CILIARY NEUROTROPHIC FACTOR-RECEPTOR EXPRESSION IN SPINAL-CORD AND MOTOR CORTEX IN AMYOTROPHIC-LATERAL-SCLEROSIS

Ciliary neurotrophic factor (CNTF) is known to rescue motor neurones i n animal models of injury and neurodegeneration and we have recently d escribed regional changes of CNTF protein levels in spinal cord from p atients 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 alpha subunit of the CNTF receptor (CNTFR alpha) and for CNTF itse lf in postmortem spinal cord and cerebral cortex in patients with spor adic ALS and matched controls. We report that in the spinal cord of AL S patients there is a marked increase in the hybridisation signal for CNTFR alpha subunit mRNA overlying motor neurones. In contrast, very l ittle mRNA for CNTFR alpha subunit was found in the motor cortex and n o differences were seen between ALS and controls. We were unable to de tect any hybridisation signal for CNTF mRNA. Our findings provide evid ence for regional differences in CNTF receptor expression in upper- an d lower-motor neurones in ALS.