Grm7

Summary

Gene Symbol: Grm7
Description: glutamate receptor, metabotropic 7
Alias: 6330570A01Rik, BB176677, C030018L03, E130018M02Rik, Gpr1g, Gprc1g, SMN2, Tg(SMN2)89Ahmb, mGluR7, metabotropic glutamate receptor 7, G protein-coupled receptor GRM7, G protein-coupled receptor, family C, group 1, member G, mGlu7a receptor
Species: mouse
Products:     Grm7

Top Publications

  1. Zhang C, Bertaso F, Eulenburg V, Lerner Natoli M, Herin G, Bauer L, et al. Knock-in mice lacking the PDZ-ligand motif of mGluR7a show impaired PKC-dependent autoinhibition of glutamate release, spatial working memory deficits, and increased susceptibility to pentylenetetrazol. J Neurosci. 2008;28:8604-14 pubmed publisher
    The metabotropic glutamate receptor 7 (mGluR7) is widely expressed throughout the brain and primarily localized at presynaptic active zones, where it is thought to regulate neurotransmitter release...
  2. Gogliotti R, Lutz C, Jorgensen M, Huebsch K, Koh S, DiDonato C. Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice. Neurobiol Dis. 2011;43:142-51 pubmed publisher
    ..we clone the genomic integration site of the transgene and demonstrate it to be in intron 4 of the metabotropic glutamate receptor 7 (mGluR7) gene...
  3. Turner B, Parkinson N, Davies K, Talbot K. Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model. Neurobiol Dis. 2009;34:511-7 pubmed publisher
    ..Genetic association studies provide compelling evidence that SMN1 and SMN2 genotypes encoding lower SMN protein levels are implicated in sporadic ALS, suggesting that SMN expression is a ..
  4. Bevan A, Hutchinson K, Foust K, Braun L, McGovern V, Schmelzer L, et al. Early heart failure in the SMNDelta7 model of spinal muscular atrophy and correction by postnatal scAAV9-SMN delivery. Hum Mol Genet. 2010;19:3895-905 pubmed publisher
    ..Therefore, we have characterized left ventricular (LV) function of SMA mice (SMN2+/+; SMN?7+/+; Smn-/-) and compared it with that of their unaffected littermates at 7 and 14 days of age...
  5. Bäumer D, Lee S, Nicholson G, Davies J, Parkinson N, Murray L, et al. Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy. PLoS Genet. 2009;5:e1000773 pubmed publisher
  6. Bowerman M, Beauvais A, Anderson C, Kothary R. Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model. Hum Mol Genet. 2010;19:1468-78 pubmed publisher
    ..Our study presents evidence linking disruption of actin cytoskeletal dynamics to SMA pathogenesis and, for the first time, identifies RhoA effectors as viable targets for therapeutic intervention in the disease. ..
  7. Murray L, Comley L, Thomson D, Parkinson N, Talbot K, Gillingwater T. Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy. Hum Mol Genet. 2008;17:949-62 pubmed
    ..Here we have undertaken a detailed morphological investigation of neuromuscular synaptic pathology in the Smn-/-;SMN2 and Smn-/-;SMN2;Delta7 mouse models of SMA...
  8. Lee A, Awano T, Park G, Monani U. Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy. PLoS ONE. 2012;7:e46353 pubmed publisher
  9. Torres Benito L, Neher M, Cano R, Ruiz R, Tabares L. SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals. PLoS ONE. 2011;6:e26164 pubmed publisher
    ..We propose that SMN is essential for the normal postnatal maturation of motor nerve terminals and that SMN deficiency disrupts the presynaptic organization leading to neurodegeneration. ..

More Information

Publications81

  1. Porensky P, Mitrpant C, McGovern V, Bevan A, Foust K, Kaspar B, et al. A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse. Hum Mol Genet. 2012;21:1625-38 pubmed publisher
    ..SMA results from deletion or mutation of the Survival Motor Neuron 1 gene (SMN1) and retention of SMN2. A single nucleotide difference between SMN1 and SMN2 results in exclusion of exon 7 from the majority of SMN2 ..
  2. Lutz C, Kariya S, Patruni S, Osborne M, Liu D, Henderson C, et al. Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy. J Clin Invest. 2011;121:3029-41 pubmed publisher
    ..Nevertheless, our results suggest that even in severe SMA, timely reinstatement of the SMN protein may halt the progression of the disease and serve as an effective postsymptomatic treatment. ..
  3. Zhang H, Robinson N, Wu C, Wang W, Harrington M. Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo development. PLoS ONE. 2010;5:e11696 pubmed publisher
  4. Gogliotti R, Quinlan K, Barlow C, Heier C, Heckman C, DiDonato C. Motor neuron rescue in spinal muscular atrophy mice demonstrates that sensory-motor defects are a consequence, not a cause, of motor neuron dysfunction. J Neurosci. 2012;32:3818-29 pubmed publisher
    ..Collectively, this work demonstrates that the SMA phenotype autonomously originates in MNs and that sensory-motor synapse loss is a consequence, not a cause, of MN dysfunction. ..
  5. Jablonka S, Karle K, Sandner B, Andreassi C, von Au K, Sendtner M. Distinct and overlapping alterations in motor and sensory neurons in a mouse model of spinal muscular atrophy. Hum Mol Genet. 2006;15:511-8 pubmed
    ..We have analysed dorsal root ganglionic sensory neurons in Smn-/-;SMN2 mice, a model of type I SMA...
  6. Ling K, Lin M, Zingg B, Feng Z, Ko C. Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophy. PLoS ONE. 2010;5:e15457 pubmed publisher
    ..Together, our results present a novel concept that synaptic defects occur at multiple levels of the spinal and neuromuscular circuitry in SMN?7 mice, and that proprioceptive spinal synapses could be a potential target for SMA therapy. ..
  7. McGovern V, Gavrilina T, Beattie C, Burghes A. Embryonic motor axon development in the severe SMA mouse. Hum Mol Genet. 2008;17:2900-9 pubmed publisher
    ..This indicates that in severe SMA mice there are no defects in motor axon formation however, we find evidence of denervation in embryogenesis. ..
  8. Kong L, Wang X, Choe D, Polley M, Burnett B, Bosch Marce M, et al. Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice. J Neurosci. 2009;29:842-51 pubmed publisher
    ..Together these results indicate that impaired synaptic vesicle release at NMJs in severe SMA is likely to contribute to failed postnatal maturation of motor units and muscle weakness. ..
  9. Monani U, Sendtner M, Coovert D, Parsons D, Andreassi C, Le T, et al. The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophy. Hum Mol Genet. 2000;9:333-9 pubmed
    ..motor neuron gene ( SMN1 ), but patients retain at least one copy of a highly homologous gene, centromeric SMN ( SMN2 ). Mice possess only one survival motor neuron gene ( Smn ) whose loss is embryonic lethal...
  10. Lee Y, Mikesh M, Smith I, Rimer M, Thompson W. Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons. Dev Biol. 2011;356:432-44 pubmed publisher
    ..Thus, if SMA in this particular mouse is a disease of motor neurons, it can act in a manner that does not result in their death or disconnection from their targets but nonetheless alters many aspects of neuromuscular development. ..
  11. Sleigh J, Gillingwater T, Talbot K. The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy. Dis Model Mech. 2011;4:457-67 pubmed publisher
    ..either through direct viral delivery or by enhancing full-length SMN protein expression from the SMN1 paralog, SMN2, are approaching the translational stage of development...
  12. Kariya S, Park G, Maeno Hikichi Y, Leykekhman O, Lutz C, Arkovitz M, et al. Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy. Hum Mol Genet. 2008;17:2552-69 pubmed publisher
    ..Studies involving SMA patients and animal models expressing the human SMN2 gene have yielded relatively little information about the earliest cellular consequences of reduced SMN protein...
  13. Jablonka S, Beck M, Lechner B, Mayer C, Sendtner M. Defective Ca2+ channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophy. J Cell Biol. 2007;179:139-49 pubmed
    ..Stimulation of Smn production from the transgenic SMN2 gene by cyclic adenosine monophosphate restores Cav2.2 accumulation and excitability...
  14. Workman E, Saieva L, Carrel T, Crawford T, Liu D, Lutz C, et al. A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice. Hum Mol Genet. 2009;18:2215-29 pubmed publisher
    ..Loss of the survival motor neuron (SMN1) gene, in the presence of the SMN2 gene causes SMA...
  15. Mutsaers C, Wishart T, Lamont D, Riessland M, Schreml J, Comley L, et al. Reversible molecular pathology of skeletal muscle in spinal muscular atrophy. Hum Mol Genet. 2011;20:4334-44 pubmed publisher
    ..We conclude that intrinsic pathology of skeletal muscle is an important and reversible event in SMA and also suggest that muscle proteins have the potential to act as novel biomarkers in SMA. ..
  16. Ling K, Gibbs R, Feng Z, Ko C. Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy. Hum Mol Genet. 2012;21:185-95 pubmed publisher
    ..Our findings suggest that neuromuscular denervation in vulnerable muscles is a widespread pathology in SMA, and can serve as a preparation for elucidating the biological basis of synapse loss, and for evaluating therapeutic efficacy. ..
  17. Gavrilina T, McGovern V, Workman E, Crawford T, Gogliotti R, DiDonato C, et al. Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect. Hum Mol Genet. 2008;17:1063-75 pubmed publisher
    Spinal muscular atrophy (SMA) is caused by loss of the survival motor neuron gene (SMN1) and retention of the SMN2 gene. The copy number of SMN2 affects the amount of SMN protein produced and the severity of the SMA phenotype...
  18. Le T, Pham L, Butchbach M, Zhang H, Monani U, Coovert D, et al. SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN. Hum Mol Genet. 2005;14:845-57 pubmed
    ..SMN is encoded by two genes, SMN1 and SMN2, which essentially differ by a single nucleotide in exon 7...
  19. Dominguez E, Marais T, Chatauret N, Benkhelifa Ziyyat S, Duque S, Ravassard P, et al. Intravenous scAAV9 delivery of a codon-optimized SMN1 sequence rescues SMA mice. Hum Mol Genet. 2011;20:681-93 pubmed publisher
    ..This study reports the most efficient rescue of SMA mice to date after a single intravenous injection of an optimized SMN-encoding scAAV9, highlighting the considerable potential of this method for the treatment of human SMA. ..
  20. Butchbach M, Edwards J, Burghes A. Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy. Neurobiol Dis. 2007;27:207-19 pubmed
    ..In summary, we have demonstrated an impairment of neonatal motor responses in SMNDelta7 SMA mice. This phenotype characterization could be used to assess the effectiveness of potential therapies for SMA. ..
  21. Avila A, Burnett B, Taye A, Gabanella F, Knight M, Hartenstein P, et al. Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy. J Clin Invest. 2007;117:659-71 pubmed
    ..SMA) is caused by mutation of the telomeric survival motor neuron 1 (SMN1) gene with retention of the centromeric SMN2 gene...
  22. Park G, Maeno Hikichi Y, Awano T, Landmesser L, Monani U. Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene. J Neurosci. 2010;30:12005-19 pubmed publisher
    ..This realization is tempered by the relatively mild SMA phenotype in our model mice, one explanation for which is the presence of normal SMN levels in non-neuronal tissue that serves to modulate disease severity. ..
  23. Shababi M, Habibi J, Yang H, Vale S, Sewell W, Lorson C. Cardiac defects contribute to the pathology of spinal muscular atrophy models. Hum Mol Genet. 2010;19:4059-71 pubmed publisher
    ..Interestingly, a human-specific copy gene is present on the same region of chromosome 5q, called SMN2. Motor neurons are the primary tissue affected in SMA...
  24. Monani U, Pastore M, Gavrilina T, Jablonka S, Le T, Andreassi C, et al. A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy. J Cell Biol. 2003;160:41-52 pubmed
    ..lack a functional survival of motor neurons (SMN1) gene, but carry one or more copies of the highly homologous SMN2 gene. A homozygous knockout of the single murine Smn gene is embryonic lethal...
  25. Voigt T, Meyer K, Baum O, Schumperli D. Ultrastructural changes in diaphragm neuromuscular junctions in a severe mouse model for Spinal Muscular Atrophy and their prevention by bifunctional U7 snRNA correcting SMN2 splicing. Neuromuscul Disord. 2010;20:744-52 pubmed publisher
    ..Because of a splicing problem, the second copy gene, SMN2, generates insufficient amounts of functional SMN protein, leading to the death of spinal cord motoneurons...
  26. Ruiz R, Casañas J, Torres Benito L, Cano R, Tabares L. Altered intracellular Ca2+ homeostasis in nerve terminals of severe spinal muscular atrophy mice. J Neurosci. 2010;30:849-57 pubmed publisher
    ..Together, these results show that the reduction of SMN affects synaptic maturation, evoked release, and regulation of intraterminal Ca(2+) levels. ..
  27. Murray L, Lee S, Bäumer D, Parson S, Talbot K, Gillingwater T. Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy. Hum Mol Genet. 2010;19:420-33 pubmed publisher
    ..development of neuromuscular connectivity in differentially vulnerable motor neuron populations in Smn(-/-);SMN2 mice, a model of severe SMA...
  28. Meyer K, Marquis J, Trüb J, Nlend Nlend R, Verp S, Ruepp M, et al. Rescue of a severe mouse model for spinal muscular atrophy by U7 snRNA-mediated splicing modulation. Hum Mol Genet. 2009;18:546-55 pubmed publisher
    ..The nearly identical SMN2 gene has a silent mutation that impairs the utilization of exon 7 and the production of functional protein...
  29. Riessland M, Ackermann B, Förster A, Jakubik M, Hauke J, Garbes L, et al. SAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophy. Hum Mol Genet. 2010;19:1492-506 pubmed publisher
    ..loss of the survival motor neuron gene 1 (SMN1), whereas disease severity is mainly influenced by the number of SMN2 copies...
  30. Wishart T, Huang J, Murray L, Lamont D, Mutsaers C, Ross J, et al. SMN deficiency disrupts brain development in a mouse model of severe spinal muscular atrophy. Hum Mol Genet. 2010;19:4216-28 pubmed publisher
    ..This study reveals novel roles for SMN protein in brain development and maintenance and provides the first insights into cellular and molecular pathways disrupted in the brain in a severe form of SMA. ..
  31. Heier C, Satta R, Lutz C, DiDonato C. Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice. Hum Mol Genet. 2010;19:3906-18 pubmed publisher
    ..Together with clinical cases that report similar symptoms, this reveals a new area of investigation that will be important to address as we move SMA therapeutics towards clinical success. ..
  32. Ruggiu M, McGovern V, Lotti F, Saieva L, Li D, Kariya S, et al. A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy. Mol Cell Biol. 2012;32:126-38 pubmed publisher
    ..In the absence of SMN1, inefficient inclusion of exon 7 in transcripts from the nearly identical SMN2 gene results in ubiquitous SMN decrease but selective motor neuron degeneration...
  33. Rossoll W, Jablonka S, Andreassi C, Kröning A, Karle K, Monani U, et al. Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneurons. J Cell Biol. 2003;163:801-12 pubmed
    ..Together, these data suggest that a complex of Smn with its binding partner hnRNP R interacts with beta-actin mRNA and translocates to axons and growth cones of motoneurons. ..
  34. Valsecchi V, Boido M, De Amicis E, Piras A, Vercelli A. Expression of Muscle-Specific MiRNA 206 in the Progression of Disease in a Murine SMA Model. PLoS ONE. 2015;10:e0128560 pubmed publisher
    ..We speculate that early modulation of miRNA-206 expression might delay SMA neurodegenerative pathway and that miRNA-206 could be an innovative, still relatively unexplored, therapeutic target for SMA. ..
  35. Tang Z, El Far O, Betz H, Scheschonka A. Pias1 interaction and sumoylation of metabotropic glutamate receptor 8. J Biol Chem. 2005;280:38153-9 pubmed
    ..Our results are consistent with post-translational sumoylation providing a novel mechanism of group III mGluR regulation. ..
  36. Meier I, Walker M, Matera A. Gemin4 is an essential gene in mice, and its overexpression in human cells causes relocalization of the SMN complex to the nucleoplasm. Biol Open. 2018;7: pubmed publisher
    ..i>Gemin4 failed to modify the early postnatal mortality phenotype of SMA type I (Smn-/-;SMN2+/+ ) mice...
  37. Woo C, Maier V, Davey R, Brennan J, Li G, Brothers J, et al. Gene activation of SMN by selective disruption of lncRNA-mediated recruitment of PRC2 for the treatment of spinal muscular atrophy. Proc Natl Acad Sci U S A. 2017;114:E1509-E1518 pubmed publisher
    ..The disease severity inversely correlates with the copy number of SMN2, a duplicated gene that is nearly identical to SMN1...
  38. Pagliarini V, Pelosi L, Bustamante M, Nobili A, Berardinelli M, D Amelio M, et al. SAM68 is a physiological regulator of SMN2 splicing in spinal muscular atrophy. J Cell Biol. 2015;211:77-90 pubmed publisher
    ..The almost identical SMN2 gene is unable to compensate for this deficiency because of the skipping of exon 7 during pre-messenger RNA (mRNA) ..
  39. Wishart T, Mutsaers C, Riessland M, Reimer M, Hunter G, Hannam M, et al. Dysregulation of ubiquitin homeostasis and ?-catenin signaling promote spinal muscular atrophy. J Clin Invest. 2014;124:1821-34 pubmed publisher
  40. Alioto T, Ngai J. The repertoire of olfactory C family G protein-coupled receptors in zebrafish: candidate chemosensory receptors for amino acids. BMC Genomics. 2006;7:309 pubmed
  41. Fallini C, Donlin Asp P, Rouanet J, Bassell G, Rossoll W. Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons. J Neurosci. 2016;36:3811-20 pubmed publisher
  42. Bogdanik L, Osborne M, Davis C, Martin W, Austin A, Rigo F, et al. Systemic, postsymptomatic antisense oligonucleotide rescues motor unit maturation delay in a new mouse model for type II/III spinal muscular atrophy. Proc Natl Acad Sci U S A. 2015;112:E5863-72 pubmed publisher
    ..of the Survival Motor Neuron 1 (SMN1) gene, and prognosis inversely correlates with the number of copies of the SMN2 gene, a human-specific homolog of SMN1...
  43. Ahmad S, Wang Y, Shaik G, Burghes A, Gangwani L. The zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy. Hum Mol Genet. 2012;21:2745-58 pubmed publisher
    ..The severity of SMA is primarily influenced by the copy number of the SMN2 gene...
  44. Masugi Tokita M, Flor P, Kawata M. Metabotropic Glutamate Receptor Subtype 7 in the Bed Nucleus of the Stria Terminalis is Essential for Intermale Aggression. Neuropsychopharmacology. 2016;41:726-35 pubmed publisher
    Metabotropic glutamate receptor subtype 7 (mGluR7) is a member of group III mGluRs, which localize to the presynaptic active zones of the mammalian central nervous system...
  45. Xia W, Liu Y, Jiao J. GRM7 regulates embryonic neurogenesis via CREB and YAP. Stem Cell Reports. 2015;4:795-810 pubmed publisher
    b>Metabotropic glutamate receptor 7 (GRM7) has recently been identified to be associated with brain developmental defects, such as attention deficit hyperactivity disorder (ADHD) and autism...
  46. Bosch Marce M, Wee C, Martinez T, Lipkes C, Choe D, Kong L, et al. Increased IGF-1 in muscle modulates the phenotype of severe SMA mice. Hum Mol Genet. 2011;20:1844-53 pubmed publisher
    ..IGF-1 may warrant further investigation in mild SMA animal models and perhaps SMA patients. ..
  47. Cryan J, Kelly P, Neijt H, Sansig G, Flor P, van der Putten H. Antidepressant and anxiolytic-like effects in mice lacking the group III metabotropic glutamate receptor mGluR7. Eur J Neurosci. 2003;17:2409-17 pubmed
    ..Of all glutamate receptors, the role of group III metabotropic glutamate receptors (mGluR4, mGluR6, mGluR7, mGluR8) in such disorders is the least investigated because of the lack of specific pharmacological tools...
  48. Subramanian N, Wetzel A, Dombert B, Yadav P, Havlicek S, Jablonka S, et al. Role of Na(v)1.9 in activity-dependent axon growth in motoneurons. Hum Mol Genet. 2012;21:3655-67 pubmed publisher
    ..Na(V)1.9 activation could therefore serve as a target for modulating axonal regeneration in motoneuron diseases such as SMA in which presynaptic activity of VGCCs is reduced. ..
  49. Taylor A, Glascock J, Rose F, Lutz C, Lorson C. Restoration of SMN to Emx-1 expressing cortical neurons is not sufficient to provide benefit to a severe mouse model of Spinal Muscular Atrophy. Transgenic Res. 2013;22:1029-36 pubmed publisher
    ..However, low, but essential, levels of SMN protein are produced by a nearly identical copy gene called SMN2. Detailed analysis of neuromuscular junctions in SMA mice has revealed a selective vulnerability in a subset of ..
  50. Miller N, Feng Z, Edens B, Yang B, Shi H, Sze C, et al. Non-aggregating tau phosphorylation by cyclin-dependent kinase 5 contributes to motor neuron degeneration in spinal muscular atrophy. J Neurosci. 2015;35:6038-50 pubmed publisher
    ..Altogether, our findings suggest a novel mechanism for SMA pathogenesis in which hyperphosphorylation of non-aggregating tau by Cdk5 contributes to motor neuron degeneration. ..
  51. Hunter G, Aghamaleky Sarvestany A, Roche S, Symes R, Gillingwater T. SMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy. Hum Mol Genet. 2014;23:2235-50 pubmed publisher
  52. Rose F, Mattis V, Rindt H, Lorson C. Delivery of recombinant follistatin lessens disease severity in a mouse model of spinal muscular atrophy. Hum Mol Genet. 2009;18:997-1005 pubmed publisher
    ..Current therapeutic research focuses on modulating the expression of a partially functioning copy gene, SMN2, which is retained in SMA patients...
  53. Fendt M, Imobersteg S, Peterlik D, Chaperon F, Mattes C, Wittmann C, et al. Differential roles of mGlu(7) and mGlu(8) in amygdala-dependent behavior and physiology. Neuropharmacology. 2013;72:215-23 pubmed publisher
    ..These receptors seem promising targets for treating anxiety disorders with different underlying pathologies with exaggerated fear learning (mGlu7) or contextual fear (mGlu8). ..
  54. Dal Mas A, Rogalska M, Bussani E, Pagani F. Improvement of SMN2 pre-mRNA processing mediated by exon-specific U1 small nuclear RNA. Am J Hum Genet. 2015;96:93-103 pubmed publisher
    ..atrophy (SMA), a genetic neuromuscular disease where a silent exonic transition on the survival motor neuron 2 (SMN2) leads to exon 7 (E7) skipping...
  55. Perroy J, Gutierrez G, Coulon V, Bockaert J, Pin J, Fagni L. The C terminus of the metabotropic glutamate receptor subtypes 2 and 7 specifies the receptor signaling pathways. J Biol Chem. 2001;276:45800-5 pubmed
    ..These results show that the C terminus of mGlu2 and mGlu7 receptors is a key structural determinant that allows these receptors to select a specific signaling pathway in neurons. ..
  56. Peterlik D, Stangl C, Bludau A, Grabski D, Strasser R, Schmidt D, et al. Relief from detrimental consequences of chronic psychosocial stress in mice deficient for the metabotropic glutamate receptor subtype 7. Neuropharmacology. 2017;115:139-148 pubmed publisher
    ..This article is part of the Special Issue entitled 'Metabotropic Glutamate Receptors, 5 years on'. ..
  57. Farooq F, Abadía Molina F, MacKenzie D, Hadwen J, Shamim F, O Reilly S, et al. Celecoxib increases SMN and survival in a severe spinal muscular atrophy mouse model via p38 pathway activation. Hum Mol Genet. 2013;22:3415-24 pubmed publisher
    ..treatment strategy for SMA is to upregulate the amount of SMN protein originating from the highly homologous SMN2 gene, compensating in part for the absence of the functional SMN1 gene...
  58. Ferrero J, Bartolomé Martín D, Torres M, Sánchez Prieto J. Potentiation of mGlu7 receptor-mediated glutamate release at nerve terminals containing N and P/Q type Ca2+ channels. Neuropharmacology. 2013;67:213-22 pubmed publisher
  59. Sumner C, Wee C, Warsing L, Choe D, Ng A, Lutz C, et al. Inhibition of myostatin does not ameliorate disease features of severe spinal muscular atrophy mice. Hum Mol Genet. 2009;18:3145-52 pubmed publisher
    ..Together these results suggest that inhibition of myostatin may not be a promising therapeutic strategy in severe forms of SMA. ..
  60. Masugi M, Yokoi M, Shigemoto R, Muguruma K, Watanabe Y, Sansig G, et al. Metabotropic glutamate receptor subtype 7 ablation causes deficit in fear response and conditioned taste aversion. J Neurosci. 1999;19:955-63 pubmed
    ..We examined the fear response of freezing after electric shock in wild-type and mGluR7(-/-) knockout littermates. Wild-type mice displayed freezing immediately after and 1 d after footshock...
  61. Khairallah M, Astroski J, Custer S, Androphy E, Franklin C, Lorson C. SMN deficiency negatively impacts red pulp macrophages and spleen development in mouse models of spinal muscular atrophy. Hum Mol Genet. 2017;26:932-941 pubmed publisher
    ..Collectively, these results demonstrate that SMN deficiency impacts spleen development and suggests a potential role for immunological development in SMA. ..
  62. Friedman R, Van Laer L, Huentelman M, Sheth S, Van Eyken E, Corneveaux J, et al. GRM7 variants confer susceptibility to age-related hearing impairment. Hum Mol Genet. 2009;18:785-96 pubmed publisher
    ..We performed histochemical studies in human and mouse and showed that mGluR7 is expressed in hair cells and in spiral ganglion cells of the inner ear...
  63. Perroy J, Richard S, Nargeot J, Bockaert J, Fagni L. Permissive effect of voltage on mGlu 7 receptor subtype signaling in neurons. J Biol Chem. 2002;277:1223-8 pubmed
    ..release of intracellular Ca(2+) and in blocking Ca(2+) channels, when applied concomitantly with the mGlu7a receptor agonist, d,l-aminophosphonobutyrate...
  64. Holscher C, Schmid S, Pilz P, Sansig G, van der Putten H, Plappert C. Lack of the metabotropic glutamate receptor subtype 7 selectively impairs short-term working memory but not long-term memory. Behav Brain Res. 2004;154:473-81 pubmed
    ..Here, we report the effect of mGluR7 gene ablation in different learning paradigms...
  65. McGivern J, Patitucci T, Nord J, Barabas M, Stucky C, Ebert A. Spinal muscular atrophy astrocytes exhibit abnormal calcium regulation and reduced growth factor production. Glia. 2013;61:1418-1428 pubmed publisher
    ..Together, for the first time, these data show early disruptions in astrocytes that may contribute to SMA disease pathology. ..
  66. Fletcher E, Simon C, Pagiazitis J, Chalif J, Vukojicic A, Drobac E, et al. Reduced sensory synaptic excitation impairs motor neuron function via Kv2.1 in spinal muscular atrophy. Nat Neurosci. 2017;20:905-916 pubmed publisher
    ..Our results demonstrate a key role of excitatory synaptic drive in shaping the function of motor neurons during development and the contribution of its disruption to a neurodegenerative disease. ..
  67. Wieronska J, Branski P, Siwek A, Dybala M, Nowak G, Pilc A. GABAergic dysfunction in mGlu7 receptor-deficient mice as reflected by decreased levels of glutamic acid decarboxylase 65 and 67kDa and increased reelin proteins in the hippocampus. Brain Res. 2010;1334:12-24 pubmed publisher
    ..The results suggest that mGlu7 receptor is involved in the regulation of GABAergic system activity at the level of GABA synthesised enzymes, specific proteins expressed by GABAergic neurons and metabotropic receptor for GABA. ..
  68. Tisdale S, Lotti F, Saieva L, Van Meerbeke J, Crawford T, Sumner C, et al. SMN is essential for the biogenesis of U7 small nuclear ribonucleoprotein and 3'-end formation of histone mRNAs. Cell Rep. 2013;5:1187-95 pubmed publisher
    ..These findings demonstrate that SMN is essential for U7 biogenesis and histone mRNA processing in vivo and identify an additional RNA pathway disrupted in SMA. ..
  69. Iyer C, McGovern V, Murray J, Gombash S, Zaworski P, Foust K, et al. Low levels of Survival Motor Neuron protein are sufficient for normal muscle function in the SMNΔ7 mouse model of SMA. Hum Mol Genet. 2015;24:6160-73 pubmed publisher
    ..SMA is caused by deletion or mutation of the Survival Motor Neuron 1 (SMN1) gene and retention of the SMN2 gene. The loss of SMN1 results in reduced levels of the SMN protein...
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