Region-specific interneuronal degeneration in the human cerebral cortex in Huntington’s disease correlates with symptomatology

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dc.contributor.advisor Faull, R en
dc.contributor.advisor Waldvogel, H en Mehrabi, Fahimeh en 2015-09-30T23:59:09Z en 2015 en
dc.identifier.citation 2015 en
dc.identifier.uri en
dc.description.abstract Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder characterised by variable symptoms (choreiform movements, cognitive, mood and neuropsychological changes), with variable neuropathology of the basal ganglia and cerebral cortex. Recent studies from our laboratory have shown that the pattern of cortical pyramidal cell loss in 8 different cortical regions correlates with the phenotypic variability in HD; and that in 2 regions of the cerebral cortex (the primary motor cortex, and the anterior cingulate gyrus) the pattern of interneuronal degeneration correlates with pyramidal cell death and variable HD symptom profiles. The present study completes this overview of human cortical neuronal degeneration in the human brain in HD. This study of the HD human brain specifically examines the pattern of interneuronal degeneration in the primary sensory cortex, superior frontal cortex, and superior parietal cortex, and correlates these findings with the well characterised clinical and pathological history of the HD cases. To undertake this investigation, unbiased stereological counting methods were used to quantify the three major types of interneurons immunoreactive for calbindin-D28k, calretinin, and parvalbumin in the three cortical regions of 14 HD and 13 control cases of post-mortem human brain. Based on their predominant symptom, the HD cases were categorised into three groups (“motor”, “mood”, “mixed”). The results demonstrated a heterogeneous loss of interneurons across the three cortical regions, which paralleled the heterogeneous pattern of pyramidal cell loss in the same cortical areas. Most interestingly, the pattern of GABAergic interneuronal loss in these cortical regions correlated with the variable symptom profiles in different human HD cases. These findings extend our understanding about the role of the cerebral cortex in the HD pathogenesis and symptomatology by showing a precise correlation between the cortical interneuronal loss and heterogeneous symptomatology in HD. en
dc.publisher ResearchSpace@Auckland en
dc.relation.ispartof PhD Thesis - University of Auckland en
dc.relation.isreferencedby UoA99264820610102091 en
dc.rights Items in ResearchSpace are protected by copyright, with all rights reserved, unless otherwise indicated. Previously published items are made available in accordance with the copyright policy of the publisher. en
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dc.title Region-specific interneuronal degeneration in the human cerebral cortex in Huntington’s disease correlates with symptomatology en
dc.type Thesis en Biomedical Science en The University of Auckland en Doctoral en PhD en
dc.rights.holder Copyright: The Author en
dc.rights.accessrights en
pubs.elements-id 500429 en Medical and Health Sciences en Medical Sciences en Pharmacology en
pubs.record-created-at-source-date 2015-10-01 en
dc.identifier.wikidata Q112909944

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