Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies

Sedehizade F, Klocke R, Jockusch H (1997)
Muscle & Nerve 20(2): 186-194.

Zeitschriftenaufsatz | Veröffentlicht | Englisch
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Sedehizade, F; Klocke, R; Jockusch, HaraldUniBi
Abstract / Bemerkung
The expression of the genes for the alpha-subunit of AChR (AChR alpha), for the myogenic factors myogenin and MyoD, for the calcium-binding protein parvalbumin (PV), and for the muscular chloride channel CIC-1 was studied in the three mouse spinal muscular atrophies (SMAs). These were the mutants ''wobbler'' (WR), ''muscle deficient'' (MDF) and ''progressive motor neuronopathy'' (PMN). Murine myopathies ''muscular dystrophy with myositis'' (MDM) and ''X-linked muscular dystrophy'' (MDX) were used as controls. AChR alpha and myogenin mRNA levels were strongly elevated in muscles affected by SMAs (reflecting denervation), whereas only myogenin mRNA was moderately elevated in MDX and MDM muscles, probably due to fiber regeneration, As in denervated muscle, CIC-1 and PV mRNA levels were lowered in SMAs, No changes were seen in muscles of up to 222-day-old symptomless ciliary neurotrophic factor (CNTF) knockout mice. The patterns of gene expression were characteristic for the type of muscle disease, indicating their possible usefulness for clinical diagnosis. (C) 1997 John Wiley & Sons, Inc.
neuromuscular diseases; mouse models; myogenic factors; acetylcholine; receptor; AChR alpha; chloride channel 1; parvalbumin; denervation; effects
Muscle & Nerve
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Sedehizade F, Klocke R, Jockusch H. Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies. Muscle & Nerve. 1997;20(2):186-194.
Sedehizade, F., Klocke, R., & Jockusch, H. (1997). Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies. Muscle & Nerve, 20(2), 186-194.<186::AID-MUS8>3.0.CO;2-8
Sedehizade, F, Klocke, R, and Jockusch, Harald. 1997. “Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies”. Muscle & Nerve 20 (2): 186-194.
Sedehizade, F., Klocke, R., and Jockusch, H. (1997). Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies. Muscle & Nerve 20, 186-194.
Sedehizade, F., Klocke, R., & Jockusch, H., 1997. Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies. Muscle & Nerve, 20(2), p 186-194.
F. Sedehizade, R. Klocke, and H. Jockusch, “Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies”, Muscle & Nerve, vol. 20, 1997, pp. 186-194.
Sedehizade, F., Klocke, R., Jockusch, H.: Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies. Muscle & Nerve. 20, 186-194 (1997).
Sedehizade, F, Klocke, R, and Jockusch, Harald. “Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies”. Muscle & Nerve 20.2 (1997): 186-194.

17 Zitationen in Europe PMC

Daten bereitgestellt von Europe PubMed Central.

Intricate effects of primary motor neuronopathy on contractile proteins and metabolic muscle enzymes as revealed by label-free mass spectrometry.
Holland A, Schmitt-John T, Dowling P, Meleady P, Henry M, Clynes M, Ohlendieck K., Biosci Rep 34(4), 2014
PMID: 24895011
Neuro-muscular function in the wobbler murine model of primary motor neuronopathy.
Broch-Lips M, Pedersen TH, Riisager A, Schmitt-John T, Nielsen OB., Exp Neurol 248(), 2013
PMID: 23872513
In vivo characterization of the role of tissue-specific translation elongation factor 1A2 in protein synthesis reveals insights into muscle atrophy.
Doig J, Griffiths LA, Peberdy D, Dharmasaroja P, Vera M, Davies FJ, Newbery HJ, Brownstein D, Abbott CM., FEBS J 280(24), 2013
PMID: 24460877
Myogenic differentiation factor 1 and myogenin expression not elevated in regenerated masticatory muscles of dystrophic (mdx) mice.
Spassov A, Gredes T, Lehmann C, Gedrange T, Lucke S, Pavlovic D, Kunert-Keil C., J Orofac Orthop 72(6), 2011
PMID: 22124510
Recovery of function in a myogenic mouse model of spinal bulbar muscular atrophy.
Johansen JA, Yu Z, Mo K, Monks DA, Lieberman AP, Breedlove SM, Jordan CL., Neurobiol Dis 34(1), 2009
PMID: 19211034
Specific isomyosin proportions in hyperexcitable and physiologically denervated mouse muscle.
Agbulut O, Noirez P, Butler-Browne G, Jockusch H., FEBS Lett 561(1-3), 2004
PMID: 15013776
The wobbler mouse: a neurodegeneration jigsaw puzzle.
Boillée S, Peschanski M, Junier MP., Mol Neurobiol 28(1), 2003
PMID: 14514986
Motoneuron morphological alterations before and after the onset of the disease in the wobbler mouse.
Blondet B, Carpentier G, Aït-Ikhlef A, Murawsky M, Rieger F., Brain Res 930(1-2), 2002
PMID: 11879795
Genetic modifiers that aggravate the neurological phenotype of the wobbler mouse.
Ulbrich M, Schmidt VC, Ronsiek M, Mussmann A, Bartsch JW, Augustin M, Jockusch H, Schmitt-John T., Neuroreport 13(4), 2002
PMID: 11930176
Progressive loss of striatal neurons causes motor dysfunction in MND2 mutant mice and is not prevented by Bcl-2.
Rathke-Hartlieb S, Schlomann U, Heimann P, Meisler MH, Jockusch H, Bartsch JW., Exp Neurol 175(1), 2002
PMID: 12009762
Motor neuron cell death in wobbler mutant mice follows overexpression of the G-protein-coupled, protease-activated receptor for thrombin.
Festoff BW, D'Andrea MR, Citron BA, Salcedo RM, Smirnova IV, Andrade-Gordon P., Mol Med 6(5), 2000
PMID: 10952021
Elevated expression of membrane type 1 metalloproteinase (MT1-MMP) in reactive astrocytes following neurodegeneration in mouse central nervous system.
Rathke-Hartlieb S, Budde P, Ewert S, Schlomann U, Staege MS, Jockusch H, Bartsch JW, Frey J., FEBS Lett 481(3), 2000
PMID: 11007969
Dystonin-deficient mice exhibit an intrinsic muscle weakness and an instability of skeletal muscle cytoarchitecture.
Dalpé G, Mathieu M, Comtois A, Zhu E, Wasiak S, De Repentigny Y, Leclerc N, Kothary R., Dev Biol 210(2), 1999
PMID: 10357897
Spatiotemporal progression of neurodegeneration and glia activation in the wobbler neuropathy of the mouse.
Rathke-Hartlieb S, Schmidt VC, Jockusch H, Schmitt-John T, Bartsch JW., Neuroreport 10(16), 1999
PMID: 10599854

52 References

Daten bereitgestellt von Europe PubMed Central.

Skeletal muscle regeneration.
Allbrook D., Muscle Nerve 4(3), 1981
PMID: 7017402
The mouse mutation muscle deficient (mdf) is characterized by a progressive motoneuron disease.
Blot S, Poirier C, Dreyfus PA., J. Neuropathol. Exp. Neurol. 54(6), 1995
PMID: 7595654
The mouse mutation progressive motor neuronopathy (pmn) maps to chromosome 13.
Brunialti AL, Poirier C, Schmalbruch H, Guenet JL., Genomics 29(1), 1995
PMID: 8530062
Muscle differentiation. Which myogenic factors make muscle?
Buckingham M., Curr. Biol. 4(1), 1994
PMID: 7922315
X chromosome-linked muscular dystrophy (mdx) in the mouse.
Bulfield G, Siller WG, Wight PA, Moore KJ., Proc. Natl. Acad. Sci. U.S.A. 81(4), 1984
PMID: 6583703
Expression of a single transfected cDNA converts fibroblasts to myoblasts.
Davis RL, Weintraub H, Lassar AB., Cell 51(6), 1987
PMID: 3690668
Myogenin and MyoD join a family of skeletal muscle genes regulated by electrical activity.
Eftimie R, Brenner HR, Buonanno A., Proc. Natl. Acad. Sci. U.S.A. 88(4), 1991
PMID: 1705035
Muscle acetylcholine receptor biosynthesis. Regulation by transcript availability.
Evans S, Goldman D, Heinemann S, Patrick J., J. Biol. Chem. 262(10), 1987
PMID: 2435720
Developmental control of the excitability of muscle: transplantation experiments on a myotonic mouse mutant.
Fuchtbauer EM, Reininghaus J, Jockusch H., Proc. Natl. Acad. Sci. U.S.A. 85(11), 1988
PMID: 3375245
Correlation of parvalbumin concentration with relaxation speed in mammalian muscles.
Heizmann CW, Berchtold MW, Rowlerson AM., Proc. Natl. Acad. Sci. U.S.A. 79(23), 1982
PMID: 6961404
Rapid inhibition of myogenin-driven acetylcholine receptor subunit gene transcription.
Huang CF, Lee YS, Schmidt MM, Schmidt J., EMBO J. 13(3), 1994
PMID: 8313908
Selective accumulation of MyoD and myogenin mRNAs in fast and slow adult skeletal muscle is controlled by innervation and hormones.
Hughes SM, Taylor JM, Tapscott SJ, Gurley CM, Carter WJ, Peterson CA., Development 118(4), 1993
PMID: 8269844
Nucleotide sequence of the mouse muscle nicotinic acetylcholine receptor alpha subunit.
Isenberg KE, Mudd J, Shah V, Merlie JP., Nucleic Acids Res. 14(12), 1986
PMID: 3755237

Jockusch, J Neurol Sci 98(), 1990

Jockusch, 1990
Exploring the mammalian neuromuscular system by analysis of mutations: spinal muscular atrophy and myotonia.
Jockusch H, Kaupmann K, Gronemeier M, Schleef M, Klocke R., Prog. Neurobiol. 42(2), 1994
PMID: 8008830

Jockusch, Genet Res Camb 52(), 1988
Wobbler, a mutation affecting motoneuron survival and gonadal functions in the mouse, maps to proximal chromosome 11.
Kaupmann K, Simon-Chazottes D, Guenet JL, Jockusch H., Genomics 13(1), 1992
PMID: 1349581

Klocke, Muscle Res Cell Motil 16(), 1995
Opposite regulation of the mRNAs for parvalbumin and p19/6.8 in myotonic mouse muscle.
Kluxen FW, Schoffl F, Berchtold MW, Jockusch H., Eur. J. Biochem. 176(1), 1988
PMID: 3138114
The skeletal muscle chloride channel in dominant and recessive human myotonia.
Koch MC, Steinmeyer K, Lorenz C, Ricker K, Wolf F, Otto M, Zoll B, Lehmann-Horn F, Grzeschik KH, Jentsch TJ., Science 257(5071), 1992
PMID: 1379744

Lane, Mouse News Lett 73(), 1985
Neural regulation of parvalbumin expression in mammalian skeletal muscle.
Leberer E, Pette D., Biochem. J. 235(1), 1986
PMID: 3741391
Identification and characterization of a spinal muscular atrophy-determining gene.
Lefebvre S, Burglen L, Reboullet S, Clermont O, Burlet P, Viollet L, Benichou B, Cruaud C, Millasseau P, Zeviani M., Cell 80(1), 1995
PMID: 7813012
Genes for SMA: multum in parvo.
Lewin B., Cell 80(1), 1995
PMID: 7813005
Disruption of the CNTF gene results in motor neuron degeneration.
Masu Y, Wolf E, Holtmann B, Sendtner M, Brem G, Thoenen H., Nature 365(6441), 1993
PMID: 8361533
The myotonic mouse mutant ADR: electrophysiology of the muscle fiber.
Mehrke G, Brinkmeier H, Jockusch H., Muscle Nerve 11(5), 1988
PMID: 2453798
Neural control of phenotypic expression in mammalian muscle fibers.
Pette D, Vrbova G., Muscle Nerve 8(8), 1985
PMID: 3903491
Two adjacent MyoD1-binding sites regulate expression of the acetylcholine receptor alpha-subunit gene.
Piette J, Bessereau JL, Huchet M, Changeux JP., Nature 345(6273), 1990
PMID: 2342565
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis.
Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, Donaldson D, Goto J, O'Regan JP, Deng HX., Nature 362(6415), 1993
PMID: 8446170
The gene for neuronal apoptosis inhibitory protein is partially deleted in individuals with spinal muscular atrophy.
Roy N, Mahadevan MS, McLean M, Shutler G, Yaraghi Z, Farahani R, Baird S, Besner-Johnston A, Lefebvre C, Kang X., Cell 80(1), 1995
PMID: 7813013
A new mouse mutant with progressive motor neuronopathy.
Schmalbruch H, Jensen HJ, Bjaerg M, Kamieniecka Z, Kurland L., J. Neuropathol. Exp. Neurol. 50(3), 1991
PMID: 2022963
The molecular basis of muscular dystrophy in the mdx mouse: a point mutation.
Sicinski P, Geng Y, Ryder-Cook AS, Barnard EA, Darlison MG, Barnard PJ., Science 244(4912), 1989
PMID: 2662404
Inactivation of muscle chloride channel by transposon insertion in myotonic mice.
Steinmeyer K, Klocke R, Ortland C, Gronemeier M, Jockusch H, Grunder S, Jentsch TJ., Nature 354(6351), 1991
PMID: 1659665
Development of electrical myotonia in the ADR mouse: role of chloride conductance in myotubes and neonatal animals.
Wischmeyer E, Nolte E, Klocke R, Jockusch H, Brinkmeier H., Neuromuscul. Disord. 3(4), 1993
PMID: 8268723
Neural factors regulate AChR subunit mRNAs at rat neuromuscular synapses.
Witzemann V, Brenner HR, Sakmann B., J. Cell Biol. 114(1), 1991
PMID: 1646821

Womack, J Hered 71(), 1980

Z�hlke, Pva. Genet Res Camb 54(), 1989

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