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Select item 101036301.

Dodecyl creatine ester improves cognitive function and identifies key protein drivers including KIF1A and PLCB1 in a mouse model of creatine transporter deficiency

Aloïse Mabondzo, Rania Harati, Léa Broca-Brisson, Anne-Cécile Guyot, Narciso Costa, Francesco Cacciante, Elena Putignano, Laura Baroncelli, Matthew R. Skelton, Cathy Saab, Emmanuelle Martini, Henri Benech, Thomas Joudinaud, Jean-Charles Gaillard, Jean Armengaud, Rifat Hamoudi

Front Mol Neurosci. 2023; 16: 1118707. Published online 2023 Mar 24. doi: 10.3389/fnmol.2023.1118707

PMCID:: PMC10103630

Abstract Article PDF–4.1M

Select item 84923312.

Creatine transporter deficiency impairs stress adaptation and brain energetics homeostasis

Hong-Ru Chen, Xiaohui Zhang-Brotzge, Yury M. Morozov, Yuancheng Li, Siming Wang, Helen Heju Zhang, Irena S. Kuan, Elizabeth M. Fugate, Hui Mao, Yu-Yo Sun, Pasko Rakic, Diana M. Lindquist, Ton DeGrauw, Chia-Yi Kuan

JCI Insight. 2021 Sep 8; 6(17): e140173. Published online 2021 Sep 8. doi: 10.1172/jci.insight.140173

PMCID:: PMC8492331

Abstract Article PDF–3.8M

Select item 75915303.

Cyclocreatine treatment ameliorates the cognitive, autistic and epileptic phenotype in a mouse model of Creatine Transporter Deficiency

Francesco Cacciante, Mariangela Gennaro, Giulia Sagona, Raffaele Mazziotti, Leonardo Lupori, Elisa Cerri, Elena Putignano, Mark Butt, Minh-Ha T. Do, John C. McKew, Maria Grazia Alessandrì, Roberta Battini, Giovanni Cioni, Tommaso Pizzorusso, Laura Baroncelli

Sci Rep. 2020; 10: 18361. Published online 2020 Oct 27. doi: 10.1038/s41598-020-75436-4

PMCID:: PMC7591530

Abstract Article PDF–2.4M

Select item 67391354.

Deletion of the creatine transporter gene in neonatal, but not adult, mice lead to cognitive deficits.

Kenea C. Udobi, Nicholas Delcimmuto, Amanda N. Kokenge, Zuhair I. Abdulla, Marla K. Perna, Matthew R. Skelton

J Inherit Metab Dis. Author manuscript; available in PMC 2020 Sep 1.

Published in final edited form as: J Inherit Metab Dis. 2019 Sep; 42(5): 966–974. Published online 2019 Jul 4. doi: 10.1002/jimd.12137

PMCID:: PMC6739135

Abstract Article PDF–920K

Select item 66969155.

Cognitive deficits and increases in creatine precursors in a brain-specific knockout of the creatine transporter gene Slc6a8

K. C. Udobi, A. N. Kokenge, E. R. Hautman, G. Ullio, J. Coene, M. T. Williams, C. V. Vorhees, A. Mabondzo, M. R. Skelton

Genes Brain Behav. Author manuscript; available in PMC 2019 Aug 16.

Published in final edited form as: Genes Brain Behav. 2018 Jul; 17(6): e12461. Published online 2018 Feb 20. doi: 10.1111/gbb.12461

PMCID:: PMC6696915

Abstract Article PDF–1.0M

Select item 63298056.

A Nervous System-Specific Model of Creatine Transporter Deficiency Recapitulates the Cognitive Endophenotype of the Disease: a Longitudinal Study

Angelo Molinaro, Maria Grazia Alessandrì, Elena Putignano, Vincenzo Leuzzi, Giovanni Cioni, Laura Baroncelli, Tommaso Pizzorusso

Sci Rep. 2019; 9: 62. Published online 2019 Jan 11. doi: 10.1038/s41598-018-37303-1

PMCID:: PMC6329805

Abstract Article PDF–1.4M

Select item 60362597.

A Mouse Model of Creatine Transporter Deficiency Reveals Impaired Motor Function and Muscle Energy Metabolism

Malte Stockebrand, Ali Sasani, Devashish Das, Sönke Hornig, Irm Hermans-Borgmeyer, Hannah A. Lake, Dirk Isbrandt, Craig A. Lygate, Arend Heerschap, Axel Neu, Chi-Un Choe

Front Physiol. 2018; 9: 773. Published online 2018 Jun 22. doi: 10.3389/fphys.2018.00773

PMCID:: PMC6036259

Abstract Article PDF–3.7M

Select item 49756338.

Creatine transporter deficiency leads to increased whole-body and cellular metabolism

Marla K. Perna, Amanda N. Kokenge, Keila N. Miles, Kenea C. Udobi, Joseph F. Clark, Gail J. Pyne-Geithman, Zaza Khuchua, Matthew R. Skelton

Amino Acids. Author manuscript; available in PMC 2017 Aug 1.

Published in final edited form as: Amino Acids. 2016 Aug; 48(8): 2057–2065. Published online 2016 Jul 11. doi: 10.1007/s00726-016-2291-3

PMCID:: PMC4975633

Abstract Article PDF–2.2M

Select item 41443449.

Creatine transporter (SLC6A8) knockout mice display an increased capacity for in vitro creatine biosynthesis in skeletal muscle

Aaron P. Russell, Lobna Ghobrial, Craig R. Wright, Séverine Lamon, Erin L. Brown, Michihiro Kon, Matthew R. Skelton, Rodney J. Snow

Front Physiol. 2014; 5: 314. Published online 2014 Aug 26. doi: 10.3389/fphys.2014.00314

PMCID:: PMC4144344

Abstract Article PDF–632K

Select item 340873010.

Cyclocreatine treatment improves cognition in mice with creatine transporter deficiency

Yuko Kurosawa, Ton J. DeGrauw, Diana M. Lindquist, Victor M. Blanco, Gail J. Pyne-Geithman, Takiko Daikoku, James B. Chambers, Stephen C. Benoit, Joseph F. Clark

J Clin Invest. 2012 Aug 1; 122(8): 2837–2846. Published online 2012 Jul 2. doi: 10.1172/JCI59373

PMCID:: PMC3408730

Abstract Article PDF–631K

Select item 302096811.

Creatine Transporter (CrT; Slc6a8) Knockout Mice as a Model of Human CrT Deficiency

Matthew R. Skelton, Tori L. Schaefer, Devon L. Graham, Ton J. deGrauw, Joseph F. Clark, Michael T. Williams, Charles V. Vorhees

PLoS One. 2011; 6(1): e16187. Published online 2011 Jan 13. doi: 10.1371/journal.pone.0016187

PMCID:: PMC3020968

Abstract Article PDF–744K

Select item 283778112.

Mice Over-Expressing the Myocardial Creatine Transporter Develop Progressive Heart Failure and Show Decreased Glycolytic Capacity

Darci Phillips, Michiel ten Hove, Jurgen E. Schneider, Colin O. Wu, Liam Sebag-Montefiore, Angel M. Aponte, Craig A. Lygate, Julie Wallis, Kieran Clarke, Hugh Watkins, Robert S. Balaban, Stefan Neubauer

J Mol Cell Cardiol. Author manuscript; available in PMC 2011 Apr 1.

Published in final edited form as: J Mol Cell Cardiol. 2010 Apr; 48(4): 582–590. Published online 2009 Nov 11. doi: 10.1016/j.yjmcc.2009.10.033

PMCID:: PMC2837781

Abstract Article PDF–1.5M

Select item 189035213.

Creatine as a compatible osmolyte in muscle cells exposed to hypertonic stress

Roberta R Alfieri, Mara A Bonelli, Andrea Cavazzoni, Maurizio Brigotti, Claudia Fumarola, Piero Sestili, Paola Mozzoni, Giuseppe De Palma, Antonio Mutti, Domenica Carnicelli, Federica Vacondio, Claudia Silva, Angelo F Borghetti, Kenneth P Wheeler, Pier Giorgio Petronini

J Physiol. 2006 Oct 15; 576(Pt 2): 391–401. Published online 2006 Jul 27. doi: 10.1113/jphysiol.2006.115006

PMCID:: PMC1890352

Abstract Article PDF–261K

Select item 999022414.

Cell-specific vulnerability to metabolic failure: the crucial role of parvalbumin expressing neurons in creatine transporter deficiency

Elsa Ghirardini, Giulia Sagona, Angel Marquez-Galera, Francesco Calugi, Carmen M. Navarron, Francesco Cacciante, Siwei Chen, Federica Di Vetta, Lorenzo Dadà, Raffaele Mazziotti, Leonardo Lupori, Elena Putignano, Pierre Baldi, Jose P. Lopez-Atalaya, Tommaso Pizzorusso, Laura Baroncelli

Acta Neuropathol Commun. 2023; 11: 34. Published online 2023 Mar 7. doi: 10.1186/s40478-023-01533-w

PMCID:: PMC9990224

Abstract Article PDF–6.1M

Select item 789184615.

Creatine Transporter, Reduced in Colon Tissues From Patients With Inflammatory Bowel Diseases, Regulates Energy Balance in Intestinal Epithelial Cells, Epithelial Integrity, and Barrier Function

Caroline H.T. Hall, Lee J. Scott, Emily M. Murphy, Mark E. Gerich, Rachael Dran, Louis E. Glover, Zuhair I Abdulla, Matthew R Skelton, Sean P. Colgan

Gastroenterology. Author manuscript; available in PMC 2021 Sep 1.

Published in final edited form as: Gastroenterology. 2020 Sep; 159(3): 984–998.e1. Published online 2020 May 17. doi: 10.1053/j.gastro.2020.05.033

PMCID:: PMC7891846

Abstract Article PDF–2.9M

Select item 700176616.

Deletion of the creatine transporter (Slc6a8) in dopaminergic neurons leads to hyperactivity in mice

Zuhair I. Abdulla, Bahar Pahlevani, Kerstin H. Lundgren, Jordan L. Pennington, Kenea C. Udobi, Kim B. Seroogy, Matthew R. Skelton

J Mol Neurosci. Author manuscript; available in PMC 2021 Jan 1.

Published in final edited form as: J Mol Neurosci. 2020 Jan; 70(1): 102–111. Published online 2019 Sep 13. doi: 10.1007/s12031-019-01405-w

PMCID:: PMC7001766

Abstract Article PDF–1.0M

Select item 424376117.

A novel mouse model of creatine transporter deficiency

Laura Baroncelli, Maria Grazia Alessandrì, Jonida Tola, Elena Putignano, Martina Migliore, Elena Amendola, Cornelius Gross, Vincenzo Leuzzi, Giovanni Cioni, Tommaso Pizzorusso

Version 1. F1000Res. 2014; 3: 228. Published online 2014 Sep 29. doi: 10.12688/f1000research.5369.1

PMCID:: PMC4243761

Abstract Article PDF–888K

Select item 290417918.

Regulation of the creatine transporter by AMP-activated protein kinase in kidney epithelial cells

Hui Li, Ramon F. Thali, Christy Smolak, Fan Gong, Rodrigo Alzamora, Theo Wallimann, Roland Scholz, Núria M. Pastor-Soler, Dietbert Neumann, Kenneth R. Hallows

Am J Physiol Renal Physiol. 2010 Jul; 299(1): F167–F177. Published online 2010 May 12. doi: 10.1152/ajprenal.00162.2010

PMCID:: PMC2904179

Abstract Article

Select item 266507019.

ERp57 Does Not Require Interactions with Calnexin and Calreticulin to Promote Assembly of Class I Histocompatibility Molecules, and It Enhances Peptide Loading Independently of Its Redox Activity

Yinan Zhang, Guennadi Kozlov, Cosmin L. Pocanschi, Ulf Brockmeier, Breanna S. Ireland, Pekka Maattanen, Chris Howe, Tim Elliott, Kalle Gehring, David B. Williams

J Biol Chem. 2009 Apr 10; 284(15): 10160–10173. doi: 10.1074/jbc.M808356200