Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice

Journal of Neuroscience Research
Christine A DennyT N Seyfried

Abstract

Caloric restriction (CR), which improves health and increases longevity, was studied as a therapy in a hexosaminidase beta knockout mouse model of Sandhoff disease (SD), an incurable neurodegenerative disease involving accumulation of brain ganglioside GM2 and asialo-GM2 (GA2). Adult mice were fed a rodent chow diet either ad libitum (AL) or restricted to reduce body weight by 15-18% (CR). Although GM2 and GA2 were elevated, no significant differences were seen between the Hexb-/- and the Hexb+/- mice for most brain phospholipids and cholesterol. Cerebrosides and sulfatides were reduced in the Hexb-/- mice. In addition, rotorod performance was significantly worse in the Hexb-/- mice than in the Hexb+/- mice. CR, which decreased circulating glucose and elevated ketone bodies, significantly improved rotorod performance and extended longevity in the Hexb-/- mice but had no significant effect on brain lipid composition or on cytoplasmic neuronal vacuoles. The expression of CD68 and F4/80 was significantly less in the CR-fed than in the AL-fed Hexb-/- mice. We suggest that the CR delays disease progression in SD and possibly in other ganglioside storage diseases through anti-inflammatory mechanisms.

References

Jan 1, 1991·Annual Review of Biochemistry·E F Neufeld
Dec 1, 1971·Journal of Neurochemistry·K SandhoffH Jatzkewitz
Oct 1, 1967·The Journal of Clinical Investigation·O E OwenG F Cahill
Jan 1, 1983·Teratogenesis, Carcinogenesis, and Mutagenesis·R Fahrig, H Remmer
Jun 1, 1984·Experimental Neurology·T N SeyfriedR K Yu
Oct 1, 1981·European Journal of Immunology·J M Austyn, S Gordon
Mar 24, 1999·Journal of the National Cancer Institute·P MukherjeeS K Clinton
May 26, 1999·Proceedings of the National Academy of Sciences of the United States of America·M JeyakumarF M Platt
Oct 28, 1999·Neurobiology of Aging·T D PughR Weindruch
Jan 12, 2000·Toxicological Sciences : an Official Journal of the Society of Toxicology·D Kritchevsky
Jan 12, 2000·Toxicological Sciences : an Official Journal of the Society of Toxicology·K P KeenanR Dixit
Jul 11, 2000·Nature Genetics·C K LeeT A Prolla
Sep 27, 2000·Proceedings of the National Academy of Sciences of the United States of America·R WadaR L Proia
May 23, 2002·Human Molecular Genetics·Rachel MyerowitzRichard L Proia
Jun 14, 2002·Physiology & Behavior·Tamara L Y BondRichard E Brown
Jun 27, 2002·British Journal of Cancer·P MukherjeeT N Seyfried
Nov 9, 2002·Microscopy Research and Technique·Hae Young ChungByung Pal Yu
Feb 6, 2003·Genes & Development·Jana Koubova, Leonard Guarente
Feb 18, 2003·Proceedings of the National Academy of Sciences of the United States of America·Wenzhen DuanMark P Mattson
Jun 14, 2003·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·Konrad Sandhoff, Thomas Kolter
Sep 11, 2003·Annals of Internal Medicine·Mark P Mattson
Jan 1, 1962·The Biochemical Journal·D H WILLIAMSONH A KREBS
Oct 2, 2003·British Journal of Cancer·T N SeyfriedP Mukherjee
Apr 17, 2004·Journal of Neurochemistry·Julie L KasperzykThomas N Seyfried
May 25, 2004·Proceedings of the National Academy of Sciences of the United States of America·Yun-Ping Wu, Richard L Proia
Jun 17, 2004·Journal of Neurochemistry·Rosaria BuccolieroAnthony H Futerman
Oct 27, 2004·Annals of Neurology·Mylvaganam JeyakumarFrances M Platt
Dec 18, 2004·Proceedings of the National Academy of Sciences of the United States of America·Navin MaswoodDonald K Ingram
Apr 13, 2005·Journal of Neuroscience Research·R MasudaY Kashiwaya
Jun 2, 2005·Mechanisms of Ageing and Development·Stephen R Spindler

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Citations

May 18, 2011·Journal of Inherited Metabolic Disease·Kim M Hemsley, John J Hopwood
Feb 27, 2010·Glycoconjugate Journal·Stephanie D BoomkampTerry D Butters
May 20, 2006·Lipids in Health and Disease·Lisa B MahoneyThomas N Seyfried
Jul 12, 2007·Journal of Negative Results in Biomedicine·Melanie A McNallyDaniel A Kirschner
Jan 19, 2012·Journal of Oncology·Ivan UritsThomas N Seyfried
Mar 1, 2012·Neurochemical Research·J R ArthurT N Seyfried
May 25, 2010·Ageing Research Reviews·Fernanda M Cerqueira, Alicia J Kowaltowski
Jan 22, 2008·Neurochemistry International·Rena C BaekThomas N Seyfried
Apr 4, 2007·Journal of Neurochemistry·Do Young KimJong M Rho
Aug 25, 2010·Journal of Neurochemistry·Christine A DennyThomas N Seyfried
Jun 30, 2007·Neurobiology of Disease·Paula A DesplatsElizabeth A Thomas
Jul 1, 2015·Trends in Molecular Medicine·Deirdre M O'Connor, Nicholas M Boulis
Apr 5, 2008·Molecular Genetics and Metabolism·Elena Elliot-SmithFrances M Platt
May 18, 2010·Biochimica Et Biophysica Acta·Joern E SchroederDavid J Virley
Dec 17, 2014·European Journal of Medical Genetics·Ives T Villamizar-SchillerCarlos E Prada
Dec 15, 2006·Molecular Genetics and Metabolism·Kim M HemsleyJohn J Hopwood
Jun 23, 2018·ASN Neuro·T N SeyfriedJ S Schneider
Oct 2, 2008·Current Opinion in Clinical Nutrition and Metabolic Care·Sophie Gillette-Guyonnet, Bruno Vellas
Jun 28, 2020·Molecular Therapy : the Journal of the American Society of Gene Therapy·Hannah G LaheyMiguel Sena-Esteves

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