J Appl Physiol 102: 2121-2127, 2007. First published March 8, 2007; doi:10.1152/japplphysiol.01327.2006
8750-7587/07
Creatine uptake in brain and skeletal muscle of mice lacking guanidinoacetate methyltransferase assessed
by magnetic resonance spectroscopy
Hermien E. Kan, Esther Meeuwissen, Jack J. van Asten, Andor Veltien, Dirk Isbrandt, and Arend Heerschap
Human Molecular Genetics Advance Access originally published online on August 21, 2006
Human Molecular Genetics 2006 15(19):2888-2902; doi:10.1093/hmg/ddl230
C-terminal HERG (LQT2) mutations disrupt IKr channel regulation through 14-3-3{epsilon}
Chi-un Choe, Eric Schulze-Bahr, Axel Neu, Jun Xu, Zheng I. Zhu, Kathrin Saute1, Robert Bähring, Silvia Priori,
Pascale Guicheney, Gerold Mönnig, Carlo Neapolitano, Jan Heidemann, Colleen E. Clancy, Olaf Pongs and Dirk Isbrandt
DOI: 10.1113/jphysiol.2005.094359
2005;568;397-412; originally published online Aug 11, 2005; J. Physiol.
Contribution of N- and C-terminal channel domains to Kv channel interacting proteins in a mammalian cell line
Britta Callsen, Dirk Isbrandt, Kathrin Sauter, L. Sven Hartmann, Olaf Pongs and Robert Bähring
Circulation. 2005;111:2477-2485.
© 2005 American Heart Association, Inc.
Reduced Inotropic Reserve and Increased Susceptibility to Cardiac Ischemia/Reperfusion Injury
in Phosphocreatine-Deficient Guanidinoacetate-N-Methyltransferase–Knockout Mice
Michiel ten Hove, Craig A. Lygate, Alexandra Fischer, Jürgen E. Schneider, A. Elisabeth Sang, Karen Hulbert;
Liam Sebag-Montefiore; Hugh Watkins, Kieran Clarke, Dirk Isbrandt, Julie Wallis, Stefan Neubauer
Journal of the Neurological Sciences
Volume 231, Issues 1-2, 15 April 2005, Pages 49-55
Biochemical and behavioural phenotyping of a mouse model for GAMT deficiency
An Torremans, Bart Marescau, Ilse Possemiers, Debby Van Dam, Rudi D'Hooge, Dirk Isbrandt and Peter Paul De Deyn
Am J Physiol Cell Physiol 289: C113-C119, 2005. First published March 2, 2005; doi:10.1152/ajpcell.00040.2005
0363-6143/05
Lower force and impaired performance during high-intensity electrical stimulation in skeletal muscle
of GAMT-deficient knockout mice
H. E. Kan, T. E. Buse-Pot, R. Peco, D. Isbrandt, A. Heerschap,and A. de Haan
Nature Neuroscience 8, 51 - 60 (2004)
Published online: 19 December 2004; | doi:10.1038/nn1375
Conditional transgenic suppression of M channels in mouse brain reveals functions in
neuronal excitability, resonance and behavior
H Christian Peters, Hua Hu, Olaf Pongs, Johan F Storm & Dirk Isbrandt
Magnetic Resonance in Medicine 52:1029–1035 (2004)
In Vivo Cardiac 1H-MRS in the Mouse
Juergen E. Schneider, Damian J. Tyler, Michiel ten Hove, Elizabeth Sang,
Paul J. Cassidy, Alexandra Fischer, Julie Wallis, Liam M. Sebag-Montefiore,
Hugh Watkins, Dirk Isbrandt, Kieran Clarke, and Stefan Neubauer
Human Molecular Genetics, 2004, Vol. 13, No. 20 2483–2491 doi:10.1093/hmg/ddh264
Mutations in classical late infantile neurona lceroid lipofuscinosis disrupt transport of tripeptidyl-peptidase I to lysosomes
Robert Steinfeld, Hans-Bertram Steinke, Dirk Isbrandt, Alfried Kohlschuetter and Jutta Gaertner
J Physiol. 2004 October 1; 560(Pt 1): 219–229.
Published online 2004 July 29. doi: 10.1113/jphysiol.2004.067926
Phosphorylated guanidinoacetate partly compensates for the lack of phosphocreatine in skeletal muscle of mice lacking guanidinoacetate methyltransferase
Hermien E Kan, W Klaas Jan Renema, Dirk Isbrandt, and Arend Heerschap
Human Molecular Genetics, 2004, Vol. 13, No. 9 905–921
Severely altered guanidino compound levels, disturbed body weight homeostasis and impaired
fertility in a mouse model of guanidinoacetate N-methyltransferase (GAMT) deficiency
Andreas Schmidt, Bart Marescau, Ernest A. Boehm, W. Klaas Jan Renema, Ruben Peco, Anib Das, Robert Steinfeld, Sharon Chan,
Julie Wallis, Michail Davidoff, Kurt Ullrich, Ralph Waldschuetz, Arend Heerschap, Peter P. De Deyn, Stefan Neubauer and Dirk Isbrandt
Clinical Genetics ,Volume 65 Issue 3 Page 233-241, March 2004
Effective long-term control of cardiac events with β-blockers in a family with a common LQT1 mutation
H Wedekind, M Schwarz, S Hauenschild, H Djonlagic, W Haverkamp, G Breithardt, T Wülfing, O Pongs, D Isbrandt, E Schulze-Bahr
Receptors Channels. 2003;9(6):387-96
Biophysical properties of Kv3.1 channels in SH-SY5Y human neuroblastoma cells.
Friederich P, Dilger JP, Isbrandt D, Sauter K, Pongs O, Urban BW.
Biophysical Journal 86:210-223 (2004)
N-type Inactivation Features of Kv4.2 Channel Gating
Manuel Gebauer, Dirk Isbrandt, Kathrin Sauter, Britta Callsen, Andreas Nolting, Olaf Pongs and Robert Bähring
Br J Anaesth. 2004 Jan;92(1):93-101
Local anaesthetic sensitivities of cloned HERG channels from human heart: comparison with HERG/MiRP1 and HERG/MiRP1 T8A.
Friederich P, Solth A, Schillemeit S, Isbrandt D.
Magnetic Resonance in Medicine 50:936–943 (2003)
MR Spectroscopy of Muscle and Brain in Guanidinoacetate Methyltransferase (GAMT)-Deficient
Mice: Validation of an Animal Model to Study Creatine Deficiency
W. Klaas Jan Renema, Andreas Schmidt, Jack J.A. van Asten, Frank Oerlemans,
Kurt Ullrich,4Be´ Wieringa, Dirk Isbrandt, and Arend Heerschap
J. Clin. Invest. 111:1537-1545 (2003). doi:10.1172/JCI200316387.
Pacemaker channel dysfunction in a patient with sinus node disease
Eric Schulze-Bahr, Axel Neu, Patrick Friederich, U. Benjamin Kaupp, Günter Breithardt, Olaf Pongs and Dirk Isbrandt
RNA. 2002 Dec;8(12):1538-47
UM 9(5)h and UM 9(5)p, human and porcine noncoding transcripts with preferential expression in the cerebellum.
Michel U, Kallmann B, Rieckmann P, Isbrandt D.
Neurobiol Dis. 2002 Nov;11(2):298-307
Activation of GABA(A) receptors by guanidinoacetate: a novel pathophysiological mechanism.
Neu A, Neuhoff H, Trube G, Fehr S, Ullrich K, Roeper J, Isbrandt D.
J Mol Med. 2002 Aug;80(8):524-32
Identification and functional characterization of a novel KCNE2 (MiRP1) mutation that alters HERG channel kinetics.
Isbrandt D, Friederich P, Solth A, Haverkamp W, Ebneth A, Borggrefe M, Funke H, Sauter K, Breithardt G, Pongs O, Schulze-Bahr E
J Mol Med. 2001 Sep;79(9):504-9
A novel long-QT 5 gene mutation in the C-terminus (V109I) is associated with a mild phenotype.
Schulze-Bahr E, Schwarz M, Hauenschild S, Wedekind H, Funke H, Haverkamp W, Breithardt G, Pongs O, Isbrandt D.
J. Biol. Chem., Vol. 276, Issue 26, 23888-23894, June 29, 2001
Conserved Kv4 N-terminal Domain Critical for Effects of Kv Channel-interacting Protein 2.2
on Channel Expression and Gating
Robert Bähring, Jens Dannenberg, H. Christian Peters, Thorsten Leicher, Olaf Pongs, and Dirk Isbrandt
J Inherit Metab Dis. 2000 Jun;23(4):375-7
Upregulation of respiratory chain enzymes in guanidinoacetate methyltransferase deficiency.
Das AM, Ullrich K, Isbrandt D.
Genomics. 2000 Mar 1;64(2):144-54
Gene structures and expression profiles of three human KCND (Kv4) potassium channels
mediating A-type currents I(TO) and I(SA).
Isbrandt D, Leicher T, Waldschütz R, Zhu X, Luhmann U, Michel U, Sauter K, Pongs O.
Institute
