[Huntington's disease: cellular and molecular basis of pathology].
Huntington's disease (HD) is an inherited autosomal dominant neurodegenerative disorder caused by a polyQ expansion (>36 glutamine repeats) in Huntingtin (Htt) protein. It is believed that dysfunction of corticostriatal system is one of the main pathological events. Special attention is paid to synaptic dysfunction of transmission between cortical and striatal neurons. This review focuses on modern concepts of molecular and cellular mechanisms of HD pathology and especially on alterations in corticostriatal connectivity. Calcium hypothesis of HD and new pharmacological targets are described.
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Korzhova
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Artamonov
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OL
Vlasova
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IB
Bezprozvannyĭ
D000978:Antiparkinson Agents / Q000627:therapeutic use; D002118:Calcium / Q000378:metabolism*; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002540:Cerebral Cortex / Q000187:drug effects / Q000378:metabolism* / Q000473:pathology; D003342:Corpus Striatum / Q000187:drug effects / Q000378:metabolism* / Q000473:pathology; D015870:Gene Expression; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000188:drug therapy / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D017275:Isradipine / Q000627:therapeutic use; D008559:Memantine / Q000627:therapeutic use; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000378:metabolism; D009474:Neurons / Q000187:drug effects / Q000378:metabolism / Q000473:pathology; D010647:Phenyl Ethers / Q000627:therapeutic use; D011799:Quinazolines / Q000627:therapeutic use; D009435:Synaptic Transmission / Q000187:drug effects
D000978:Antiparkinson Agents; D002121:Calcium Channel Blockers; C562064:EVP 4593; C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D010647:Phenyl Ethers; D011799:Quinazolines; D002118:Calcium; D008559:Memantine; D017275:Isradipine