Research map: Creatine Supplementation Reduces Guanidinoacetate via Downregulation of AGAT in a Mouse Model of GAMT Deficiency
Back to the article
Papers in this map
Accurate normalization of real-time quantitative RT-PCR data by geometric averaging of multiple internal control genes
· Jo Vandesompele · 2002 · 20367 citations · Cited by this paper
Metabolism Controls the Timing of Human Brain Development and Maturation
· 2026 · Related
Creatine and Creatinine Metabolism
· Markus Wyss · 2000 · 2966 citations · Cited by this paper
Liver Gene Therapy in Fabry Disease Mice With Low Doses of rAAV2 /8 Expressing a Codon‐Optimized h GLA cDNA Results in Long‐Term Disease Correction
· 2026 · Related
Creatine Deficiency in the Brain: A New, Treatable Inborn Error of Metabolism
· Sylvia Stöckler · 1994 · 417 citations · Cited by this paper
Vitamin‐Responsive Disorders: From Molecular Basis to Clinical Presentation and Therapy
· 2026 · Related
Endogenous synthesis and transport of creatine in the rat brain: an in situ hybridization study
· Olivier Braissant · 2001 · 234 citations · Cited by this paper
Quantitative Muscle MRI of the Lower Extremities Reveals Different Patterns of Involvement in Classic Infantile and Young Late‐Onset Pompe Patients
· 2026 · Related
Creatine deficiency syndromes
· Andreas Schulze · 2003 · 193 citations · Cited by this paper
Tri‐Parametric Assessment of α‐Galactosidase A Activity, lysoGb3 and X‐Inactivation Aids Genotype‐Phenotype Categorization of Fabry Disease Female Patients
· 2026 · Related
Severely altered guanidino compound levels, disturbed body weight homeostasis and impaired fertility in a mouse model of guanidinoacetate N-methyltransferase (GAMT) deficiency
· Andreas C. Schmidt · 2004 · 176 citations · Cited by this paper
Acute Neurological Complications After Transplantation in Methylmalonic Acidemia: A 35‐Patient French Cohort
· 2026 · Related
Improving Treatment of Guanidinoacetate Methyltransferase Deficiency: Reduction of Guanidinoacetic Acid in Body Fluids by Arginine Restriction and Ornithine Supplementation
· Andreas Schulze · 2001 · 162 citations · Cited by this paper
Maternal and Fetal Outcomes in Imiglucerase‐Treated Patients With Gaucher Disease: Real‐World Evidence From the International Collaborative Gaucher Group ( ICGG ) Gaucher Registry Pregnancy Sub‐Registry
· 2026 · Related
Guanidinoacetate methyltransferase (GAMT) deficiency: Outcomes in 48 individuals and recommendations for diagnosis, treatment and monitoring
· Sylvia Stöckler‐Ipsiroglu · 2013 · 144 citations · Cited by this paper
How Much AI Can Scientific Publishing Tolerate?
· 2026 · Related
Guanidino compounds in serum, urine, liver, kidney, and brain of man and some ureotelic animals
· Bart Marescau · 1992 · 137 citations · Cited by this paper
Mapping Clinical Progression to Brain Atrophy in CLN2 Patients Under Cerliponase Alfa Treatment: A Prospective Neuroimaging Study
· 2026 · Related
Metabolic Control of Creatine Biosynthesis
· James B. Walker · 1960 · 124 citations · Cited by this paper
Immune Dysregulation in Branched Chain Organic Acidemias
· 2026 · Related
Repression of rat kidney L-arginine:glycine amidinotransferase synthesis by creatine at a pretranslational level.
· Denise M. McGuire · 1984 · 103 citations · Cited by this paper
Creatine transporter activity and content in the rat heart supplemented by and depleted of creatine
· Ernest A. Boehm · 2003 · 66 citations · Cited by this paper
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 · 2003 · 58 citations · Cited by this paper
In vivo cardiac 1H‐MRS in the mouse
· Jürgen E. Schneider · 2004 · 58 citations · Cited by this paper
Repression of arginine-glycine transamidinase activity by dietary creatine
· James B. Walker · 1959 · 53 citations · Cited by this paper
Brain creatine depletion in vegetarians? A cross-sectional 1 H-magnetic resonance spectroscopy ( 1 H-MRS) study
· Marina Yázigi Solis · 2013 · 47 citations · Cited by this paper
Biochemical and behavioural phenotyping of a mouse model for GAMT deficiency
· An Torremans · 2005 · 39 citations · Cited by this paper
Creatine uptake in brain and skeletal muscle of mice lacking guanidinoacetate methyltransferase assessed by magnetic resonance spectroscopy
· Hermien E. Kan · 2007 · 28 citations · Cited by this paper
Creatine uptake in mouse hearts with genetically altered creatine levels
· Michiel ten Hove · 2008 · 25 citations · Cited by this paper
Systemic availability of guanidinoacetate affects GABAA receptor function and seizure threshold in GAMT deficient mice
· Andreas Schulze · 2016 · 23 citations · Cited by this paper
Age-Dependent Decline in Cardiac Function in Guanidinoacetate-N-Methyltransferase Knockout Mice
· Dunja Aksentijević · 2020 · 18 citations · Cited by this paper
Contribution of monocarboxylate transporter 12 to blood supply of creatine on the sinusoidal membrane of the hepatocytes
· Ryuta Jomura · 2021 · 18 citations · Cited by this paper
Quantitative Studies on Enzyme Repression in the Developing Chick Embryo and Newly Hatched Chick.
· James B. Walker · 1963 · 9 citations · Cited by this paper
Influence of homoarginine on creatine accumulation and biosynthesis in the mouse
· Craig A. Lygate · 2022 · 7 citations · Cited by this paper
Magnetic resonance imaging reveals specific anatomical changes in the brain of Agat‐ and Gamt‐mice attributed to creatine depletion and guanidinoacetate alteration
· Ankit Kumar Sinha · 2020 · 5 citations · Cited by this paper
[18F]FDG-PET and [18F]MPPF-PET are brain biomarkers for the creatine transporter Slc6a8 loss of function mutation
· Isabel L Day · 2025 · 3 citations · Cited by this paper
Validation and Optimization of a Stable Isotope-Labeled Substrate Assay for Measuring AGAT Activity
· Alex Lee · 2024 · 1 citation · Cited by this paper
Source: OpenAlex (CC0)