Early Functional Impairment of Sensory-Motor Connectivity in a Mouse Model of Spinal Muscular Atrophy

To define alterations of neuronal connectivity that occur during motor neuron degeneration, we characterized the function and structure of spinal circuitry in spinal muscular atrophy (SMA) model mice. SMA motor neurons show reduced proprioceptive reflexes that correlate with decreased number and fun...

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Published in:	Neuron (Cambridge, Mass.) Vol. 69; no. 3; pp. 453 - 467
Main Authors:	Mentis, George Z., Blivis, Dvir, Liu, Wenfang, Drobac, Estelle, Crowder, Melissa E., Kong, Lingling, Alvarez, Francisco J., Sumner, Charlotte J., O'Donovan, Michael J.
Format:	Journal Article
Language:	English
Published:	United States Elsevier Inc 10-02-2011 Elsevier Limited
Subjects:	Age Factors Amyotrophic lateral sclerosis Animals Animals, Newborn Disease Models, Animal Mice Mice, Transgenic Motor Neurons - pathology Motor Neurons - physiology Muscular Atrophy, Spinal - genetics Muscular Atrophy, Spinal - pathology Muscular Atrophy, Spinal - physiopathology Muscular system Rodents Sensory Receptor Cells - pathology Sensory Receptor Cells - physiology Spinal cord Studies Synapses - pathology Synapses - physiology
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Abstract	To define alterations of neuronal connectivity that occur during motor neuron degeneration, we characterized the function and structure of spinal circuitry in spinal muscular atrophy (SMA) model mice. SMA motor neurons show reduced proprioceptive reflexes that correlate with decreased number and function of synapses on motor neuron somata and proximal dendrites. These abnormalities occur at an early stage of disease in motor neurons innervating proximal hindlimb muscles and medial motor neurons innervating axial muscles, but only at end-stage disease in motor neurons innervating distal hindlimb muscles. Motor neuron loss follows afferent synapse loss with the same temporal and topographical pattern. Trichostatin A, which improves motor behavior and survival of SMA mice, partially restores spinal reflexes, illustrating the reversibility of these synaptic defects. Deafferentation of motor neurons is an early event in SMA and may be a primary cause of motor dysfunction that is amenable to therapeutic intervention. ► Reduced proprioceptive reflex function precedes loss of motor neurons in SMA mice ► Motor neurons in SMA mice lose VGluT1 primary afferent terminals ► Medial motor neurons are affected earlier than lateral motor neurons ► Treatment with TSA improves reflex function and reverses motor neuron cell death
AbstractList	To define alterations of neuronal connectivity that occur during motor neuron degeneration, we characterized the function and structure of spinal circuitry in spinal muscular atrophy (SMA) model mice. SMA motor neurons show reduced proprioceptive reflexes that correlate with decreased number and function of synapses on motor neuron somata and proximal dendrites. These abnormalities occur at an early stage of disease in motor neurons innervating proximal hindlimb muscles and medial motor neurons innervating axial muscles, but only at end-stage disease in motor neurons innervating distal hindlimb muscles. Motor neuron loss follows afferent synapse loss with the same temporal and topographical pattern. Trichostatin A, which improves motor behavior and survival of SMA mice, partially restores spinal reflexes illustrating the reversibility of these synaptic defects. De-afferentation of motor neurons is an early event in SMA and may be a primary cause of motor dysfunction that is amenable to therapeutic intervention. To define alterations of neuronal connectivity that occur during motor neuron degeneration, we characterized the function and structure of spinal circuitry in spinal muscular atrophy (SMA) model mice. SMA motor neurons show reduced proprioceptive reflexes that correlate with decreased number and function of synapses on motor neuron somata and proximal dendrites. These abnormalities occur at an early stage of disease in motor neurons innervating proximal hindlimb muscles and medial motor neurons innervating axial muscles, but only at end-stage disease in motor neurons innervating distal hindlimb muscles. Motor neuron loss follows afferent synapse loss with the same temporal and topographical pattern. Trichostatin A, which improves motor behavior and survival of SMA mice, partially restores spinal reflexes, illustrating the reversibility of these synaptic defects. Deafferentation of motor neurons is an early event in SMA and may be a primary cause of motor dysfunction that is amenable to therapeutic intervention. ► Reduced proprioceptive reflex function precedes loss of motor neurons in SMA mice ► Motor neurons in SMA mice lose VGluT1 primary afferent terminals ► Medial motor neurons are affected earlier than lateral motor neurons ► Treatment with TSA improves reflex function and reverses motor neuron cell death To define alterations of neuronal connectivity that occur during motor neuron degeneration, we characterized the function and structure of spinal circuitry in spinal muscular atrophy (SMA) model mice. SMA motor neurons show reduced proprioceptive reflexes that correlate with decreased number and function of synapses on motor neuron somata and proximal dendrites. These abnormalities occur at an early stage of disease in motor neurons innervating proximal hindlimb muscles and medial motor neurons innervating axial muscles, but only at end-stage disease in motor neurons innervating distal hindlimb muscles. Motor neuron loss follows afferent synapse loss with the same temporal and topographical pattern. Trichostatin A, which improves motor behavior and survival of SMA mice, partially restores spinal reflexes, illustrating the reversibility of these synaptic defects. Deafferentation of motor neurons is an early event in SMA and may be a primary cause of motor dysfunction that is amenable to therapeutic intervention.
Author	Liu, Wenfang Sumner, Charlotte J. Mentis, George Z. Blivis, Dvir Alvarez, Francisco J. Kong, Lingling Crowder, Melissa E. Drobac, Estelle O'Donovan, Michael J.
AuthorAffiliation	1 Section of Developmental Biology, NINDS, NIH, Bethesda, MD, 20892, USA 4 Dept. of Neurology, Johns Hopkins University, Baltimore, MD, 21287, USA 5 Dept. of Neuroscience, Cell Biology & Physiology, Wright State University, Dayton, OH 45435, USA 3 Center for Motor Neuron Biology and Disease, Columbia University, New York, NY, 10032, USA 2 Dept. of Neurology & Columbia University, New York, NY, 10032, USA
AuthorAffiliation_xml	– name: 1 Section of Developmental Biology, NINDS, NIH, Bethesda, MD, 20892, USA – name: 3 Center for Motor Neuron Biology and Disease, Columbia University, New York, NY, 10032, USA – name: 5 Dept. of Neuroscience, Cell Biology & Physiology, Wright State University, Dayton, OH 45435, USA – name: 4 Dept. of Neurology, Johns Hopkins University, Baltimore, MD, 21287, USA – name: 2 Dept. of Neurology & Columbia University, New York, NY, 10032, USA
Author_xml	– sequence: 1 givenname: George Z. surname: Mentis fullname: Mentis, George Z. email: gzmentis@columbia.edu organization: Section on Developmental Biology, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA – sequence: 2 givenname: Dvir surname: Blivis fullname: Blivis, Dvir organization: Section on Developmental Biology, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA – sequence: 3 givenname: Wenfang surname: Liu fullname: Liu, Wenfang organization: Section on Developmental Biology, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA – sequence: 4 givenname: Estelle surname: Drobac fullname: Drobac, Estelle organization: Department of Neurology, Columbia University, New York, NY 10032, USA – sequence: 5 givenname: Melissa E. surname: Crowder fullname: Crowder, Melissa E. organization: Section on Developmental Biology, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA – sequence: 6 givenname: Lingling surname: Kong fullname: Kong, Lingling organization: Departments of Neurology and Neuroscience, The Johns Hopkins University, Baltimore, MD 21205, USA – sequence: 7 givenname: Francisco J. surname: Alvarez fullname: Alvarez, Francisco J. organization: Department of Neuroscience, Cell Biology, and Physiology, Wright State University, Dayton, OH 45435, USA – sequence: 8 givenname: Charlotte J. surname: Sumner fullname: Sumner, Charlotte J. organization: Departments of Neurology and Neuroscience, The Johns Hopkins University, Baltimore, MD 21205, USA – sequence: 9 givenname: Michael J. surname: O'Donovan fullname: O'Donovan, Michael J. organization: Section on Developmental Biology, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
BackLink	https://www.ncbi.nlm.nih.gov/pubmed/21315257$$D View this record in MEDLINE/PubMed
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Snippet	To define alterations of neuronal connectivity that occur during motor neuron degeneration, we characterized the function and structure of spinal circuitry in...
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SubjectTerms	Age Factors Amyotrophic lateral sclerosis Animals Animals, Newborn Disease Models, Animal Mice Mice, Transgenic Motor Neurons - pathology Motor Neurons - physiology Muscular Atrophy, Spinal - genetics Muscular Atrophy, Spinal - pathology Muscular Atrophy, Spinal - physiopathology Muscular system Rodents Sensory Receptor Cells - pathology Sensory Receptor Cells - physiology Spinal cord Studies Synapses - pathology Synapses - physiology
Title	Early Functional Impairment of Sensory-Motor Connectivity in a Mouse Model of Spinal Muscular Atrophy
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