Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease

The Wobbler mouse, a mutant characterized by motoneuron degeneration in the cervical spinal cord, has been used to test the efficacy of novel treatments for human motoneuron diseases (HMD). Previous reports have shown that slow axonal transport is impaired in Wobblers and other models of HMD. Since...

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Publicado:	2005
Materias:	Axonal transport Fluorogold Motoneuron degeneration Progesterone Spinal cord Wobbler mouse fluorescent dye fluorogold neurotrophic factor progesterone animal behavior animal experiment animal model animal tissue arm muscle article biceps brachii muscle cell count cell labeling cervical spinal cord controlled study drug effect drug exposure drug mechanism female flexor muscle fluorescence gastrocnemius muscle leg muscle male motoneuron motor neuron disease motor performance mouse mouse mutant nerve degeneration nerve fiber transport neuromuscular function nonhuman priority journal spinal cord motoneuron weight gain Animals Axonal Transport Behavior, Animal Cervical Vertebrae Disease Models, Animal Female Male Mice Mice, Neurologic Mutants Motor Neuron Disease Motor Neurons Progestins Spinal Cord Stilbamidines Upper Extremity
Acceso en línea:	https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00144886_v195_n2_p518_GonzalezDeniselle http://hdl.handle.net/20.500.12110/paper_00144886_v195_n2_p518_GonzalezDeniselle
Aporte de:	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires

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spelling	paper:paper_00144886_v195_n2_p518_GonzalezDeniselle2023-06-08T14:37:20Z Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease Axonal transport Fluorogold Motoneuron degeneration Progesterone Spinal cord Wobbler mouse fluorescent dye fluorogold neurotrophic factor progesterone animal behavior animal experiment animal model animal tissue arm muscle article biceps brachii muscle cell count cell labeling cervical spinal cord controlled study drug effect drug exposure drug mechanism female flexor muscle fluorescence gastrocnemius muscle leg muscle male motoneuron motor neuron disease motor performance mouse mouse mutant nerve degeneration nerve fiber transport neuromuscular function nonhuman priority journal spinal cord motoneuron weight gain Animals Axonal Transport Behavior, Animal Cervical Vertebrae Disease Models, Animal Female Male Mice Mice, Neurologic Mutants Motor Neuron Disease Motor Neurons Progestins Spinal Cord Stilbamidines Upper Extremity The Wobbler mouse, a mutant characterized by motoneuron degeneration in the cervical spinal cord, has been used to test the efficacy of novel treatments for human motoneuron diseases (HMD). Previous reports have shown that slow axonal transport is impaired in Wobblers and other models of HMD. Since progesterone (PROG) corrects some morphological, molecular, and functional abnormalities of Wobbler mice, we studied if steroid exposure for 8 weeks restored retrograde axonal transport by measuring motoneuron labeling after injection of fluorogold into the limb muscles. The dye was injected into forelimb biceps bracchii and flexor or into the rearlimb gastrocnemius muscles; 6 days later, the number of fluorescent motoneurons and the total number of cresyl violet stained motoneurons were counted in the cervical (C5-T1) or lumbar (L3-L5) spinal cord regions. A pronounced reduction (- 42.2%) of the percent of fluorescent motoneurons in Wobbler mice cervical cord was noted, which was significantly corrected after PROG treatment. In contrast, labeling of lumbar motoneurons was not reduced in Wobbler mice and was not affected by PROG treatment. In no case PROG showed an effect in control mice. Concomitantly, PROG slightly but significantly increased biceps weight of Wobbler mice. Behaviorally, PROG-treated Wobblers performed better on a motor test (hanging time from a horizontal rope) compared to untreated counterparts. We postulate a dual role for PROG in the Wobbler mouse, in part by prevention of motoneuron degeneration and also by enhancement of axonal transport. The latter mechanism could improve the traffic of neurotrophic factors from the forelimb muscles into the ailing motoneurons, improving neuromuscular function in this murine model of HMD. © 2005 Elsevier Inc. All rights reserved. 2005 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00144886_v195_n2_p518_GonzalezDeniselle http://hdl.handle.net/20.500.12110/paper_00144886_v195_n2_p518_GonzalezDeniselle
institution	Universidad de Buenos Aires
institution_str	I-28
repository_str	R-134
collection	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA)
topic	Axonal transport Fluorogold Motoneuron degeneration Progesterone Spinal cord Wobbler mouse fluorescent dye fluorogold neurotrophic factor progesterone animal behavior animal experiment animal model animal tissue arm muscle article biceps brachii muscle cell count cell labeling cervical spinal cord controlled study drug effect drug exposure drug mechanism female flexor muscle fluorescence gastrocnemius muscle leg muscle male motoneuron motor neuron disease motor performance mouse mouse mutant nerve degeneration nerve fiber transport neuromuscular function nonhuman priority journal spinal cord motoneuron weight gain Animals Axonal Transport Behavior, Animal Cervical Vertebrae Disease Models, Animal Female Male Mice Mice, Neurologic Mutants Motor Neuron Disease Motor Neurons Progestins Spinal Cord Stilbamidines Upper Extremity
spellingShingle	Axonal transport Fluorogold Motoneuron degeneration Progesterone Spinal cord Wobbler mouse fluorescent dye fluorogold neurotrophic factor progesterone animal behavior animal experiment animal model animal tissue arm muscle article biceps brachii muscle cell count cell labeling cervical spinal cord controlled study drug effect drug exposure drug mechanism female flexor muscle fluorescence gastrocnemius muscle leg muscle male motoneuron motor neuron disease motor performance mouse mouse mutant nerve degeneration nerve fiber transport neuromuscular function nonhuman priority journal spinal cord motoneuron weight gain Animals Axonal Transport Behavior, Animal Cervical Vertebrae Disease Models, Animal Female Male Mice Mice, Neurologic Mutants Motor Neuron Disease Motor Neurons Progestins Spinal Cord Stilbamidines Upper Extremity Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease
topic_facet	Axonal transport Fluorogold Motoneuron degeneration Progesterone Spinal cord Wobbler mouse fluorescent dye fluorogold neurotrophic factor progesterone animal behavior animal experiment animal model animal tissue arm muscle article biceps brachii muscle cell count cell labeling cervical spinal cord controlled study drug effect drug exposure drug mechanism female flexor muscle fluorescence gastrocnemius muscle leg muscle male motoneuron motor neuron disease motor performance mouse mouse mutant nerve degeneration nerve fiber transport neuromuscular function nonhuman priority journal spinal cord motoneuron weight gain Animals Axonal Transport Behavior, Animal Cervical Vertebrae Disease Models, Animal Female Male Mice Mice, Neurologic Mutants Motor Neuron Disease Motor Neurons Progestins Spinal Cord Stilbamidines Upper Extremity
description	The Wobbler mouse, a mutant characterized by motoneuron degeneration in the cervical spinal cord, has been used to test the efficacy of novel treatments for human motoneuron diseases (HMD). Previous reports have shown that slow axonal transport is impaired in Wobblers and other models of HMD. Since progesterone (PROG) corrects some morphological, molecular, and functional abnormalities of Wobbler mice, we studied if steroid exposure for 8 weeks restored retrograde axonal transport by measuring motoneuron labeling after injection of fluorogold into the limb muscles. The dye was injected into forelimb biceps bracchii and flexor or into the rearlimb gastrocnemius muscles; 6 days later, the number of fluorescent motoneurons and the total number of cresyl violet stained motoneurons were counted in the cervical (C5-T1) or lumbar (L3-L5) spinal cord regions. A pronounced reduction (- 42.2%) of the percent of fluorescent motoneurons in Wobbler mice cervical cord was noted, which was significantly corrected after PROG treatment. In contrast, labeling of lumbar motoneurons was not reduced in Wobbler mice and was not affected by PROG treatment. In no case PROG showed an effect in control mice. Concomitantly, PROG slightly but significantly increased biceps weight of Wobbler mice. Behaviorally, PROG-treated Wobblers performed better on a motor test (hanging time from a horizontal rope) compared to untreated counterparts. We postulate a dual role for PROG in the Wobbler mouse, in part by prevention of motoneuron degeneration and also by enhancement of axonal transport. The latter mechanism could improve the traffic of neurotrophic factors from the forelimb muscles into the ailing motoneurons, improving neuromuscular function in this murine model of HMD. © 2005 Elsevier Inc. All rights reserved.
title	Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease
title_short	Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease
title_full	Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease
title_fullStr	Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease
title_full_unstemmed	Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease
title_sort	progesterone restores retrograde labeling of cervical motoneurons in wobbler mouse motoneuron disease
publishDate	2005
url	https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00144886_v195_n2_p518_GonzalezDeniselle http://hdl.handle.net/20.500.12110/paper_00144886_v195_n2_p518_GonzalezDeniselle
_version_	1768544579352526848

Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease

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