GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology

Genomics

Volumn 88, Issue 5, 2006, Pages 580-590

  Al Mahdawi, Sahar ^a   Pinto, Ricardo Mouro ^a   Varshney, Dhaval ^a   Lawrence, Lorraine ^b   Lowrie, Margaret B ^b   Hughes, Sian ^c   Webster, Zoe ^d   Blake, Julian ^e   Cooper, J Mark ^f   King, Rosalind ^f   Pook, Mark A ^a  

^a BRUNEL UNIVERSITY   (United Kingdom)

^b IMPERIAL COLLEGE LONDON   (United Kingdom)

^c UNIVERSITY COLLEGE LONDON   (United Kingdom)

^d HAMMERSMITH HOSPITAL   (United Kingdom)

^e NORFOLK AND NORWICH UNIVERSITY HOSPITAL   (United Kingdom)

^f ROYAL FREE AND UNIVERSITY COLLEGE MEDICAL SCHOOL   (United Kingdom)

Author keywords

Frataxin; FRDA; Friedreich ataxia; FXN; GAA repeat; Mouse model

Indexed keywords

ACONITATE HYDRATASE; FRATAXIN; GAA PROTEIN; MESSENGER RNA; PROTEIN; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; CROSS BREEDING; DEMYELINATION; DNA MODIFICATION; ENZYME ACTIVITY; FRIEDREICH ATAXIA; GENE MUTATION; HEART DISEASE; HETEROZYGOSITY; HUMAN; HUMAN CELL; KNOCKOUT MOUSE; LOCOMOTION; MOUSE; MOUSE STRAIN; NERVE FIBER; NONHUMAN; OXIDATIVE STRESS; PATHOLOGY; PHENOTYPE; PRIORITY JOURNAL; PROTEIN EXPRESSION; ROTAROD TEST; SPINAL GANGLION; TRANSGENIC MOUSE;

ACONITATE HYDRATASE; ANIMALS; DISEASE MODELS, ANIMAL; ELECTRON TRANSPORT; ELECTROPHYSIOLOGY; FRIEDREICH ATAXIA; GANGLIA, SPINAL; GENE EXPRESSION; GENOMICS; HUMANS; IRON-BINDING PROTEINS; MICE; MICE, KNOCKOUT; MICE, TRANSGENIC; MYOCARDIUM; NEURONS; OXIDATIVE STRESS; PHENOTYPE; RNA, MESSENGER; TRINUCLEOTIDE REPEAT EXPANSION;

ATAXIA; MUS MUSCULUS;

EID: 33749638768     PISSN: 08887543     EISSN: 10898646     Source Type: Journal    
DOI: 10.1016/j.ygeno.2006.06.015     Document Type: Article

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