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The Effect Of Peripheral Nerve Injury On Disease Progression In The SOD1(G93A) Mouse Model Of Amyotrophic Lateral Sclerosis

P. S. Sharp, J. Dick, L. Greensmith
Published 2005 · Medicine

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Around 20% of familial cases of amyotrophic lateral sclerosis have been shown to carry mutations in Cu/Zn superoxide dismutase 1 (Cu/Zn SOD1). Transgenic mice over-expressing human mutant SOD1 genes have been developed and in this study we examined the effect of nerve injury on disease progression in these mice. Firstly, disease progression in uninjured mice was characterised using physiological methods. Muscle force, contractile characteristics and motor unit survival was established at 90 days, an early symptomatic stage and also at the end-stage of the disease, at 130 days. In addition, muscle histochemistry was examined and the extent of motoneuron survival established morphologically. By 90 days of age, there is a significant reduction in muscle force, and nearly 40% of motoneurons within the sciatic motor pool have already died. By 130 days, the muscles are significantly weaker, and there is a dramatic change in the phenotype of extensor digitorum longus (EDL), which changes from a fast fatigable muscle, to a fatigue resistant muscle with a high oxidative capacity. By this stage of the disease, only 40% of motor units in EDL survive, with only 29% of motoneurons surviving within the sciatic motor pool. Following injury to the sciatic nerve in SOD1(G93A) mice, there is an acceleration in disease progression so that 90 day old mice show deficits that are only seen at the end stage in uninjured SOD1(G93A) mice. It is therefore possible that mutant SOD1 toxicity increases the vulnerability of motoneurons and muscles to stressful stimuli such as nerve injury.
This paper references
10.1046/j.1460-9568.1999.00640.x
Overexpression of GAP‐43 induces prolonged sprouting and causes death of adult motoneurons
D. I. Harding (1999)
10.1113/jphysiol.1960.sp006395
Interactions between motoneurones and muscles in respect of the characteristic speeds of their responses
A. Buller (1960)
10.1016/S0306-4522(99)00512-6
Repeated stimuli for axonal growth causes motoneuron death in adult rats: the effect of botulinum toxin followed by partial denervation
C. M. White (1999)
10.1038/362059a0
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
D. R. Rosen (1993)
10.1097/00001756-199709080-00012
Quantitative motor assessment in FALS mice: a longitudinal study
P. Barnéoud (1997)
10.1016/S0969-9961(03)00041-X
Skeletal muscle properties in a transgenic mouse model for amyotrophic lateral sclerosis: effects of creatine treatment
W. Derave (2003)
10.1016/0896-6273(95)90259-7
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
P. Wong (1995)
10.1038/nm1021
Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice
D. Kieran (2004)
10.1016/S0896-6273(00)80272-X
ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions
L. Bruijn (1997)
10.1038/6568
Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis
P. Klivényi (1999)
10.1097/00001756-199605310-00021
Neuromuscular function impairment is not caused by motor neurone loss in FALS mice: an electromyographic study.
P. Kennel (1996)
10.1016/S0306-4522(02)00448-7
Altered reaction of facial motoneurons to axonal damage in the presymptomatic phase of a murine model of familial amyotrophic lateral sclerosis
R. Mariotti (2002)
10.1113/jphysiol.1982.sp014364
Recovery of slow and fast muscles following nerve injury during early post‐natal development in the rat.
M. Lowrie (1982)
10.1073/PNAS.220417997
Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.
J. A. Johnston (2000)
10.1126/SCIENCE.8209258
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation.
M. Gurney (1994)
10.1152/JN.00665.2003
Hyperexcitability of cultured spinal motoneurons from presymptomatic ALS mice.
J. J. Kuo (2004)
10.1136/JCP.11.3.285-B
Handbook of Histopathological Technique
W. Park (1958)
10.1016/S0304-3940(00)01034-X
Activation and response to axotomy of microglia in the facial motor nuclei of G93A superoxide dismutase transgenic mice
R. Mariotti (2000)
10.1073/PNAS.0402026101
Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model.
L. Dupuis (2004)
10.1177/5.4.420
CYTOCHEMICAL DEMONSTRATION OF SUCCINIC DEHYDROGENASE BY THE USE OF A NEW p-NITROPHENYL SUBSTITUTED DITETRAZOLE
MARVIN M. Nachlas (1957)
Motor localization and the effects of nerve injury on the ventral horn cells of the spinal cord.
Romanes Gj (1946)
Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis.
M. D. Dal Canto (1994)
10.1146/ANNUREV.BI.64.070195.000525
Superoxide radical and superoxide dismutases.
I. Fridovich (1995)
10.1002/(SICI)1096-9861(19990920)412:2<373::AID-CNE13>3.0.CO;2-N
Peripheral axotomy slows motoneuron degeneration in a transgenic mouse line expressing mutant SOD1 G93A
J. Kong (1999)
10.1126/SCIENCE.1083129
Mutations in Dynein Link Motor Neuron Degeneration to Defects in Retrograde Transport
M. Hafezparast (2003)
10.1073/PNAS.92.3.689
Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis.
M. E. Ripps (1995)
10.1080/14660820050515377
Current status of SOD1 mutations in familial amyotrophic lateral sclerosis.
M. Gaudette (2000)
10.1007/BF00587391
Effects of long-term electrical stimulation on some contractile and metabolic characteristics of fast rabbit muscles
D. Pette (2004)
10.1212/WNL.53.6.1239
Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy
J. Shefner (1999)
10.1523/JNEUROSCI.18-09-03241.1998
Massive Mitochondrial Degeneration in Motor Neurons Triggers the Onset of Amyotrophic Lateral Sclerosis in Mice Expressing a Mutant SOD1
J. Kong (1998)
10.1113/jphysiol.1969.sp008771
The influence of activity on some contractile characteristics of mammalian fast and slow muscles
S. Salmons (1969)
10.1097/01.wnr.0000073685.00308.89
Assessing disease onset and progression in the SOD1 mouse model of ALS
P. Weydt (2003)
10.1006/mcne.1995.1027
Age-Dependent Penetrance of Disease in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis
A. Y. Chiu (1995)
10.1002/AJA.1001100203
Histochemical classification of individual skeletal muscle fibers of the rat.
J. Stein (1962)
10.1098/RSTB.1981.0082
The localization of motoneurons supplying the hindlimb muscles of the mouse.
S. Mchanwell (1981)
10.1113/jphysiol.1984.sp015270
Differential effect of nerve injury at birth on the activity pattern of reinnervated slow and fast muscles of the rat.
R. Navarrete (1984)
10.1111/J.1471-4159.1993.TB07478.X
Superoxide Dismutase Activity, Oxidative Damage, and Mitochondrial Energy Metabolism in Familial and Sporadic Amyotrophic Lateral Sclerosis
A. Bowling (1993)
10.1073/PNAS.93.11.5472
The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease.
Z. Mourelatos (1996)
10.1073/PNAS.93.7.3155
Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.
P. Tu (1996)
10.1016/0165-3806(87)90086-1
Permanent changes in muscle and motoneurones induced by nerve injury during a critical period of development of the rat.
M. Lowrie (1987)
10.1126/SCIENCE.1086137
Retrograde Viral Delivery of IGF-1 Prolongs Survival in a Mouse ALS Model
B. Kaspar (2003)
10.1038/35097565
From charcot to lou gehrig: deciphering selective motor neuron death in als
D. Cleveland (2001)
10.1126/SCIENCE.8351519
Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase.
H. X. Deng (1993)
10.1002/(SICI)1097-4598(199701)20:1<45::AID-MUS6>3.0.CO;2-H
Progressive motor neuron impairment in an animal model of familial amyotrophic lateral sclerosis
M. Azzouz (1997)



This paper is referenced by
Rôle de la stéaroyl-coenzyme A désaturase 1, une enzyme de synthèse des acides gras mono-insaturés, dans un modèle transgénique d’étude de la Sclérose Latérale Amyotrophique
F. Schmitt (2013)
10.1016/j.expneurol.2008.10.014
Mechano-growth factor, an IGF-I splice variant, rescues motoneurons and improves muscle function in SOD1G93A mice
J. Riddoch-Contreras (2009)
10.1038/mt.2010.213
Physiological characterization of muscle strength with variable levels of dystrophin restoration in mdx mice following local antisense therapy.
P. S. Sharp (2011)
10.3109/17482968.2012.660953
Treatment with a coinducer of the heat shock response delays muscle denervation in the SOD1-G93A mouse model of amyotrophic lateral sclerosis
B. Kalmar (2012)
10.1093/hmg/dds215
Amyloid precursor protein (APP) contributes to pathology in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis.
J. B. Bryson (2012)
10.1007/s10072-011-0918-4
Clinically definite ALS presenting weeks after mild electric injury: causality or coincidence?
A. Alajmi (2011)
10.1016/j.nbd.2018.12.020
Mutant SOD1 prevents normal functional recovery through enhanced glial activation and loss of motor neuron innervation after peripheral nerve injury
Sarah Schram (2019)
Characterisation of human and mouse SOD1-ALS proteins in vivo and in vitro
R. Saccon (2015)
10.1002/9780470942390.mo110155
In Vivo Assessment of Mouse Hindlimb Muscle Force, Contractile, and Fatigue Characteristics, and Motor Unit Number
B. Kalmar (2012)
10.1371/journal.pone.0040998
Plasma Neurofilament Heavy Chain Levels Correlate to Markers of Late Stage Disease Progression and Treatment Response in SOD1G93A Mice that Model ALS
C. Lu (2012)
Strategies to prevent motoneuron degeneration in models of Amyotrophic Lateral Sclerosis.
L. G. Bilsland (2006)
10.1073/pnas.0707906105
Deletion of the BH3-only protein puma protects motoneurons from ER stress-induced apoptosis and delays motoneuron loss in ALS mice
D. Kieran (2007)
10.1007/s13273-010-0048-8
Identification of atrophy-related proteins produced in response to cast immobilization in rat gastrocnemius muscle
J. Kim (2010)
10.1093/hmg/ddp539
The artificial gene Jazz, a transcriptional regulator of utrophin, corrects the dystrophic pathology in mdx mice.
M. D. Di Certo (2010)
10.1093/hmg/ddv439
Amyotrophic lateral sclerosis and denervation alter sphingolipids and up-regulate glucosylceramide synthase
A. Henriques (2015)
10.1242/dmm.042424
Deterioration of muscle force and contractile characteristics are early pathological events in spinal and bulbar muscular atrophy mice
A. Gray (2020)
10.1016/j.nbd.2010.03.021
Peripheral hyperstimulation alters site of disease onset and course in SOD1 rats
A. Lepore (2010)
The Bioenergetic Engine : Muscle Metabolic Properties and Hypermetabolism in ALS
Alberto Ferri (2017)
10.1016/j.nbd.2020.105052
Muscle fiber-type specific terminal Schwann cell pathology leads to sprouting deficits following partial denervation in SOD1G93A mice
J. Harrison (2020)
10.1007/978-3-030-24285-5_11
Protective Role of Glial Heat Shock Proteins in Amyotrophic Lateral Sclerosis
Benjamin E. Clarke (2019)
10.1111/j.1471-4159.2008.05595.x
Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1G93A mouse model of ALS
B. Kalmar (2008)
10.1242/dmm.002527
An ENU-induced mutation in mouse glycyl-tRNA synthetase (GARS) causes peripheral sensory and motor phenotypes creating a model of Charcot-Marie-Tooth type 2D peripheral neuropathy
F. Achilli (2009)
Role of stearoyl-CoA desaturase-1 in maintaining muscle activity : study of a lesion model for understanding the meatbolic alterations characteristics of amyotrophic lateral sclerosis
G. Hussain (2013)
10.1016/j.nbd.2007.12.002
Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression
P. S. Sharp (2008)
10.1523/JNEUROSCI.1748-18.2020
Properties of Glial Cell at the Neuromuscular Junction Are Incompatible with Synaptic Repair in the SOD1G37R ALS Mouse Model
Éric Martineau (2020)
10.1186/1471-2164-11-633
The human G93A-SOD1 mutation in a pre-symptomatic rat model of amyotrophic lateral sclerosis increases the vulnerability to a mild spinal cord compression
Nataša Jokić (2010)
10.1016/J.NBD.2006.05.009
Myostatin inhibition slows muscle atrophy in rodent models of amyotrophic lateral sclerosis
Erika L F Holzbaur (2006)
10.1111/bpa.12350
The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis
J. Loeffler (2016)
10.1038/nn.2297
A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice
Smita Saxena (2009)
10.1186/1750-1326-7-6
Spinal cord trauma and the molecular point of no return
P. Yip (2011)
10.1017/S0317167100016516
Why is ALS so Difficult to Treat?
J. Turnbull (2014)
Analyse de la composition en acides gras des lipides dans la sclérose latérale amyotrophique : implications dans le processus pathologique
L. Robelin (2016)
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