Publications by authors named "S Gonzalez-Granero"
Article Synopsis
- - Germinal matrix hemorrhage (GMH) is a serious condition in preterm infants linked to blood vessel rupture in the brain, but the reasons behind this vulnerability are not well understood.
- - Research shows that microglia (immune cells in the brain) interact with developing blood vessels differently as the brain matures and their absence can hinder blood vessel growth in key brain areas.
- - In preterm infants with GMH, immune cells show abnormal activation, leading to inflammation and factors that compromise blood vessel integrity, suggesting that the immune response plays a crucial role in the development of GMH.
Article Synopsis
- Mutations in the () gene cause a serious metabolic condition leading to symptoms like seizures, psychomotor delays, and severe lactic acidosis due to lipoylation defects in key enzyme complexes.
- Research using patient-derived fibroblasts and neurons revealed reduced levels of essential proteins and enzyme activities, resulting in cellular energy failure and damage.
- A combination of antioxidants and mitochondrial boosters was found to improve cell function and protein lipoylation, suggesting a potential treatment strategy for this genetic disorder, primarily through SIRT3 activation.
Neurogenesis and gliogenesis continue in the Ventricular-Subventricular Zone (V-SVZ) of the adult rodent brain. B1 cells are astroglial cells derived from radial glia that function as primary progenitors or neural stem cells (NSCs) in the V-SVZ. B1 cells, which have a small apical contact with the ventricle, decline in numbers during early postnatal life, yet neurogenesis continues into adulthood.
View Article and Find Full Text PDFAntioxidants Prevent Iron Accumulation and Lipid Peroxidation, but Do Not Correct Autophagy Dysfunction or Mitochondrial Bioenergetics in Cellular Models of BPAN.
Int J Mol Sci
September 2023
Neurodegeneration with brain iron accumulation (NBIA) is a group of rare neurogenetic disorders frequently associated with iron accumulation in the basal nuclei of the brain. Among NBIA subtypes, β-propeller protein-associated neurodegeneration (BPAN) is associated with mutations in the autophagy gene . The aim of this study was to demonstrate the autophagic defects and secondary pathological consequences in cellular models derived from two patients harboring mutations.
View Article and Find Full Text PDFThe molecular mechanisms and evolutionary changes accompanying synapse development are still poorly understood. Here we generate a cross-species proteomic map of synapse development in the human, macaque and mouse neocortex. By tracking the changes of more than 1,000 postsynaptic density (PSD) proteins from midgestation to young adulthood, we find that PSD maturation in humans separates into three major phases that are dominated by distinct pathways.
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