نتایج جستجو برای: neurodegeneration with brain iron accumulation

تعداد نتایج: 9480785  

Journal: :The Journal of neuroscience : the official journal of the Society for Neuroscience 2016
Jon Iker Etchegaray Emma J Elguero Jennifer A Tran Vincent Sinatra Mel B Feany Kimberly McCall

UNLABELLED The removal of apoptotic cell corpses is important for maintaining homeostasis. Previously, defects in apoptotic cell clearance have been linked to neurodegeneration. However, the mechanisms underlying this are still poorly understood. In this study, we report that the absence of the phagocytic receptor Draper in glia leads to a pronounced accumulation of apoptotic neurons in the bra...

2014
Clifford R. Jack Heather J. Wiste David S. Knopman Prashanthi Vemuri Michelle M. Mielke Stephen D. Weigand Matthew L. Senjem Jeffrey L. Gunter Val Lowe Brian E. Gregg Vernon S. Pankratz Ronald C. Petersen

OBJECTIVE To test the hypotheses predicted in a hypothetical model of Alzheimer disease (AD) biomarkers that rates of β-amyloid (Aβ) accumulation on PET imaging are not related to hippocampal neurodegeneration whereas rates of neurodegenerative brain atrophy depend on the presence of both amyloid and neurodegeneration in a population-based sample. METHODS A total of 252 cognitively normal (CN...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2008
Julio Salazar Natalia Mena Stephane Hunot Annick Prigent Daniel Alvarez-Fischer Miguel Arredondo Charles Duyckaerts Veronique Sazdovitch Lin Zhao Laura M Garrick Marco T Nuñez Michael D Garrick Rita Raisman-Vozari Etienne C Hirsch

Dopaminergic cell death in the substantia nigra (SN) is central to Parkinson's disease (PD), but the neurodegenerative mechanisms have not been completely elucidated. Iron accumulation in dopaminergic and glial cells in the SN of PD patients may contribute to the generation of oxidative stress, protein aggregation, and neuronal death. The mechanisms involved in iron accumulation also remain unc...

Journal: :Turkish Journal of Biology 2022

Neurodegeneration is the progressive loss of structure or function neurons. Amyloid beta oligomers and aggregates have been linked to neurodegeneration. While previous studies suggested that dietary α-tocopherol intake can prevent amyloid aggregation protect brain against neurotoxicity, other research, however, indicated tocotrienol forms might be used as an alternate agent this kind degenerati...

Journal: :Brain : a journal of neurology 1999
P D Griffiths B R Dobson G R Jones D T Clarke

Iron is found in high concentration in some areas of the brain, and increased iron in the substantia nigra is a feature of Parkinson's disease. The purpose of this study was to investigate the physical environment of brain iron in post-mortem tissue to provide information on the possible role of iron in neurodegeneration in Parkinson's disease. Iron has also been implicated as the cause of sign...

2012
Dario Brunetti Sabrina Dusi Michela Morbin Andrea Uggetti Fabio Moda Ilaria D'Amato Carla Giordano Giulia d'Amati Anna Cozzi Sonia Levi Susan Hayflick Valeria Tiranti

Neurodegeneration with brain iron accumulation (NBIA) comprises a group of neurodegenerative disorders characterized by high brain content of iron and presence of axonal spheroids. Mutations in the PANK2 gene, which encodes pantothenate kinase 2, underlie an autosomal recessive inborn error of coenzyme A metabolism, called pantothenate kinase-associated neurodegeneration (PKAN). PKAN is charact...

2015
Anthony R. White Katja M. Kanninen Peter J. Crouch

There is considerable evidence that abnormal biometal homeostasis is a key feature of many neurodegenerative diseases and may have an important role in the onset and progression of disorders such as Alzheimer's (AD), Parkinson's (PD), prion, and motor neuron diseases. The role of biometals in a growing list of brain disorders is supported by evidence from a wide range of sources including molec...

2012
Coro Paisán-Ruiz Abi Li Susanne A. Schneider Janice L. Holton Robert Johnson Desmond Kidd Jeremy Chataway Kailash P. Bhatia Andrew J. Lees John Hardy Tamas Revesz Henry Houlden

The 2 major types of neurodegeneration with brain iron accumulation (NBIA) are the pantothenate kinase type 2 (PANK2)-associated neurodegeneration (PKAN) and NBIA2 or infantile neuroaxonal dystrophy (INAD) due to mutations in the phospholipase A2, group VI (PLA2G6) gene. We have recently demonstrated clinical heterogeneity in patients with mutations in the PLA2G6 gene by identifying a poorly de...

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