Skip Navigation

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (14)
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by CHIMELLI, L.
Right arrow Articles by SCARAVILLI, F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by CHIMELLI, L.
Right arrow Articles by SCARAVILLI, F.
Social Bookmarking
 Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

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

LEILA CHIMELLI and FRANCESCO SCARAVILLI

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.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
J. Med. Genet.Home page
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]


Home page
Hum Mol GenetHome page
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]



Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.