Ciliary neurotrophic factor receptor expression in spinal cord and motor cortex in amyotrophic lateral sclerosis

R. M. Duberley*, I. P. Johnson, P. Anand, M. Swash, J. Martin, P. N. Leigh, S. Zeman

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

28 Citations (Scopus)

Abstract

Ciliary neurotrophic factor (CNTF) is known to rescue motor neurones in animal models of injury and neurodegeneration and we have recently described regional changes of CNTF protein levels in spinal cord from patients with sporadic motor neurone disease of the amyotrophic lateral sclerosis (ALS) type. However, information is lacking about the CNTF receptor in this condition. We have therefore studied mRNA levels for the α subunit of the CNTF receptor (CNTFRα) and for CNTF itself in postmortem spinal cord and cerebral cortex in patients with sporadic ALS and matched controls. We report that in the spinal cord of ALS patients there is a marked increase in the hybridisation signal for CNTFRα subunit mRNA overlying motor neurones. In contrast, very little mRNA for CNTFRα subunit was found in the motor cortex and no differences were seen between ALS and controls. We were unable to detect any hybridisation signal for CNTF mRNA. Our findings provide evidence for regional differences in CNTF receptor expression in upper- and lower-motor neurones in ALS.

Original languageEnglish
Pages (from-to)109-113
Number of pages5
JournalJournal of the Neurological Sciences
Volume129
Issue numberSupplement
DOIs
Publication statusPublished - 1 Jan 1995
Externally publishedYes

Keywords

  • ALS
  • CNTF receptors
  • Human motor neurone disease
  • In situ hybridisation
  • Motor cortex
  • Spinal cord

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