Results 31 to 40 of about 18,329 (223)
Cerebral Iron Deposition in Neurodegeneration
Biomolecules, 2022 Disruption of cerebral iron regulation appears to have a role in aging and in the pathogenesis of various neurodegenerative disorders. Possible unfavorable impacts of iron accumulation include reactive oxygen species generation, induction of ferroptosis,
Petr Dusek +4 more
doaj +1 more source
Novel PANK2 mutation identified in patient with pantothenate kinase-associated neurodegeneration [PDF]
Srpski Arhiv za Celokupno Lekarstvo, 2020 Introduction. Pantothenate kinase-associated neurodegeneration (PKAN) is a rare, recessively inherited disorder caused by mutations in the pantothenate kinase 2 (PANK2) gene on chromosome 20p13.
Svetel Marina +4 more
doaj +1 more source
The neuropathology of neurodegeneration with brain iron accumulation. [PDF]
Int Rev Neurobiol, 2013 Neuropathology plays a key role in characterizing the pathogenesis of neurodegenerative diseases including forms of neurodegeneration with brain iron accumulation (NBIA). Despite important differences, several genetically diverse forms of NBIA nevertheless share common features in addition to iron deposition, such as the presence of neuroaxonal ...
Kruer MC.
europepmc +4 more sources
Neurobiology of Disease, 2022 Dopamine metabolism, alpha-synuclein pathology, and iron homeostasis have all been implicated as potential contributors to the unique vulnerability of substantia nigra dopaminergic neurons which preferentially decline in Parkinson's disease and some rare
Rachel M. Wise +8 more
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Pantothenate kinase-associated neurodegeneration: Clinical aspects, diagnosis and treatments
Neurology International, 2018 Pantothenate Kinase-Associated Neurodegeneration (PKAN) is an autosomal recessive disorder characterized by a mutation in the PANK2 gene. The clinical presentation may range from only speech disorder to severe generalized dystonia, spasticity, Visual ...
Saeed Razmeh +5 more
doaj +1 more source
Iron Dyshomeostasis in Neurodegeneration with Brain Iron Accumulation (NBIA): Is It the Cause or the Effect? [PDF]
Cells: Iron is an essential metal ion implicated in several cellular processes. However, the reactive nature of iron renders this metal ion potentially dangerous for cells, and its levels need to be tightly controlled.
Agostini F, Sgalletta B, Bisaglia M.
europepmc +2 more sources
A pilot trial of deferiprone in pantothenate kinase-associated neurodegeneration patients
Neurology International, 2018 Pantothenate kinase-associated neurodegeneration (PKAN) is the most common form of neurodegeneration with brain iron accumulation, it is an autosomal recessive disease due to mutation in PANK 2 on chromosome 20, which causes the accumulation of iron in ...
Mohammad Rohani +3 more
doaj +1 more source
NeuroImage: Clinical, 2023 Introduction: Strong evidence suggests a significant role for iron accumulation in the brain in addition to the well-documented neurodegenerative aspects of Huntington’s disease (HD). The putative mechanisms by which iron is linked to the HD pathogenesis
Nadine Anniek van de Zande +7 more
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Molecular Genetics and Metabolism Reports, 2019 Beta-propeller protein-associated neurodegeneration (BPAN) is a subtype of neurodegeneration with brain iron accumulation (NBIA) that presents with childhood developmental delay (especially speech delay), occasionally associated with epileptic ...
Marisa Chard +6 more
doaj +1 more source
NeuroImage, 2021 Myelin loss and iron accumulation are cardinal features of aging and various neurodegenerative diseases. Oligodendrocytes incorporate iron as a metabolic substrate for myelin synthesis and maintenance.
Nikkita Khattar +6 more
doaj +1 more source