Brain, Vol. 109, No. 4, 629-647, 1986
© 1986 Guarantors of Brain
research-article |
THE DEVELOPMENT AND PATHOGENESIS OF THE SENSORY NEUROPATHY IN THE MUTANT RAT mf
Department of Neuropathology, Institute of Neurology London
Correspondence to:
Correspondence to Dr F. Scaravilli, Institute of Neurology, Queen Square, London WC1N 3BG, UK.
SUMMARY
A study was made of the development of sensory pathways in the mutant rat mutilated foot (mf) which is affected by a sensory neuropathy with autosomal recessive inheritance. Microscopic abnormalities are well recognizable at the fifteenth embryonic day By day 16, dorsal root ganglia are smaller than normal and show more numerous foci of cell necrosis which continue throughout the remainder of gestation and during the first and second postnatal days. During this period the number of ganglion cells decreases sharply. Reconstruction of cell volumes shows that the larger cells are more severely affected. The secondary sensory nuclei (gracile nuclei) are normal at birth but during the first two postnatal weeks become progressively smaller than in normal rats
The results suggest that the mutant gene acts primarily on the dorsal root ganglia causing excessive neuronal cell death. Qualitatively, the events in this mutant are closely similar to programmed cell death in the normal. It is likely that neurons of second order nuclei, which are not contacted by afferent fibres, undergo a process of transneuronal degeneration as a secondary effect of excessive ganglion cell loss.
Received September 20, 1985. Revised October 31, 1985. Accepted November 19, 1985.
![]()
CiteULike
Connotea
Del.icio.us What's this?
This article has been cited by other articles:
![]() |
A Bouhouche, A Benomar, N Bouslam, T Chkili, and M Yahyaoui Mutation in the epsilon subunit of the cytosolic chaperonin-containing t-complex peptide-1 (Cct5) gene causes autosomal recessive mutilating sensory neuropathy with spastic paraplegia J. Med. Genet., May 1, 2006; 43(5): 441 - 443. [Abstract] [Full Text] [PDF] |
||||
![]() |
M.-J. Lee, D. A. Stephenson, M. J. Groves, M. G. Sweeney, M. B. Davis, S.-F. An, H. Houlden, M. A. M. Salih, V. Timmerman, P. de Jonghe, et al. Hereditary sensory neuropathy is caused by a mutation in the delta subunit of the cytosolic chaperonin-containing t-complex peptide-1 (Cct4 ) gene Hum. Mol. Genet., August 1, 2003; 12(15): 1917 - 1925. [Abstract] [Full Text] [PDF] |
||||

