TY  - JOUR
A1  - Sendtner, Michael
A1  - Stöckli, Kurt A.
A1  - Thoenen, Hans
A1  - Schmalbruch, H.
A1  - Carroll, P.
A1  - Kreutzberg, Georg W.
T1  - Ciliary neurotrophic factor prevents the degeneration of motor neurons in mouse mutant progressive motor neuronopathy
N2  - CILIARY neurotrophic factor (CNTF) supports the survival of embryonic motor neurons in vitro and in vivo and prevents lesion-mediated degeneration of rat motor neuron~ during early post-natal stages. Here we report that CNTF greatly reduces all the functional and morphological changes in pmnlpmn mice5, an autosomal recessive mutant leading to progressive caudo-cranial motor neuron degeneration. The first manifestations of progressive motor neuronopathy in homozygous pmnl pmn mice become apparent in the hind limbs at the end of the third post-natal week and all the mice die up to 6 or 7 weeks after birth from respiratory paralysis. Treatment with CNTF prolongs- survival- and greatly Impoves motor function of these mice. Moreover, morphological manifestations, such as loss of motor axons in the phrenic nerve and degeneration of facial motor neurons, were greatly reduced by CNTF, although the treatment did not start until the first symptoms of the disease had already become apparent and substantial degenerative changes were already present. The protective and restorative effects of CNTF in this mouse mutant give new perspectives for the treatment of human degenerative motor neuron diseases with CNTF.
Y1  - 1992
UR  - https://opus.bibliothek.uni-wuerzburg.de/frontdoor/index/index/docId/3566
UR  - https://nbn-resolving.org/urn:nbn:de:bvb:20-opus-42563
ER  -