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Nieuwenhuis S, Okkersen K, Widomska J, et al. Insulin Signaling as a Key Moderator in Myotonic Dystrophy Type 1. Front Neurol. 2019;10:1229doi: 10.3389/fneur.2019.01229.
Nieuwenhuis, S., Okkersen, K., Widomska, J., Blom, P., 't Hoen, P. A. C., van Engelen, B., & Glennon, J. C. (2019). Insulin Signaling as a Key Moderator in Myotonic Dystrophy Type 1. Frontiers in neurology, 101229. https://doi.org/10.3389/fneur.2019.01229
Nieuwenhuis, Sylvia, et al. "Insulin Signaling as a Key Moderator in Myotonic Dystrophy Type 1." Frontiers in neurology vol. 10 (2019): 1229. doi: https://doi.org/10.3389/fneur.2019.01229
Nieuwenhuis S, Okkersen K, Widomska J, Blom P, 't Hoen PAC, van Engelen B, Glennon JC. Insulin Signaling as a Key Moderator in Myotonic Dystrophy Type 1. Front Neurol. 2019 Nov 26;10:1229. doi: 10.3389/fneur.2019.01229. eCollection 2019. PMID: 31849810; PMCID: PMC6901991.
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Front Neurol. 2019 Nov 26;10:1229. doi: 10.3389/fneur.2019.01229. eCollection 2019.

Insulin Signaling as a Key Moderator in Myotonic Dystrophy Type 1.

Frontiers in neurology

Sylvia Nieuwenhuis, Kees Okkersen, Joanna Widomska, Paul Blom, Peter A C 't Hoen, Baziel van Engelen, Jeffrey C Glennon

Affiliations

  1. Department of Cognitive Neuroscience, Donders Institute for Brain Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, Netherlands.
  2. Department of Neurology, Donders Institute for Brain Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, Netherlands.
  3. VDL Enabling Technologies Group B.V., Eindhoven, Netherlands.
  4. Centre for Molecular and Biomolecular Informatics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Centre, Nijmegen, Netherlands.

PMID: 31849810 PMCID: PMC6901991 DOI: 10.3389/fneur.2019.01229

Abstract

Myotonic dystrophy type 1 (DM1) is an autosomal dominant genetic disease characterized by multi-system involvement. Affected organ system includes skeletal muscle, heart, gastro-intestinal system and the brain. In this review, we evaluate the evidence for alterations in insulin signaling and their relation to clinical DM1 features. We start by summarizing the molecular pathophysiology of DM1. Next, an overview of normal insulin signaling physiology is given, and evidence for alterations herein in DM1 is presented. Clinically, evidence for involvement of insulin signaling pathways in DM1 is based on the increased incidence of insulin resistance seen in clinical practice and recent trial evidence of beneficial effects of metformin on muscle function. Indirectly, further support may be derived from certain CNS derived symptoms characteristic of DM1, such as obsessive-compulsive behavior features, for which links with altered insulin signaling has been demonstrated in other diseases. At the basic scientific level, several pathophysiological mechanisms that operate in DM1 may compromise normal insulin signaling physiology. The evidence presented here reflects the importance of insulin signaling in relation to clinical features of DM1 and justifies further basic scientific and clinical, therapeutically oriented research.

Copyright © 2019 Nieuwenhuis, Okkersen, Widomska, Blom, 't Hoen, van Engelen and Glennon.

Keywords: behavioral flexibility; diabetes type 2; insulin; insulin resistance; insulin-like growth factor 1 (IGF1); metformin; myotonic dystrophy (DM1); obsessive–compulsive disorder

References

  1. JAMA Neurol. 2018 May 1;75(5):573-581 - PubMed
  2. Brain. 1987 Apr;110 ( Pt 2):469-88 - PubMed
  3. EBioMedicine. 2017 Feb;15:150-162 - PubMed
  4. Int J Neuropsychopharmacol. 2017 Jun 1;20(6):476-484 - PubMed
  5. Ann Neurol. 2005 May;57(5):754-7 - PubMed
  6. Cell Calcium. 2018 Jan;69:81-100 - PubMed
  7. Diabetes. 1987 Jun;36(6):693-701 - PubMed
  8. N Engl J Med. 1968 Sep 26;279(13):718-9 - PubMed
  9. Clin Investig. 1994 Jul;72(7):508-11 - PubMed
  10. Brain. 1970;93(4):731-42 - PubMed
  11. Curr Vasc Pharmacol. 2017;15(1):30-39 - PubMed
  12. Nat Genet. 2001 Sep;29(1):40-7 - PubMed
  13. J Neuromuscul Dis. 2018;5(3):279-294 - PubMed
  14. Structure. 2015 Jul 7;23(7):1271-82 - PubMed
  15. Ann Neurol. 1979 Jul;6(1):60-5 - PubMed
  16. Hum Mol Genet. 2002 May 1;11(9):1045-58 - PubMed
  17. Diabetes. 2002 Oct;51(10):2944-50 - PubMed
  18. J Clin Invest. 1997 Apr 1;99(7):1691-8 - PubMed
  19. Metab Brain Dis. 2017 Jun;32(3):849-857 - PubMed
  20. Mol Cell Biol. 2005 Feb;25(4):1402-14 - PubMed
  21. Biochim Biophys Acta. 2007 Dec;1772(11-12):1183-91 - PubMed
  22. Ageing Res Rev. 2017 Jan;33:105-114 - PubMed
  23. Cell. 2015 Feb 12;160(4):745-758 - PubMed
  24. J Mammary Gland Biol Neoplasia. 2008 Dec;13(4):371-9 - PubMed
  25. Indian J Med Res. 2012;135:127-30 - PubMed
  26. Neurosci Res. 2019 Dec;149:1-13 - PubMed
  27. Am J Pathol. 2009 Jan;174(1):216-27 - PubMed
  28. Front Neurol. 2018 Aug 21;9:646 - PubMed
  29. Enzyme. 1991;45(1-2):14-22 - PubMed
  30. Hum Mol Genet. 1995 Jun;4(6):1063-72 - PubMed
  31. Am J Med Sci. 1970 Dec;260(6):351-8 - PubMed
  32. J R Soc Interface. 2013 Sep 18;10(88):20130605 - PubMed
  33. Eur J Neurol. 2019 Jan;26(1):58-65 - PubMed
  34. J Cell Biol. 2002 Apr 1;157(1):137-48 - PubMed
  35. J Clin Endocrinol Metab. 1979 Aug;49(2):216-22 - PubMed
  36. J Neurosci. 2011 Oct 5;31(40):14361-6 - PubMed
  37. FEBS Lett. 2008 Jan 9;582(1):97-105 - PubMed
  38. Mol Neuropsychiatry. 2017 Nov;3(2):97-107 - PubMed
  39. Biochim Biophys Acta. 2015 Apr;1852(4):594-606 - PubMed
  40. Diabet Med. 2005 Mar;22(3):346-7 - PubMed
  41. Obes Rev. 2019 Sep;20(9):1205-1217 - PubMed
  42. J Cachexia Sarcopenia Muscle. 2017 Jun;8(3):349-369 - PubMed
  43. Ann Neurol. 1984 Feb;15(2):157-62 - PubMed
  44. Nat Biotechnol. 2010 Dec;28(12):1248-50 - PubMed
  45. J Mol Endocrinol. 2015 Feb;54(1):R1-13 - PubMed
  46. Cytokine Growth Factor Rev. 2017 Feb;33:73-82 - PubMed
  47. Diabetes Care. 2003 Jul;26(7):2112-8 - PubMed
  48. Sci Rep. 2015 May 18;5:10227 - PubMed
  49. Nature. 2010 Mar 25;464(7288):529-35 - PubMed
  50. Handb Clin Neurol. 2011;101:193-237 - PubMed
  51. Pediatr Allergy Immunol. 2019 May;30(3):277-288 - PubMed
  52. PLoS One. 2017 Sep 15;12(9):e0184987 - PubMed
  53. J Rehabil Med. 2011 Jul;43(8):695-702 - PubMed
  54. J Neurol Sci. 2009 Jan 15;276(1-2):60-5 - PubMed
  55. Endocr Rev. 2009 Oct;30(6):586-623 - PubMed
  56. PLoS One. 2017 Jun 1;12(6):e0178885 - PubMed
  57. Can J Neurol Sci. 2013 Jan;40(1):42-7 - PubMed
  58. Proc Natl Acad Sci U S A. 1981 Apr;78(4):2567-71 - PubMed
  59. Muscle Nerve. 2011 Oct;44(4):503-8 - PubMed
  60. PLoS Genet. 2012;8(11):e1003043 - PubMed
  61. Front Aging Neurosci. 2015 Jul 09;7:125 - PubMed
  62. Front Endocrinol (Lausanne). 2018 Jan 17;9:2 - PubMed
  63. Eur Neuropsychopharmacol. 2014 Dec;24(12):1947-53 - PubMed
  64. BMC Neurol. 2015 Aug 22;15:148 - PubMed
  65. Front Cell Neurosci. 2019 Feb 05;13:17 - PubMed
  66. Ageing Res Rev. 2017 Jan;33:3-17 - PubMed
  67. Elife. 2014 Sep 25;3: - PubMed
  68. J Clin Endocrinol Metab. 1968 Jul;28(7):992-8 - PubMed
  69. Neurology. 2017 Aug 29;89(9):960-969 - PubMed
  70. Front Endocrinol (Lausanne). 2017 Feb 22;8:31 - PubMed
  71. Neurobiol Dis. 2010 Jul;39(1):21-7 - PubMed
  72. Nature. 2011 Jan 27;469(7331):491-7 - PubMed
  73. Dev Dyn. 2015 Mar;244(3):377-90 - PubMed
  74. J Clin Invest. 1995 Jan;95(1):179-86 - PubMed
  75. Brain Res. 2015 Oct 5;1622:466-73 - PubMed
  76. J Neurol Sci. 1986 Jul;74(2-3):247-55 - PubMed
  77. Sci Signal. 2012 Dec 18;5(255):ra92 - PubMed
  78. J Clin Endocrinol Metab. 1969 May;29(5):684-90 - PubMed
  79. Cortex. 2017 Oct;95:143-155 - PubMed
  80. Lancet Neurol. 2018 Aug;17(8):671-680 - PubMed
  81. Brain. 1959 Jun;82:221-31 - PubMed
  82. N Engl J Med. 1967 Oct 19;277(16):837-41 - PubMed
  83. Nat Rev Endocrinol. 2015 Dec;11(12):701-11 - PubMed
  84. Physiol Rev. 2016 Oct;96(4):1169-209 - PubMed
  85. Am J Physiol Endocrinol Metab. 2006 Oct;291(4):E745-54 - PubMed
  86. J Psychiatry Neurosci. 2016 Jun;41(4):280-5 - PubMed
  87. J Clin Endocrinol Metab. 1977 Oct;45(4):821-4 - PubMed
  88. Curr Opin Genet Dev. 2017 Jun;44:135-140 - PubMed
  89. Transl Psychiatry. 2019 Sep 12;9(1):225 - PubMed
  90. Curr Genomics. 2011 May;12(3):225-35 - PubMed
  91. Am J Physiol Endocrinol Metab. 2000 Nov;279(5):E1145-58 - PubMed
  92. J Lab Clin Med. 1976 Mar;87(3):384-96 - PubMed
  93. Diabetes. 1973 Apr;22(4):238-42 - PubMed
  94. PLoS One. 2019 Mar 22;14(3):e0214254 - PubMed
  95. Science. 1997 Oct 17;278(5337):419-24 - PubMed
  96. Endocr Rev. 2005 Dec;26(7):916-43 - PubMed
  97. Muscle Nerve. 2015 Aug;52(2):273-7 - PubMed
  98. Oncotarget. 2015 Nov 24;6(37):39469-81 - PubMed
  99. Brain. 2018 Oct 1;141(10):2855-2865 - PubMed
  100. J Clin Invest. 1978 Oct;62(4):857-67 - PubMed
  101. Int J Cancer. 2013 Jan 15;132(2):411-6 - PubMed
  102. J Pharmacol Exp Ther. 2003 Jan;304(1):453-63 - PubMed
  103. Nucleic Acids Res. 2018 Jul 6;46(12):6069-6086 - PubMed
  104. N Engl J Med. 1969 Jan 23;280(4):223 - PubMed
  105. Metabolism. 1990 Sep;39(9):938-42 - PubMed
  106. Arch Toxicol. 2015 Sep;89(9):1401-38 - PubMed
  107. Mol Cell Biol. 2003 Aug;23(16):5489-501 - PubMed
  108. Science. 2003 Apr 18;300(5618):502-5 - PubMed
  109. Endocrinology. 1999 Sep;140(9):4244-50 - PubMed
  110. Exp Mol Med. 2015 Mar 13;47:e149 - PubMed
  111. J Clin Endocrinol Metab. 1995 Dec;80(12):3715-23 - PubMed
  112. Diabetes Obes Metab. 2018 Sep;20 Suppl 2:28-50 - PubMed
  113. Pediatr Res. 2005 Jul;58(1):89-93 - PubMed
  114. Sci Rep. 2015 Jan 21;5:7911 - PubMed
  115. Diabetes Care. 2018 Jan;41(Suppl 1):S13-S27 - PubMed
  116. Mol Cell Biochem. 2013 Aug;380(1-2):259-65 - PubMed
  117. J Affect Disord. 2019 May 1;250:260-269 - PubMed
  118. Mitochondrion. 2018 Jan;38:58-70 - PubMed
  119. Neurology. 1992 Jan;42(1):91-4 - PubMed
  120. Sci Rep. 2019 Jun 28;9(1):9388 - PubMed
  121. Diabetologia. 1992 Dec;35(12):1170-2 - PubMed
  122. J Gerontol A Biol Sci Med Sci. 2012 Dec;67(12):1329-31 - PubMed
  123. PLoS One. 2007 Nov 07;2(11):e1134 - PubMed
  124. Metabolism. 1974 Jun;23(6):561-8 - PubMed
  125. Stem Cells Int. 2017;2017:8286248 - PubMed
  126. J Neurophysiol. 2003 Mar;89(3):1177-86 - PubMed
  127. Neuromuscul Disord. 1998 Feb;8(1):39-45 - PubMed
  128. Front Neurol. 2018 Jul 25;9:601 - PubMed
  129. Acta Neurol Scand. 2010 Feb;121(2):94-8 - PubMed
  130. J Neurol Sci. 1983 Feb;58(2):289-95 - PubMed
  131. Arch Neurol. 1998 May;55(5):635-40 - PubMed
  132. Diabetes. 2014 Dec;63(12):3992-7 - PubMed
  133. Front Cell Neurosci. 2017 Feb 01;11:14 - PubMed
  134. Arch Neurol. 2011 Jan;68(1):37-44 - PubMed
  135. Metabolism. 1969 Sep;18(9):764-9 - PubMed
  136. Medicine (Baltimore). 1974 Jul;53(4):307-23 - PubMed
  137. Am J Clin Nutr. 2004 Aug;80(2):357-64 - PubMed
  138. Front Mol Neurosci. 2014 Jan 09;6:57 - PubMed
  139. Pharmacogn Rev. 2017 Jul-Sep;11(22):128-135 - PubMed
  140. Neuromuscul Disord. 2017 Feb;27(2):163-169 - PubMed
  141. J Neurol. 2013 Sep;260(9):2330-4 - PubMed
  142. J Clin Invest. 1999 Feb;103(4):R1-7 - PubMed
  143. Brain Dev. 2015 Aug;37(7):661-8 - PubMed
  144. Med Hypotheses. 2002 Jan;58(1):83-4 - PubMed
  145. Arch Neurol. 1984 Jul;41(7):740-3 - PubMed
  146. Continuum (Minneap Minn). 2016 Dec;22(6, Muscle and Neuromuscular Junction Disorders):1889-1915 - PubMed
  147. Circ Res. 2019 Jan 4;124(1):121-141 - PubMed
  148. Traffic. 2001 May;2(5):303-10 - PubMed
  149. Neuroimage Clin. 2019;21:101615 - PubMed
  150. Hum Mol Genet. 2004 Dec 15;13(24):3079-88 - PubMed
  151. Expert Opin Drug Metab Toxicol. 2008 Mar;4(3):311-24 - PubMed
  152. Am J Med. 2001 Apr 1;110(5):385-98 - PubMed
  153. Exp Clin Endocrinol Diabetes. 2001;109 Suppl 2:S135-48 - PubMed
  154. J Clin Endocrinol Metab. 1977 Mar;44(3):491-8 - PubMed
  155. Mayo Clin Proc. 2012 Feb;87(2):130-5 - PubMed
  156. Mol Ther Nucleic Acids. 2015 Nov 03;4:e262 - PubMed
  157. Arch Argent Pediatr. 2017 Aug 1;115(4):391-398 - PubMed
  158. Cell Metab. 2012 May 2;15(5):595-605 - PubMed
  159. Eur J Endocrinol. 2002 Mar;146(3):397-405 - PubMed
  160. Am J Pathol. 2003 Mar;162(3):1001-9 - PubMed
  161. J Neurol Sci. 1981 Oct;52(1):11-23 - PubMed
  162. Neurology. 1983 Jun;33(6):679-85 - PubMed
  163. Endocrinology. 2008 Dec;149(12):5951 - PubMed

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