A5030080374- Mitochondrial Function and Pathology
- Metabolism and Genetic Disorders
- ATP Synthase and ATPases Research
- Adipose Tissue and Metabolism
- Nuclear Structure and Function
- Signaling Pathways in Disease
- Glioma Diagnosis and Treatment
- FOXO transcription factor regulation
- RNA modifications and cancer
- Telomeres, Telomerase, and Senescence
- Reproductive System and Pregnancy
- RNA and protein synthesis mechanisms
- Nanoparticle-Based Drug Delivery
- Cancer, Hypoxia, and Metabolism
- Autophagy in Disease and Therapy
- Parkinson's Disease Mechanisms and Treatments
- Neuroinflammation and Neurodegeneration Mechanisms
- MicroRNA in disease regulation
- Genetic Neurodegenerative Diseases
- Neurological diseases and metabolism
- Nanoplatforms for cancer theranostics
- Cell death mechanisms and regulation
Granta Design (United Kingdom)
2024
Institute for Research in Biomedicine
2023-2024
Altos Labs
2024
MRC Mitochondrial Biology Unit
2019-2023
Medical Research Council
2019-2023
Roland Hill (United Kingdom)
2023
The Royal Free Hospital
2023
University College London
2018-2023
University of Cambridge
2019-2022
King's College London
2020
Article8 January 2020Open Access Source DataTransparent process Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV Margherita Protasoni orcid.org/0000-0001-6557-461X Medical Research Council-Mitochondrial Biology Unit, University Cambridge, UK Search more papers by this author Rafael Pérez-Pérez orcid.org/0000-0001-7726-5873 Instituto de Investigación Hospital 12 Octubre (i+12), Madrid, Spain Teresa Lobo-Jarne...
Abstract Doxycycline has anti-tumour effects in a range of tumour systems. The aims this study were to define the role mitochondria play process and examine potential doxycycline combination with gemcitabine. We studied adenocarcinoma cell line A549, its mitochondrial DNA-less derivative A549 ρ° cultured fibroblasts. Treatment for 5 days resulted decrease mitochondrial-encoded proteins, respiration membrane potential, an increase reactive oxygen species cells fibroblasts, but fibroblasts...
Mitochondria are dynamic organelles that undergo membrane remodeling events in response to metabolic alterations generate an adequate mitochondrial network. Here, we investigated the function of fission regulator 1-like protein (MTFR1L), uncharacterized has been identified phosphoproteomic screens as a potential AMP-activated kinase (AMPK) substrate. We showed MTFR1L is outer membrane-localized modulating morphology. Loss led elongation associated with increased fusion and levels protein,...
The hereditary spastic paraplegias (HSP) are among the most genetically diverse of all Mendelian disorders. They comprise a large group neurodegenerative diseases that may be divided into 'pure HSP' in forms disease primarily entailing progressive lower-limb weakness and spasticity, 'complex when these features accompanied by other neurological (or non-neurological) clinical signs. Here, we identified biallelic variants transmembrane protein 63C (TMEM63C) gene, encoding predicted...
// Margherita Protasoni 1 , Albert M. Kroon and Jan-Willem Taanman Department of Clinical Movement Neurosciences, Institute Neurology, University College London, NW3 2PF, UK Correspondence to: Taanman, email: j.taanman@ucl.ac.uk Keywords: cancer; COL-3; doxycycline; mitochondria; tetracycline Received: April 28, 2018 Accepted: August 24, Published: September 18, ABSTRACT Tetracyclines have anticancer properties in addition to their well-known antibacterial properties. It has been proposed...
Mitochondrial dysfunction has been reported in obesity and insulin resistance, but primary genetic mitochondrial is generally not associated with these, arguing against a straightforward causal relationship. A rare exception, recently identified humans, syndrome of lower body adipose loss, leptin-deficient severe upper overgrowth, resistance caused by the p.Arg707Trp mutation MFN2 , encoding mitofusin 2. How resulting selective form leads to tissue- depot-specific growth abnormalities...
Mitochondrial oxidative phosphorylation defects underlie many neurological and neuromuscular diseases. Patients' primary dermal fibroblasts are one of the most commonly used in vitro models to study mitochondrial pathologies. However, tend rely more on glycolysis than for their energy when cultivated standard high-glucose medium, rendering it difficult expose dysfunctions. This aimed systematically investigate which extent use galactose- or fructose-based medium switches fibroblasts'...