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Georg F Hoffmann Selected Research

Histidine (L-Histidine)

7/2016CNDP1 genotype and renal survival in pediatric nephropathies.
11/2015Carnosine metabolism in diabetes is altered by reactive metabolites.
1/2011Anserine inhibits carnosine degradation but in human serum carnosinase (CN1) is not correlated with histidine dipeptide concentration.

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Georg F Hoffmann Research Topics

Disease

19Phenylketonurias (Phenylketonuria)
10/2021 - 03/2002
12Propionic Acidemia
04/2024 - 10/2004
11Brain Diseases (Brain Disorder)
01/2022 - 01/2003
10Inborn Urea Cycle Disorders
12/2023 - 09/2005
9Methylmalonic acidemia
04/2024 - 04/2002
9Metabolic Diseases (Metabolic Disease)
01/2024 - 07/2002
8Dystonia (Limb Dystonia)
08/2017 - 07/2002
7Seizures (Absence Seizure)
01/2019 - 02/2003
7Inborn Errors Metabolism (Inborn Errors of Metabolism)
09/2017 - 11/2002
6Homocystinuria
04/2024 - 09/2007
6Vitamin B 12 Deficiency (Vitamin B12 Deficiency)
07/2023 - 04/2017
6Intellectual Disability (Idiocy)
01/2019 - 12/2011
5Aromatic amino acid decarboxylase deficiency
01/2024 - 03/2004
5Cystic Fibrosis (Mucoviscidosis)
01/2024 - 10/2010
5Hyperammonemia
11/2023 - 05/2011
5Epilepsy (Aura)
10/2022 - 04/2005
5Medium chain acyl CoA dehydrogenase deficiency
06/2022 - 10/2002
5due to Sepiapterin Reductase Deficiency Dopa-Responsive Dystonia
11/2021 - 11/2006
5Mevalonate Kinase Deficiency
01/2021 - 02/2003
5Atrophy
01/2021 - 05/2002
5Muscle Hypotonia (Hypotonia)
01/2021 - 01/2007
4Citrullinemia
12/2023 - 09/2018
4Mitochondrial Diseases (Mitochondrial Disease)
11/2023 - 08/2006
4Multiple Acyl Coenzyme A Dehydrogenase Deficiency
11/2023 - 10/2008
4Hypoglycemia (Reactive Hypoglycemia)
06/2022 - 10/2002
4Neurodevelopmental Disorders
01/2022 - 03/2016
4Movement Disorders (Movement Disorder)
01/2021 - 10/2004
4B BH4-Deficient Hyperphenylalaninemia
09/2020 - 06/2011
4Acute Liver Failure (Fulminant Hepatic Failure)
01/2019 - 01/2009
4Congenital, Hereditary, and Neonatal Diseases and Abnormalities (Congenital Disorders)
01/2019 - 03/2002
4Congenital Disorders of Glycosylation
01/2018 - 11/2010
3Methylenetetrahydrofolate reductase deficiency
04/2024 - 10/2018
3Ornithine Carbamoyltransferase Deficiency Disease
12/2023 - 09/2011
3Infections
05/2023 - 01/2016
3Fever (Fevers)
05/2023 - 06/2004
3Nervous System Diseases (Neurological Disorders)
03/2021 - 04/2005
3autosomal recessive Segawa syndrome
01/2021 - 01/2003
3Diabetic Nephropathies (Diabetic Nephropathy)
07/2020 - 11/2015
3Glutaric Acidemia I
01/2017 - 05/2002
3Neoplasms (Cancer)
04/2016 - 04/2013
3Cerebellar Ataxia (Dysmetria)
02/2016 - 02/2003
3Dopa-responsive Dystonia
03/2013 - 10/2007
2Argininosuccinic Aciduria
12/2023 - 01/2019
2Spinal Muscular Atrophy (Progressive Muscular Atrophy)
10/2021 - 01/2019
2Folic Acid Deficiency (Folic Acid Deficiencies)
06/2021 - 09/2007
2Cognitive Dysfunction
01/2021 - 01/2019
2Liver Failure
11/2020 - 01/2020
2Muscular Diseases (Myopathy)
10/2020 - 03/2002
26-pyruvoyl-tetrahydropterin synthase deficiency
09/2020 - 03/2008
2Glycogen Storage Disease Type II (Pompe's Disease)
01/2020 - 08/2002
2Gaucher Disease (Gaucher's Disease)
01/2020 - 09/2017
2Guanidinoacetate methyltransferase deficiency
01/2020 - 02/2003
2Globoid Cell Leukodystrophy (Krabbe Disease)
10/2019 - 09/2017
2Tuberous Sclerosis (Bourneville's Disease)
01/2019 - 10/2016

Drug/Important Bio-Agent (IBA)

15EnzymesIBA
12/2023 - 03/2002
12Neurotransmitter Agents (Neurotransmitter)IBA
07/2023 - 01/2003
11Proteins (Proteins, Gene)FDA Link
12/2023 - 02/2010
11AcidsIBA
01/2013 - 04/2002
9Phenylalanine Hydroxylase (Phenylalanine 4 Monooxygenase)IBA
09/2020 - 04/2003
8Phenylalanine (L-Phenylalanine)FDA Link
10/2021 - 03/2002
7Vitamin B 12 (Cyanocobalamin)FDA LinkGeneric
04/2024 - 09/2007
7Levodopa (L Dopa)FDA LinkGeneric
07/2023 - 07/2002
7Fatty Acids (Saturated Fatty Acids)IBA
06/2022 - 10/2002
5Dopamine (Intropin)FDA LinkGeneric
01/2024 - 07/2002
5Serotonin (5 Hydroxytryptamine)IBA
01/2024 - 07/2002
5Methylmalonic AcidIBA
01/2023 - 08/2007
5CholesterolIBA
01/2021 - 02/2003
4Oxidoreductases (Dehydrogenase)IBA
01/2024 - 04/2005
4Carnitine (L-Carnitine)FDA LinkGeneric
11/2023 - 11/2002
46-pyruvoyltetrahydropterin synthase (PTPS)IBA
11/2021 - 03/2008
4HomocysteineIBA
10/2020 - 03/2010
4sapropterin (tetrahydrobiopterin)FDA Link
09/2020 - 03/2008
4Coenzyme A (CoA)IBA
01/2019 - 10/2010
4Vitamin DFDA LinkGeneric
10/2017 - 01/2009
4Lysine (L-Lysine)FDA Link
01/2017 - 09/2012
3CystathionineIBA
04/2024 - 01/2017
3Pancreatitis-Associated ProteinsIBA
01/2024 - 10/2010
3Amino Acyl-tRNA Synthetases (Aminoacyl-tRNA Synthetase)IBA
11/2023 - 08/2016
3IronIBA
01/2022 - 10/2017
3Amino AcidsFDA Link
01/2020 - 06/2011
3GlycoconjugatesIBA
01/2018 - 03/2012
3Histidine (L-Histidine)FDA Link
07/2016 - 01/2011
3DipeptidesIBA
07/2016 - 01/2011
2Norepinephrine (Noradrenaline)FDA LinkGeneric
01/2024 - 01/2017
2Epinephrine (Adrenaline)FDA LinkGeneric
01/2024 - 01/2017
2CitrullineIBA
12/2023 - 11/2023
2Arginine (L-Arginine)FDA Link
12/2023 - 09/2012
2Argininosuccinate Synthase (Argininosuccinate Synthetase)IBA
11/2023 - 01/2019
2Biomarkers (Surrogate Marker)IBA
11/2023 - 10/2021
2Vitamin B 6IBA
07/2023 - 01/2017
2Biogenic Amines (Biogenic Amine)IBA
07/2023 - 02/2004
2Acyl-CoA Dehydrogenase (Medium-Chain Acyl-Coenzyme A Dehydrogenase)IBA
06/2022 - 10/2002
2Aromatic-L-Amino-Acid DecarboxylasesIBA
01/2021 - 04/2015
2Transaminases (Aminotransferases)IBA
11/2020 - 12/2017
2Systemic carnitine deficiencyIBA
10/2020 - 10/2010
2GTP Cyclohydrolase (GTP Cyclohydrolase I)IBA
09/2020 - 03/2013

Therapy/Procedure

17Therapeutics
12/2023 - 11/2004
6Liver Transplantation
12/2023 - 09/2005
2Enzyme Replacement Therapy
01/2020 - 01/2019
2Emergency Treatment
01/2019 - 01/2014