Friedreich Ataxia Mouse Models with Progressive Cerebellar and Sensory Ataxia Reveal Autophagic Neurodegeneration in Dorsal Root Ganglia

Journal of Neuroscience

Volumn 24, Issue 8, 2004, Pages 1987-1995

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

^a INSTITUT DE GÉNÉTIQUE ET DE BIOLOGIE MOLÉCULAIRE ET CELLULAIRE   (France)

^b CU de Strasbourg ^*  (France)

^c HÔPITAL NECKER ENFANTS MALADES   (France)

Author keywords

Behavior; Degeneration; Dorsal root ganglion; DRG; Friedreich ataxia; Mitochondria; Mouse model; Neuropathology; Purkinje cell

Indexed keywords

FRATAXIN; IRON SULFUR PROTEIN; LIPOFUSCIN; MITOCHONDRIAL PROTEIN;

ADOLESCENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; AUTOPHAGY; CARDIOMYOPATHY; CEREBELLAR ATAXIA; FRIEDREICH ATAXIA; GENE TARGETING; MITOCHONDRION; MOUSE; MOUSE MUTANT; NERVE CELL DEGENERATION; NEUROPATHOLOGY; NONHUMAN; PRIORITY JOURNAL; PROTEIN SYNTHESIS; PURKINJE CELL; RECESSIVE INHERITANCE; SENSORY ATAXIA; SENSORY DYSFUNCTION; SENSORY NERVE CELL; SPINAL CORD; SPINAL GANGLION;

ANIMALS; ATAXIA; AUTOPHAGY; CEREBELLAR ATAXIA; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; FRIEDREICH ATAXIA; GANGLIA, SPINAL; IRON-BINDING PROTEINS; MICE; MICE, KNOCKOUT; MICE, NEUROLOGIC MUTANTS; MOTOR ACTIVITY; NERVE DEGENERATION; PHENOTYPE; SENSATION DISORDERS;

EID: 1442324707     PISSN: 02706474     EISSN: None     Source Type: Journal    
DOI: 10.1523/JNEUROSCI.4549-03.2004     Document Type: Article

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