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Journal of Physiology, ISSN 0022-3751, 2013, Volume 591, Issue 2, pp. 571 - 592
Creatine (Cr) plays an important role in muscle energy homeostasis by its participation in the ATP-phosphocreatine phosphoryl exchange reaction mediated by... 
GUANIDINOACETATE METHYLTRANSFERASE DEFICIENCY | RESPIRATORY-CHAIN | PHYSIOLOGY | MAGNETIC-RESONANCE-SPECTROSCOPY | ACTIVATED PROTEIN-KINASE | HUMAN BRAIN | IN-VIVO | ACETYL-COA CARBOXYLASE | NEUROSCIENCES | HUMAN SKELETAL-MUSCLE | BETA-GUANIDINOPROPIONIC ACID | ORAL SUPPLEMENTATION | Speech Disorders - diet therapy | Developmental Disabilities - metabolism | Amino Acid Metabolism, Inborn Errors - physiopathology | Amidinotransferases - genetics | Muscle, Skeletal - metabolism | Amino Acid Metabolism, Inborn Errors - diet therapy | Muscle Fibers, Skeletal - metabolism | Mitochondria - ultrastructure | Intellectual Disability - metabolism | Proton-Translocating ATPases - metabolism | Speech Disorders - pathology | Amino Acid Metabolism, Inborn Errors - metabolism | Developmental Disabilities - pathology | Adenosine Triphosphate - metabolism | Creatine - deficiency | Amino Acid Metabolism, Inborn Errors - pathology | Intellectual Disability - diet therapy | Hand Strength | Developmental Disabilities - physiopathology | Speech Disorders - metabolism | Amidinotransferases - deficiency | Magnetic Resonance Spectroscopy | Intellectual Disability - pathology | Ischemia - metabolism | Lipid Metabolism | Muscular Atrophy - genetics | Mitochondria - metabolism | Amidinotransferases - metabolism | Speech Disorders - physiopathology | Mice, Knockout | Intellectual Disability - physiopathology | Phosphates - metabolism | Animals | Energy Metabolism | Muscle, Skeletal - physiopathology | Hindlimb - pathology | Muscle Fibers, Skeletal - pathology | Creatine Kinase - metabolism | Creatine - therapeutic use | Mice | Muscle, Skeletal - pathology | Developmental Disabilities - diet therapy | Hydrogen-Ion Concentration | Physiological aspects | Creatine | Homeostasis | Enzymes | Musculoskeletal system | Biosynthesis | Metabolism | Morphology | Index Medicus | Skeletal Muscle and Exercise
Journal Article
Neurobiology of Disease, ISSN 0969-9961, 2009, Volume 37, Issue 2, pp. 423 - 433
Journal Article
Molecular and Cellular Proteomics, ISSN 1535-9476, 05/2015, Volume 14, Issue 5, pp. 1400 - 1410
The data-independent acquisition (DIA) approach has recently been introduced as a novel mass spectrometric method that promises to combine the high content... 
DRUG-INDUCED HEPATOTOXICITY | ACCURATE | TARGETED MASS-SPECTROMETRY | PEPTIDE IDENTIFICATION | BIOCHEMICAL RESEARCH METHODS | VALIDATION | NORMALIZATION | ABSOLUTE QUANTIFICATION | PROTEINS | DISCOVERY | Glutamate Formimidoyltransferase - metabolism | Oncogene Proteins - genetics | Glutamate Formimidoyltransferase - genetics | Proteome - genetics | Humans | Analgesics, Non-Narcotic - pharmacology | Voltage-Dependent Anion Channel 2 - genetics | Amidinotransferases - genetics | Intracellular Signaling Peptides and Proteins - metabolism | Hepatocytes - metabolism | Peroxiredoxin VI - genetics | Peroxiredoxin VI - metabolism | Liver - drug effects | Acetaminophen - pharmacology | Proteolysis | Peroxiredoxin VI - analysis | Annexin A2 - analysis | Ammonia-Lyases - analysis | Proteomics - methods | Amidinotransferases - analysis | Oncogene Proteins - analysis | Intracellular Signaling Peptides and Proteins - genetics | Hepatocytes - drug effects | Gene Expression | Glutamate Formimidoyltransferase - analysis | Voltage-Dependent Anion Channel 2 - analysis | Ammonia-Lyases - genetics | Tissue Culture Techniques | Liver - metabolism | Intracellular Signaling Peptides and Proteins - analysis | Oncogene Proteins - metabolism | Ammonia-Lyases - metabolism | Proteome - analysis | Voltage-Dependent Anion Channel 2 - metabolism | Amidinotransferases - metabolism | Protein Deglycase DJ-1 | Annexin A2 - genetics | Annexin A2 - metabolism | Proteome - metabolism | Trypsin - chemistry | Peptides - analysis | Index Medicus | Research
Journal Article
Molecular Genetics and Metabolism, ISSN 1096-7192, 07/2013, Volume 109, Issue 3, pp. 260 - 268
Cerebral creatine deficiency syndromes (CCDS) are a group of inborn errors of creatine metabolism that involve and for creatine biosynthesis disorders and for... 
Creatine transporter defect | AGAT (Arginine:Glycine amidinotransferase) deficiency | GAMT (Guanidinoacetate Methyltransferase) deficiency | Cerebral creatine deficiency syndrome | GUANIDINOACETATE METHYLTRANSFERASE DEFICIENCY | MISSENSE VARIANTS | MEDICINE, RESEARCH & EXPERIMENTAL | GLYCINE AMIDINOTRANSFERASE DEFICIENCY | BIOCHEMISTRY & MOLECULAR BIOLOGY | TRANSPORTER GENE SLC6A8 | INBORN ERROR | GAMT DEFICIENCY | ENZYME ASSAY | LINKED MENTAL-RETARDATION | METABOLISM | AGAT DEFICIENCY | GENETICS & HEREDITY | Humans | Movement Disorders - diagnosis | Amidinotransferases - genetics | Male | Intellectual Disability - metabolism | Amino Acid Metabolism, Inborn Errors - genetics | Membrane Transport Proteins - genetics | Amidinotransferases - blood | Amidinotransferases - chemistry | Brain Diseases, Metabolic, Inborn - genetics | Creatine - genetics | Models, Molecular | Plasma Membrane Neurotransmitter Transport Proteins - metabolism | Guanidinoacetate N-Methyltransferase - metabolism | Amidinotransferases - metabolism | Plasma Membrane Neurotransmitter Transport Proteins - genetics | Phenotype | Protein Conformation | Movement Disorders - genetics | Mutation | Developmental Disabilities - metabolism | Guanidinoacetate N-Methyltransferase - genetics | Developmental Disabilities - genetics | Language Development Disorders - metabolism | Brain Diseases, Metabolic, Inborn - diagnosis | Intellectual Disability - genetics | Amino Acid Metabolism, Inborn Errors - diagnosis | Amino Acid Metabolism, Inborn Errors - metabolism | Mental Retardation, X-Linked - genetics | Language Development Disorders - diagnosis | Mental Retardation, X-Linked - diagnosis | Creatine - metabolism | Creatine - deficiency | Female | Developmental Disabilities - diagnosis | Guanidinoacetate N-Methyltransferase - blood | Speech Disorders - diagnosis | Plasma Membrane Neurotransmitter Transport Proteins - deficiency | Speech Disorders - metabolism | Amidinotransferases - deficiency | Brain Diseases, Metabolic, Inborn - metabolism | Language Development Disorders - genetics | Speech Disorders - genetics | Creatinine - urine | Syndrome | Movement Disorders - metabolism | Guanidinoacetate N-Methyltransferase - deficiency | Intellectual Disability - diagnosis | Movement Disorders - congenital | Mental Retardation, X-Linked - metabolism | Physiological aspects | Enzymes | Metabolites | Creatine | Index Medicus
Journal Article
Amino Acids, ISSN 0939-4451, 8/2016, Volume 48, Issue 8, pp. 2025 - 2039
Skeletal muscles require energy either at constant low (e.g., standing and posture) or immediate high rates (e.g., exercise). To fulfill these requirements,... 
Biochemistry, general | Alanine | Neurobiology | Pyruvate | Glucose | Creatine | Skeletal muscle | Life Sciences | Analytical Chemistry | Life Sciences, general | Biochemical Engineering | Proteomics | Arginine:glycine amidinotransferase (AGAT) | HOMEOSTASIS | PGC-1-ALPHA | BIOCHEMISTRY & MOLECULAR BIOLOGY | KINASE | EXPENDITURE | SKELETAL-MUSCLE | AMIDINOTRANSFERASE | GLUCONEOGENESIS | MAGNETIC-RESONANCE | Arginine: glycine amidinotransferase (AGAT) | AMPK | ENERGY-METABOLISM | Developmental Disabilities - metabolism | Transcriptome | Adipose Tissue, White - metabolism | Amidinotransferases - genetics | Muscle, Skeletal - metabolism | Developmental Disabilities - genetics | Phosphocreatine - genetics | Obesity - genetics | Intellectual Disability - genetics | Intellectual Disability - metabolism | Speech Disorders - pathology | Amino Acid Metabolism, Inborn Errors - metabolism | Developmental Disabilities - pathology | Amino Acid Metabolism, Inborn Errors - genetics | Amino Acid Metabolism, Inborn Errors - pathology | Adipose Tissue, White - pathology | Speech Disorders - metabolism | Amidinotransferases - deficiency | Obesity - chemically induced | Intellectual Disability - pathology | Oxidative Phosphorylation | Speech Disorders - genetics | Metabolome | Amidinotransferases - metabolism | Mice, Knockout | Obesity - metabolism | Obesity - pathology | Animals | Mice | Phosphocreatine - metabolism | Muscle, Skeletal - pathology | Index Medicus
Journal Article
Journal Article
FEBS Journal, ISSN 1742-464X, 12/2008, Volume 275, Issue 23, pp. 5899 - 5909
The creatine/creatine kinase system decreases drastically in sarcoma. In the present study, an investigation of catalytic activities, western blot and mRNA... 
normal muscle | arginine | sarcoma | methionine | creatine | Normal muscle | Arginine | Sarcoma | Creatine | Methionine | DNA METHYLATION | RAT-KIDNEY | BIOCHEMISTRY & MOLECULAR BIOLOGY | ORNITHINE-DECARBOXYLASE ACTIVITY | CARDIAC-CELLS | POLYAMINE METABOLISM | BREAST-CANCER | S-ADENOSYLMETHIONINE | SKELETAL-MUSCLE | GLYCINE AMIDINOTRANSFERASE | ASCITES TUMOR-CELLS | Neoplasms - metabolism | Liver - enzymology | Ornithine - blood | Guanidinoacetate N-Methyltransferase - genetics | Kidney - enzymology | Arginase - metabolism | Sarcoma 180 - pathology | Amidinotransferases - genetics | Muscle, Skeletal - metabolism | Carcinoma, Ehrlich Tumor - metabolism | Creatine - blood | Sarcoma 180 - metabolism | Creatinine - metabolism | Kidney - metabolism | Adenosylhomocysteinase - genetics | Membrane Transport Proteins - genetics | Enzymes - genetics | Creatine - metabolism | Female | Carcinoma, Ehrlich Tumor - pathology | Enzymes - metabolism | Gene Expression Regulation, Neoplastic - genetics | Ornithine Decarboxylase - genetics | Muscle, Skeletal - enzymology | Creatine - biosynthesis | Methionine - metabolism | Ornithine - metabolism | Liver - metabolism | Carcinoma, Ehrlich Tumor - enzymology | Neoplasms - enzymology | Arginase - genetics | Guanidinoacetate N-Methyltransferase - metabolism | Amidinotransferases - metabolism | Animals | 5-Methyltetrahydrofolate-Homocysteine S-Methyltransferase - genetics | Cell Line, Tumor | Creatinine - blood | Mice | Sarcoma 180 - enzymology | Neoplasms - pathology | Arginine - metabolism | Messenger RNA | Creatine kinase | Physiological aspects | Arginase | Amino acids | Polyamines | Biochemistry | Cellular biology | Kinases | Ribonucleic acid--RNA | Cancer | Index Medicus
Journal Article
Clinica Chimica Acta, ISSN 0009-8981, 09/2014, Volume 436, pp. 160 - 168
To develop an accurate stable isotope dilution assay for simultaneous quantification of creatine metabolites ornithine, arginine, creatine, creatinine, and... 
Stable isotope dilution | Tandem mass spectrometry | Creatine deficiency syndromes | Mouse models | Creatine metabolites | GUANIDINOACETATE METHYLTRANSFERASE DEFICIENCY | DIAGNOSIS | TANDEM MASS-SPECTROMETRY | CHROMATOGRAPHY | GC-MS | TRANSPORT | GAMT DEFICIENCY | AMIDINOTRANSFERASE | CLINICAL-CHEMISTRY | URINE | MEDICAL LABORATORY TECHNOLOGY | Developmental Disabilities - blood | Language Development Disorders - blood | Developmental Disabilities - metabolism | Humans | Language Development Disorders - metabolism | Creatine - blood | Intellectual Disability - metabolism | Intellectual Disability - blood | Isotopes - chemistry | Tandem Mass Spectrometry | Amino Acid Metabolism, Inborn Errors - metabolism | Plasma - metabolism | Dried Blood Spot Testing - methods | Amidinotransferases - blood | Creatine - metabolism | Chromatography, Liquid | Creatine - deficiency | Guanidinoacetate N-Methyltransferase - blood | Speech Disorders - blood | Disease Models, Animal | Speech Disorders - metabolism | Amidinotransferases - deficiency | Limit of Detection | Reproducibility of Results | Linear Models | Guanidinoacetate N-Methyltransferase - metabolism | Movement Disorders - blood | Amidinotransferases - metabolism | Amino Acid Metabolism, Inborn Errors - blood | Movement Disorders - metabolism | Animals | Guanidinoacetate N-Methyltransferase - deficiency | Movement Disorders - congenital | Mice | Metabolites | Creatine | Models | Index Medicus
Journal Article
Journal Article
The American Journal of Human Genetics, ISSN 0002-9297, 2001, Volume 69, Issue 5, pp. 1127 - 1133
Journal Article
Proteins: Structure, Function, and Bioinformatics, ISSN 0887-3585, 01/2017, Volume 85, Issue 1, pp. 103 - 116
The tunneling‐fold (T‐fold) structural superfamily has emerged as a versatile protein scaffold of diverse catalytic activities. This is especially evident in... 
7‐cyano‐7‐deazaguanine | 7‐deazaguanosine | thioimide | T‐fold | amidinotransferase | transfer‐RNA | preQ | modified nucleoside | QueF‐L | tunneling‐fold enzyme | 7-deazaguanosine | tunneling-fold enzyme | transfer-RNA | T-fold | QueF-L | 7-cyano-7-deazaguanine | MECHANISM | TETRAHYDROBIOPTERIN BIOSYNTHESIS | BIOCHEMISTRY & MOLECULAR BIOLOGY | ESCHERICHIA-COLI | NUCLEOSIDE-Q | ENZYMATIC-SYNTHESIS | QUEUOSINE | BIOPHYSICS | GTP CYCLOHYDROLASE-I | GUANINE TRANSGLYCOSYLASE | ARCHAEAL TRANSFER-RNA | 6-CARBOXY-5,6,7,8-TETRAHYDROPTERIN SYNTHASE | Protein Multimerization | RNA, Archaeal - chemistry | Archaeal Proteins - chemistry | Substrate Specificity | Amidinotransferases - genetics | Crystallography, X-Ray | Pyrimidinones - metabolism | Protein Subunits - metabolism | Pyrimidinones - chemistry | Guanosine - chemistry | Cloning, Molecular | Escherichia coli - metabolism | RNA, Transfer - genetics | Archaeal Proteins - genetics | Protein Interaction Domains and Motifs | Amidinotransferases - chemistry | RNA, Archaeal - genetics | RNA, Transfer - chemistry | Protein Subunits - genetics | Archaeal Proteins - metabolism | Guanosine - analogs & derivatives | Recombinant Proteins - metabolism | Amino Acid Sequence | Protein Conformation, alpha-Helical | Pyrroles - metabolism | Catalytic Domain | Gene Expression | RNA Processing, Post-Transcriptional | Pyrobaculum - enzymology | RNA, Transfer - metabolism | Recombinant Proteins - chemistry | Recombinant Proteins - genetics | Guanosine - metabolism | Amidinotransferases - metabolism | Sequence Homology, Amino Acid | Sequence Alignment | Protein Conformation, beta-Strand | Escherichia coli - genetics | RNA, Archaeal - metabolism | Protein Binding | Pyrroles - chemistry | Molecular Docking Simulation | Protein Subunits - chemistry | Pyrobaculum - genetics | Enzymes | Cysteine | Analysis | Crystals | Physiological aspects | Nitriles | Phylogeny | Structure | Transfer RNA | Index Medicus | Tunneling-fold enzyme | preQ0
Journal Article