC Adebayo… - Cells, 2021 - mdpi.com
Epithelial cells exfoliated in human urine can include cells anywhere from the urinary tract
and kidneys; however, podocytes and proximal tubular epithelial cells (PTECs) are by far the …

Cell-based phenotypic drug screening identifies luteolin as candidate therapeutic for nephropathic cystinosis

E De Leo, MA Elmonem, SP Berlingerio… - Journal of the …, 2020 - journals.lww.com
Background Mutations in the gene that encodes the lysosomal cystine transporter cystinosin
cause the lysosomal storage disease cystinosis. Defective cystine transport leads to …