Neuroanatomic profile of polyglutamine immunoreactivity inhuntington disease brains

Emily S. Herndon, Christa L. Hladik, Ping Shang, Dennis K. Burns, Jack Raisanen, Charles L. White

Research output: Contribution to journalArticle

29 Citations (Scopus)

Abstract

A pathologic hallmark of Huntington disease (HD) is the presence of intraneuronal aggregates of polyglutamine-containing huntingtin protein fragments. Monoclonal antibody 1C2 is a commercial antibody to normal human TATA-binding protein that detects long stretches of glutamine residues. Using 1C2 as a surrogate marker formutant huntingtin protein, we immunostained 19 HD cases, 10 normal controls, and 10 cases of frontotemporal degeneration with ubiquitinated inclusions as diseased controls. In the HD cases, there was consistent 1C2 immunoreactivity in the neocortex, striatum, hippocampus, lateral geniculate body, basis pontis, medullary reticular formation, and cerebellar dentate nucleus. The normal and diseased controls demonstrated 1C2 immunoreactivity only in the substantia nigra, locus coeruleus, and pituitary gland. Staining of 5 HD cases and 5 normal controls revealed a less consistent and less diagnostically useful morphologic immunoreactivity profile. These results indicate that widespread 1C2 immunoreactivity is present in diverse central nervous system areas in HD, and that in the appropriate setting, 1C2 staining can be a useful tool in the postmortem diagnosis of HD when neuromelanin-containing neuronal populations are avoided.

Original languageEnglish (US)
Pages (from-to)250-261
Number of pages12
JournalJournal of Neuropathology and Experimental Neurology
Volume68
Issue number3
DOIs
StatePublished - Mar 2009

Fingerprint

Huntington Disease
Brain Diseases
TATA-Box Binding Protein
Staining and Labeling
Geniculate Bodies
Cerebellar Nuclei
Reticular Formation
Locus Coeruleus
Neocortex
Cytomegalovirus Infections
Substantia Nigra
Pituitary Gland
Glutamine
polyglutamine
Hippocampus
Central Nervous System
Biomarkers
Monoclonal Antibodies
Antibodies
Population

Keywords

  • 1C2
  • Frontotemporal Degeneration With Ubiquitinated Inclusions
  • Huntington Disease
  • Immunohistochemistry
  • Inclusions
  • Neuromelain
  • Polyglutamine

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Neurology
  • Cellular and Molecular Neuroscience

Cite this

Neuroanatomic profile of polyglutamine immunoreactivity inhuntington disease brains. / Herndon, Emily S.; Hladik, Christa L.; Shang, Ping; Burns, Dennis K.; Raisanen, Jack; White, Charles L.

In: Journal of Neuropathology and Experimental Neurology, Vol. 68, No. 3, 03.2009, p. 250-261.

Research output: Contribution to journalArticle

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