Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset.
Neuronal intranuclear inclusions were recently found in the brain of patients with inherited neurodegenerative disorders characterized by the expansion of a polyglutamine stretch in the mutated protein. These inclusions are ubiquitinated and, for some of these diseases, the presence of the mutated protein could be also identified. Using immunohistochemistry, we show here that ubiquitinated intranuclear inclusions are also observed postmortem in the brain of patients suffering from Huntington's disease characterized by small polyglutamine expansions and adult onset. We were, however, unable to detect the mutated form of huntingtin in these inclusions. These intranuclear inclusions were detected only in the affected cerebral regions, suggesting that their presence is probably linked to the neurodegenerative process.
Gourfinkel-An
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I
INSERM U289, R. Escourolle, Hôpital de la Salpêtrière, Paris, France.
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G
G
Duyckaerts
C
C
Faucheux
B
B
Hauw
J J
JJ
Trottier
Y
Y
Brice
A
A
Agid
Y
Y
Hirsch
E C
EC
D000328:Adult; D017668:Age of Onset; D000911:Antibodies, Monoclonal; D016022:Case-Control Studies; D002467:Cell Nucleus / Q000648:ultrastructure*; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics / Q000473:pathology*; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008875:Middle Aged; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000032:analysis / Q000235:genetics*; D009474:Neurons / Q000648:ultrastructure*; D009687:Nuclear Proteins / Q000032:analysis / Q000235:genetics*; D018911:Trinucleotide Repeats; D014452:Ubiquitins / Q000032:analysis
D000911:Antibodies, Monoclonal; C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D014452:Ubiquitins