Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 25 Feb 2004

Simon Delphine, Seznec Hervé, Gansmuller Anne, Carelle Nadège, Weber Philipp, Metzger Daniel, Rustin Pierre, Koenig Michel, Puccio Hélène

Abstract excerpt

Friedreich ataxia (FRDA), the most common recessive ataxia, is characterized by degeneration of the large sensory neurons of the spinal cord and cardiomyopathy. It is caused by severely reduced levels of frataxin, a mitochondrial protein involved in iron-sulfur cluster (ISC) biosynthesis. Through...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Ataxia
Share to Cerebellar Ataxia

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Frataxin

Share to Friedreich Ataxia

Share to Ganglia, Spinal

Share to Iron-Binding Proteins

Share to Mice, Neurologic Mutants

Share to Sensation Disorders

Source

Open in PubMed Open full text

Identifiers

PMID: 14985441
DOI: 10.1523/JNEUROSCI.4549-03.2004
PMCID: PMC6730414

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026. Prepared record publication-14985441-2026-09-01.1. Manifest publication-graph-cohort-2026-09-01.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 1 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia.PMID 14985441Read ↗

GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology.Semantic proximity Read ↗

Frataxin deficiency and the pathology of Friedreich’s Ataxia across tissuesSemantic proximity Read ↗

Partial correction of sensitivity to oxidant stress in Friedreich ataxia patient fibroblasts by frataxin-encoding adeno-associated virus and lentivirus vectors.Semantic proximity Read ↗

Oxidative stress and loss of Fe-S proteins in Friedreich ataxia induced pluripotent stem cell-derived PSNs can be reversed by restoring FXN expression with a benzamide HDAC inhibitorSemantic proximity Read ↗

Friedreich Ataxia: Neuropathology RevisedSemantic proximity Read ↗

Inducible and reversible phenotypes in a novel mouse model of Friedreich’s AtaxiaSemantic proximity Read ↗

Inducible and reversible phenotypes in a novel mouse model of Friedreich's Ataxia.Semantic proximity Read ↗

Pathophysiogical and therapeutic progress in Friedreich ataxia.Semantic proximity Read ↗

A multiple animal and cellular models approach to study frataxin deficiency in Friedreich Ataxia.Semantic proximity Read ↗

Friedreich's ataxia: from disease mechanisms to therapeutic interventions.Semantic proximity Read ↗

'Mitochondrial energy imbalance and lipid peroxidation cause cell death in Friedreich's ataxia'.Semantic proximity Read ↗

The First Cellular Models Based on Frataxin Missense Mutations That Reproduce Spontaneously the Defects Associated with Friedreich AtaxiaSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.