Discover Community Create You

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

Inducible and reversible phenotypes in a novel mouse model of Friedreich's Ataxia.

eLife - 19 Dec 2017

Chandran Vijayendran, Gao Kun, Swarup Vivek, Versano Revital, Dong Hongmei, Jordan Maria C, Geschwind Daniel H

Abstract excerpt

Friedreich's ataxia (FRDA), the most common inherited ataxia, is caused by recessive mutations that reduce the levels of frataxin (FXN), a mitochondrial iron binding protein. We developed an inducible mouse model of Fxn deficiency that enabled us to control the onset and progression of disease phenotypes by the modulation of Fxn levels. Systemic knockdown of Fxn in adult mice led to multiple phenotypes...

Read the complete abstract on PubMed

Topics

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

Share to Disease Models, Animal
Share to Frataxin
Share to Friedreich Ataxia

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Gene Expression Regulation

Share to Gene Knockdown Techniques

Share to Iron-Binding Proteins

Share to Phenotype

Source

Open in PubMed Open full text

Identifiers

PMID: 29257745
DOI: 10.7554/eLife.30054
PMCID: PMC5736353

Genes

FXN

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Inducible and reversible phenotypes in a novel mouse model of Friedreich's Ataxia.PMID 29257745Read ↗

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

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

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

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

A modified mouse model of Friedreich's ataxia with conditional Fxn allele homozygosity delays onset of cardiomyopathy.Semantic proximity Read ↗

Beyond loss of frataxin: the complex molecular pathology of Friedreich ataxia.Semantic proximity Read ↗

Biochemical alterations precede neurobehavioral deficits in a novel mouse model of Friedreich ataxiaSemantic proximity Read ↗

Advances in Gene and Cellular Therapy in Friedreich Ataxia.Semantic proximity Read ↗

SYSTEMIC FRATAXIN DEFICIENCY CAUSES TISSUE-DEPENDENT IRON HOMEOSTASIS ALTERATIONS: IMPLICATIONS FOR FRIEDREICH ATAXIASemantic proximity Read ↗

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

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

Neurodegeneration in Friedreich’s Ataxia: From Defective Frataxin to Oxidative StressSemantic proximity Read ↗

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