Huntington's disease: new paths to pathogenesis.
Huntington's disease is a progressive autosomal dominant neurodegenerative disorder caused by expansion of a CAG repeat coding for polyglutamine in the huntingtin protein. A recent report suggests a new mechanism involving altered interactions with a protein involved in axonal transport, leading to loss of neurotrophic factor transport. This suggests an intriguing convergence to previously described pathways implicating neurotrophin transcription in HD pathogenesis.
Ross
Christopher A
CA
Department of Psychiatry, 618 Ross Research Building, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205, USA.
D001692:Biological Transport; D019208:Brain-Derived Neurotrophic Factor / Q000378:metabolism*; D002470:Cell Survival; D002478:Cells, Cultured; D022162:Cytoplasmic Vesicles / Q000378:metabolism*; D004268:DNA-Binding Proteins / Q000378:metabolism; D006816:Huntington Disease / Q000209:etiology* / Q000378:metabolism*; D008954:Models, Biological; D009419:Nerve Tissue Proteins / Q000378:metabolism; D009474:Neurons / Q000378:metabolism* / Q000473:pathology; D009687:Nuclear Proteins / Q000378:metabolism
D019208:Brain-Derived Neurotrophic Factor; D004268:DNA-Binding Proteins; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins