A5048016361- Mitochondrial Function and Pathology
- Cell death mechanisms and regulation
- Autophagy in Disease and Therapy
- Coenzyme Q10 studies and effects
- Lysosomal Storage Disorders Research
- Phagocytosis and Immune Regulation
- Hepatitis C virus research
- Carbohydrate Chemistry and Synthesis
- Microtubule and mitosis dynamics
- ATP Synthase and ATPases Research
- Metabolism and Genetic Disorders
- Advanced battery technologies research
- Metabolism, Diabetes, and Cancer
- Glycosylation and Glycoproteins Research
- Nanoparticle-Based Drug Delivery
- Gold and Silver Nanoparticles Synthesis and Applications
- Erythrocyte Function and Pathophysiology
- Biochemical effects in animals
- Cancer, Hypoxia, and Metabolism
- Amino Acid Enzymes and Metabolism
- Liver Disease Diagnosis and Treatment
- Metalloenzymes and iron-sulfur proteins
- Genetics and Neurodevelopmental Disorders
- Diet, Metabolism, and Disease
- Calcium signaling and nucleotide metabolism
Universidad de Granada
2025
Centro Andaluz de Biología del Desarrollo
2011-2019
Centro de Investigación Biomédica en Red
2011-2019
Universidad Pablo de Olavide
2011-2019
Instituto de Salud Carlos III
2011-2019
Instituto de Investigación de Enfermedades Raras
2018
Consejo Superior de Investigaciones Científicas
2011-2017
Junta de Andalucía
2012-2017
Centre for Biomedical Network Research on Rare Diseases
2012-2017
Unidades Centrales Científico-Técnicas
2012
For a number of years, coenzyme Q10 (CoQ10) was known for its key role in mitochondrial bioenergetics; later studies demonstrated presence other subcellular fractions and blood plasma, extensively investigated antioxidant role. These 2 functions constitute the basis supporting clinical use CoQ10. Also, at inner membrane level, CoQ10 is recognized as an obligatory cofactor function uncoupling proteins modulator transition pore. Furthermore, recent data indicate that affects expression genes...
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a mitochondrial disease most usually caused by point mutations in tRNA genes encoded mtDNA. Here, we report on how this mutation affects function primary fibroblast cultures established from 2 patients with MELAS who harbored the A3243G mutation. Both respiratory chain enzyme activities coenzyme Q(10) (CoQ) levels were significantly decreased fibroblasts. A similar decrease membrane potential was found...
Abstract Gaucher disease (GD) is caused by mutations in the GBA1 gene, which encodes lysosomal β-glucocerebrosidase. Homozygosity for L444P mutation associated with high risk of neurological manifestations are not improved enzyme replacement therapy. Alternatively, pharmacological chaperones (PCs) capable restoring correct folding and trafficking mutant represent promising alternative therapies.Here, we report on how affects mitochondrial function primary fibroblast derived from GD patients....
Efficient delivery of pharmacological chaperones for Gaucher disease to macrophages has been achieved.
One of the most prevalent types congenital myopathy is nemaline (NM), which recognized by histopathological examination muscle fibers for presence "nemaline bodies" (rods). Mutations in actin alpha 1 (ACTA1) and nebulin (NEB) genes result NM. Muscle weakness hypotonia are main clinical characteristics this disease. Unfortunately, pathogenetic mechanisms still unknown, there no cure. In previous work, we showed that filament polymerization defects patient-derived fibroblasts were associated...
MELAS (mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes) is a mitochondrial disease most usually caused by point mutations in tRNA genes encoded DNA (mtDNA). Approximately 80% of cases syndrome are associated with m.3243A > G mutation the MT-TL1 gene, which encodes tRNALeu (UUR). Currently, no effective treatments available for this chronic progressive disorder. Treatment strategies other diseases consist several drugs that diminish deleterious effects abnormal...
Camptothecin (CPT; ( S )-(+)-4-ethyl-4-hydroxy-1 H -pyrano[3',4':6,7]indolizino[1,2- b ]quinoline-3,14-(4 ,12 )-dione) is a highly cytotoxic natural alkaloid that has not yet found use as chemotherapeutic agent due to its poor water-solubility and chemical instability and, consequence, no effective administration means have been designed. In this work, camptothecin successfully loaded into iron oxide superparamagnetic nanoparticles with an average size of 14 nm. It was surface modification...
Amphiphilic glycomimetics encompassing a rigid, undistortable nortropane skeleton based on 1,6-anhydro-l-idonojirimycin and polyfluorinated antenna, when formulated as the corresponding inclusion complexes with β-cyclodextrin (βCD), have been shown to behave pharmacological chaperones (PCs) that efficiently rescue lysosomal β-glucocerebrosidase mutants associated neuronopathic variants of Gaucher disease (GD), including highly refractory L444P/L444P L444P/P415R single nucleotide polymorphs,...
Apoptotic microtubule network (AMN) is organized during apoptosis, forming a cortical structure beneath plasma membrane, which has an important role in preserving cell morphology and membrane permeability. The aim of this study was to examine the AMN maintaining integrity execution phase apoptosis. We demonstrated camptothecin-induced apoptosis H460 cells that delimits active caspase free area permits preservation cellular cortex transmembrane proteins. depolymerization apoptotic by short...
Systemic treatments for hepatocellular carcinoma (HCC) have been largelyunsuccessful.This study investigated the antitumoral activity of Amitriptyline, a tricyclic antidepressant, in hepatoma cells.Amitriptyline-induced toxicity involved early mitophagy activation that subsequently switched to apoptosis.Amitriptyline induced mitochondria dysfunction and oxidative stress HepG2 cells.Amitriptyline specifically inhibited mitochondrial complex III is associated with decreased membrane potential...