Mitochondrial iron deficiency triggers cytosolic iron overload in PKAN hiPS-derived astrocytes.

ABSTRACT: Disease models of neurodegeneration with brain iron accumulation (NBIA) offer the possibility to explore the relationship between iron dyshomeostasis and neurodegeneration. We analyzed hiPS-derived astrocytes from PANK2-associated neurodegeneration (PKAN), an NBIA disease characterized by progressive neurodegeneration and high iron accumulation in the globus pallidus. Previous data indicated that PKAN astrocytes exhibit alterations in iron metabolism, general impairment of constitutive endosomal trafficking, mitochondrial dysfunction and acquired neurotoxic features. Here, we performed a more in-depth analysis of the interactions between endocytic vesicles and mitochondria via superresolution microscopy experiments. A significantly lower number of transferrin-enriched vesicles were in cont

SUBMITTER: Santambrogio P 

PROVIDER: S-EPMC11128011 | biostudies-literature | 2024 May

REPOSITORIES: biostudies-literature