Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia
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Caspase-12 controls West Nile virus infection via the viral RNA receptor RIG-I.In vivo maturation of human frataxinA pool of extramitochondrial frataxin that promotes cell survivalIron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster deliveryUnderstanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular modelsHDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse modelOxidative stress and the homeodynamics of iron metabolismThe first cellular models based on frataxin missense mutations that reproduce spontaneously the defects associated with Friedreich ataxiaReduced expression of mitochondrial frataxin in mice exacerbates diet-induced obesityProlonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse modelMutational ataxia resulting from abnormal vestibular acquisition and processing is partially compensated forNeurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich's ataxia.Iron redistribution as a therapeutic strategy for treating diseases of localized iron accumulation.Mitochondrial dysfunction induced by frataxin deficiency is associated with cellular senescence and abnormal calcium metabolismGAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology.Gene expression profiling in frataxin deficient mice: microarray evidence for significant expression changes without detectable neurodegenerationHighlighting anatomical sub-structures in rat brain tissue using lipid imaging.Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.Intracellular iron transport and storage: from molecular mechanisms to health implications.Clinical data and characterization of the liver conditional mouse model exclude neoplasia as a non-neurological manifestation associated with Friedreich's ataxia.Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration.Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammalsFriedreich's ataxia: pathology, pathogenesis, and molecular genetics.Smad2 protein disruption in the central nervous system leads to aberrant cerebellar development and early postnatal ataxia in miceA novel GAA-repeat-expansion-based mouse model of Friedreich's ataxiaMitochondrial Diseases Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function.A new cellular model to follow Friedreich's ataxia development in a time-resolved way.Pathways to motor incoordination: the inherited ataxias.Rapid Onset of Motor Deficits in a Mouse Model of Spinocerebellar Ataxia Type 6 Precedes Late Cerebellar DegenerationThe Immp2l mutation causes age-dependent degeneration of cerebellar granule neurons prevented by antioxidant treatment.The role of mitochondria in inherited neurodegenerative diseases.Autophagy induction extends lifespan and reduces lipid content in response to frataxin silencing in C. elegans.Dorsal root ganglia in Friedreich ataxia: satellite cell proliferation and inflammation.Autophagy in neuroprotection and neurodegeneration: A question of balance.Unanswered questions in Friedreich ataxia.Friedreich ataxia: new pathways.Gene Transfer of Brain-derived Neurotrophic Factor (BDNF) Prevents Neurodegeneration Triggered by FXN DeficiencyFunctional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARgamma pathway as a therapeutic target in Friedreich's ataxia.Vitamin E is essential for Purkinje neuron integrity.Targeting of sodium channel blockers into nociceptors to produce long-duration analgesia: a systematic study and review.
P2860
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P2860
Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia
description
2004 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 2004
@ast
im Februar 2004 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2004/02/25)
@sk
vědecký článek publikovaný v roce 2004
@cs
wetenschappelijk artikel (gepubliceerd op 2004/02/25)
@nl
наукова стаття, опублікована в лютому 2004
@uk
مقالة علمية (نشرت في 25-2-2004)
@ar
name
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@ast
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@en
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@nl
type
label
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@ast
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@en
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@nl
prefLabel
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@ast
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@en
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@nl
P2093
P3181
P1476
Friedreich ataxia mouse models ...... eration in dorsal root ganglia
@en
P2093
Anne Gansmuller
Delphine Simon
Hervé Seznec
Michel Koenig
Nadège Carelle
Philipp Weber
Pierre Rustin
P304
P3181
P356
10.1523/JNEUROSCI.4549-03.2004
P407
P577
2004-02-01T00:00:00Z