Fibroblasts and hiPS‐Derived Astrocytes From CoPAN Patients Showed Different Levels of Iron Overload Correlated With Senescent Phenotype
Sign Up to like & getrecommendations! Published in 2025 at "Glia"
DOI: 10.1002/glia.70017
Abstract: COASY protein‐associated neurodegeneration (CoPAN) is a rare autosomal recessive disorder within the Neurodegeneration with Brain Iron Accumulation spectrum, resulting from mutations in COASY. This gene encodes the bifunctional enzyme essential for the final steps of… read more here.
Keywords: iron; hips derived; derived astrocytes; accumulation ... See more keywords
PKAN hiPS-Derived Astrocytes Show Impairment of Endosomal Trafficking: A Potential Mechanism Underlying Iron Accumulation
Sign Up to like & getrecommendations! Published in 2022 at "Frontiers in Cellular Neuroscience"
DOI: 10.3389/fncel.2022.878103
Abstract: PKAN disease is caused by mutations in the PANK2 gene, encoding the mitochondrial enzyme pantothenate kinase 2, catalyzing the first and key reaction in Coenzyme A (CoA) biosynthetic process. This disorder is characterized by progressive… read more here.
Keywords: derived astrocytes; hips derived; endosomal trafficking; iron ... See more keywords
PPAR Gamma Agonist Leriglitazone Recovers Alterations Due to Pank2-Deficiency in hiPS-Derived Astrocytes
Sign Up to like & getrecommendations! Published in 2023 at "Pharmaceutics"
DOI: 10.3390/pharmaceutics15010202
Abstract: The novel brain-penetrant peroxisome proliferator-activated receptor gamma agonist leriglitazone, previously validated for other rare neurodegenerative diseases, is a small molecule that acts as a regulator of mitochondrial function and exerts neuroprotective, anti-oxidative and anti-inflammatory effects.… read more here.
Keywords: derived astrocytes; hips derived; gamma agonist; agonist leriglitazone ... See more keywords