Search Results - "Porras, Gracia"
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Tideglusib, a Non-ATP Competitive Inhibitor of GSK-3β as a Drug Candidate for the Treatment of Amyotrophic Lateral Sclerosis
Published in International journal of molecular sciences (20-08-2021)“…Amyotrophic Lateral Sclerosis (ALS) is the most common degenerative motor neuron disease in adults. About 97% of ALS patients present TDP-43 aggregates with…”
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Functional Characterization of a Familial ALS-Associated Missense TBK1 (p-Arg573Gly) Mutation in Patient-Derived Lymphoblasts
Published in International journal of molecular sciences (02-02-2023)“…The goal of this work was to elucidate the pathogenic mechanism of an ALS-associated missense mutation, p.Arg573Gly (R573G), in the gene. In particular, we…”
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Discovery of Mitophagy Inhibitors with Therapeutic Potential in Different Familial Amyotrophic Lateral Sclerosis Mutations
Published in International journal of molecular sciences (21-10-2022)“…Mitophagy is the selective degradation of mitochondria by autophagy. It promotes the turnover of mitochondria and prevents the accumulation of dysfunctional…”
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Molecular Alterations in Sporadic and SOD1 -ALS Immortalized Lymphocytes: Towards a Personalized Therapy
Published in International journal of molecular sciences (16-03-2021)“…Amyotrophic lateral sclerosis (ALS) is a fatal neurological condition where motor neurons (MNs) degenerate. Most of the ALS cases are sporadic (sALS), whereas…”
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CdSe Quantum Dots in Human Models Derived from ALS Patients: Characterization, Nuclear Penetration Studies and Multiplexing
Published in Nanomaterials (Basel, Switzerland) (09-03-2021)“…CdSe quantum dots (QDs) are valuable tools for deciphering molecular mechanisms in cells. Their conjugation with antibodies offers a unique staining source…”
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Regulation of the mhp Cluster Responsible for 3-(3-Hydroxyphenyl)propionic Acid Degradation in Escherichia coli
Published in The Journal of biological chemistry (25-07-2003)“…The mhp gene cluster from Escherichia coli constitutes a model system to study bacterial degradation of 3-(3-hydroxyphenyl)propionic acid (3HPP). In this work…”
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Leukocyte-endothelial cell interaction is enhanced in podocalyxin-deficient mice
Published in The international journal of biochemistry & cell biology (01-06-2018)“…The highly sialoglycosylated extracellular domain of podocalyxin (Podxl) is a constituent of the endothelial glycocalyx of most blood vessels but it is unknown…”
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Recapitulation of Pathological TDP-43 Features in Immortalized Lymphocytes from Sporadic ALS Patients
Published in Molecular neurobiology (01-04-2019)“…Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder of still unknown etiology that results in loss of motoneurons, paralysis,…”
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Multitarget Hybrid Fasudil Derivatives as a New Approach to the Potential Treatment of Amyotrophic Lateral Sclerosis
Published in Journal of medicinal chemistry (10-02-2022)“…Hybrid compounds containing structural fragments of the Rho kinase inhibitor fasudil and the NRF2 inducers caffeic and ferulic acids were designed with the aid…”
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Therapeutic potential of novel Cell Division Cycle Kinase 7 inhibitors on TDP‐43‐related pathogenesis such as Frontotemporal Lobar Degeneration (FTLD) and amyotrophic lateral sclerosis (ALS)
Published in Journal of neurochemistry (01-02-2021)“…TDP‐43 has been identified as the major component of protein aggregates found in affected neurons in FTLD‐TDP and amyotrophic lateral sclerosis (ALS) patients…”
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Loss of endothelial barrier integrity in mice with conditional ablation of podocalyxin (Podxl) in endothelial cells
Published in European journal of cell biology (01-08-2016)“…Podocalyxin (Podxl) has an essential role in the development and function of the kidney glomerular filtration barrier. It is also expressed by vascular…”
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Multicentre physiological reference values for some urinary component-to-creatinine (creatininium) concentration ratios
Published in Clinical chemistry and laboratory medicine (01-01-2005)“…Nine clinical laboratories in different regions of Spain have shared the search for reference individuals and the production of reference values for urinary…”
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Proteome Aggregation in Cells Derived from Amyotrophic Lateral Sclerosis Patients for Personalized Drug Evaluation
Published in ACS chemical neuroscience (06-11-2024)“…Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that currently lacks effective therapy. Given the heterogeneity of clinical and…”
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Generation of mice with conditional ablation of the Cd40lg gene: new insights on the role of CD40L
Published in Transgenic research (01-02-2014)“…CD40 ligand (CD40L) acts as an immune modulator in activated T cells, and mutations in the extracellular domain are associated to X-linked hyper IgM syndrome…”
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Analytical performance evaluation of a particle-enhanced turbidimetric cystatin C assay on the Roche COBAS 6000 analyzer
Published in Clinical biochemistry (01-07-2010)“…Cystatin C is a low molecular protein that has been proposed to estimate the glomerular filtration rate. Here we investigated the performance of the Roche…”
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