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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
MeSH Review

Intranuclear Inclusion Bodies

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Disease relevance of Intranuclear Inclusion Bodies


Psychiatry related information on Intranuclear Inclusion Bodies


High impact information on Intranuclear Inclusion Bodies


Chemical compound and disease context of Intranuclear Inclusion Bodies


Biological context of Intranuclear Inclusion Bodies


Anatomical context of Intranuclear Inclusion Bodies


Associations of Intranuclear Inclusion Bodies with chemical compounds

  • Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila [27].
  • We have identified a novel guanosine triphosphatase (GTPase) named CRAG that contains a nuclear localization signal (NLS) sequence and forms nuclear inclusions in response to stress [28].
  • These data provide the first physiological evidence that polyalanine expansions are not essential to induce polymerization of PABPN1 into filamentous nuclear inclusions [29].
  • However, two mutant isoforms, R256C and R275W, within RING finger 1 of the Parkin protein (238-293 amino acids), produced an unusual distribution of the protein, with large cytoplasmic and nuclear inclusions [30].
  • Extrapolating from TEM to standard light microscopy on hematoxylin and eosin-stained paraffin sections, eosinophilic, targetoid intranuclear inclusions were identified within spindle cells which often lined vascular lumina [31].

Gene context of Intranuclear Inclusion Bodies

  • Neuronal intranuclear inclusions and cytoplasmic aggregates composed of the mutant huntingtin within certain neuronal populations are a characteristic hallmark of HD [32].
  • However, in 293 cells HHR23A is recruited to intranuclear inclusions formed by the mutant ataxin-3 through its interaction with ataxin-3 [33].
  • Neuronal intranuclear inclusions were detected in the brain of an early onset SCA7 case with the 1C2 antibody directed against an expanded polyglutamine domain [34].
  • FXTAS is thought to be caused by a toxic 'gain-of-function' of the expanded CGG-repeat FMR1 mRNA, which is found in the neuronal and astrocytic intranuclear inclusions associated with the disorder [35].
  • The observations in expanded-repeat mice support a direct role of the Fmr1 gene, by either CGG expansion per se or by mRNA level, in the formation of the inclusions and suggest a correlation between the presence of intranuclear inclusions in distinct regions of the brain and the clinical features in symptomatic premutation carriers [36].

Analytical, diagnostic and therapeutic context of Intranuclear Inclusion Bodies


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