Dwight D Koeberl Selected Research

Glucose-6-Phosphatase (Glucose 6 Phosphatase)

6/2023	Genome editing using Staphylococcus aureus Cas9 in a canine model of glycogen storage disease Ia.
1/2020	Fenofibrate rapidly decreases hepatic lipid and glycogen storage in neonatal mice with glycogen storage disease type Ia.
12/2019	Pathogenesis of Hepatic Tumors following Gene Therapy in Murine and Canine Models of Glycogen Storage Disease.
10/2019	Gene therapy for glycogen storage diseases.
6/2019	Bezafibrate Enhances AAV Vector-Mediated Genome Editing in Glycogen Storage Disease Type Ia.
1/2019	Bezafibrate induces autophagy and improves hepatic lipid metabolism in glycogen storage disease type Ia.
4/2016	In Vivo Zinc Finger Nuclease-mediated Targeted Integration of a Glucose-6-phosphatase Transgene Promotes Survival in Mice With Glycogen Storage Disease Type IA.
2/2016	Induction of autophagy improves hepatic lipid metabolism in glucose-6-phosphatase deficiency.
4/2012	Long-term efficacy following readministration of an adeno-associated virus vector in dogs with glycogen storage disease type Ia.
11/2011	Hepatorenal correction in murine glycogen storage disease type I with a double-stranded adeno-associated virus vector.

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Dwight D Koeberl Research Topics

Disease

37	Glycogen Storage Disease Type II (Pompe's Disease) 09/2023 - 04/2003
18	Hepatorenal form of glycogen storage disease 06/2023 - 07/2007
13	Hypoglycemia (Reactive Hypoglycemia) 07/2023 - 07/2007
8	Glycogen Storage Disease Type I 07/2023 - 07/2007
5	Glycogen Storage Disease (Glycogenosis) 07/2023 - 11/2012
4	Muscular Diseases (Myopathy) 07/2023 - 09/2015
4	Carcinoma (Carcinomatosis) 07/2023 - 11/2007
3	Adenoma (Adenomas) 07/2023 - 11/2007
3	Hepatocellular Carcinoma (Hepatoma) 06/2023 - 08/2009
3	Non-alcoholic Fatty Liver Disease 01/2019 - 01/2017
3	Seizures (Absence Seizure) 05/2018 - 08/2003
2	Cardiomyopathies (Cardiomyopathy) 07/2023 - 11/2007
2	Disease Progression 06/2023 - 01/2018
2	Respiratory Insufficiency (Respiratory Failure) 01/2021 - 11/2005
2	Spasm (Spasms) 01/2020 - 01/2018
2	Liver Cell Adenoma (Hepatocellular Adenoma) 01/2020 - 11/2007
2	Lactic Acidosis 01/2020 - 09/2015
2	Tremor (Tremors) 01/2020 - 01/2018
2	Hypertrophy 01/2020 - 05/2016
2	Guanidinoacetate methyltransferase deficiency 05/2018 - 06/2013
2	Weight Gain 02/2016 - 06/2013
2	Muscle Weakness 02/2016 - 11/2005
2	Short chain Acyl CoA dehydrogenase deficiency 11/2003 - 08/2003
1	Hepatitis 09/2023
1	Chronic Kidney Failure (Chronic Renal Failure) 07/2023
1	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) 06/2023
1	Rare Diseases (Rare Disease) 01/2023
1	Pyruvate Carboxylase Deficiency Disease 01/2023
1	Hemolytic Anemia 01/2022
1	Hemiplegia (Spastic Hemiplegia) 01/2021
1	Fabry Disease (Fabry's Disease) 01/2020
1	Fatty Liver 01/2020
1	Hypertriglyceridemia 01/2020
1	Neoplasms (Cancer) 12/2019
1	Urolithiasis 11/2018
1	Movement Disorders (Movement Disorder) 05/2018
1	Ataxia (Dyssynergia) 01/2018
1	Muscle Hypotonia (Hypotonia) 01/2018
1	Cardiomegaly (Heart Hypertrophy) 11/2017

Drug/Important Bio-Agent (IBA)

23	AcidsIBA 01/2021 - 04/2003
22	GlycogenIBA 07/2023 - 01/2005
16	EnzymesIBA 09/2023 - 12/2006
14	GlucosidasesIBA 06/2020 - 11/2010
13	Glucose-6-Phosphatase (Glucose 6 Phosphatase)IBA 06/2023 - 07/2007
8	human GAA protein (Myozyme)FDA Link 01/2021 - 12/2008
8	alpha-Glucosidases (Acid Maltase)IBA 01/2021 - 04/2003
6	Glucose (Dextrose)FDA LinkGeneric 07/2023 - 04/2008
6	ClenbuterolIBA 06/2023 - 06/2011
6	IGF Type 2 Receptor (Insulin-Like-Growth-Factor II Receptor)IBA 01/2019 - 11/2010
4	Albuterol (Salbutamol)FDA LinkGeneric 01/2020 - 02/2012
4	CationsIBA 01/2019 - 01/2013
2	Propranolol (Inderal)FDA LinkGeneric 01/2020 - 02/2016
2	Liver GlycogenIBA 01/2020 - 11/2012
2	LipidsIBA 01/2020 - 02/2016
2	Biomarkers (Surrogate Marker)IBA 01/2020 - 01/2005
2	Insulin-Like PeptidesIBA 01/2020 - 06/2013
2	BezafibrateIBA 06/2019 - 01/2019
2	Salmeterol Xinafoate (Serevent)FDA Link 01/2019 - 05/2016
2	Glucose-6-PhosphateIBA 01/2019 - 02/2016
2	glycocyamineIBA 05/2018 - 06/2013
2	CreatineIBA 05/2018 - 01/2018
2	Guanidinoacetate N-Methyltransferase (Guanidinoacetate Methyltransferase)IBA 05/2018 - 06/2013
2	Carnitine (L-Carnitine)FDA LinkGeneric 09/2015 - 08/2003
2	AntibodiesIBA 03/2015 - 02/2010
2	HepcidinsIBA 05/2010 - 04/2010
2	acylcarnitineIBA 11/2003 - 08/2003
2	Butyryl-CoA Dehydrogenase (Short-Chain Acyl-CoA Dehydrogenase)IBA 11/2003 - 08/2003
1	Alanine Transaminase (SGPT)IBA 09/2023
1	Antisense OligonucleotidesIBA 01/2023
1	Bortezomib (Velcade)FDA Link 01/2023
1	triheptanoinIBA 01/2023
1	Neutralizing AntibodiesIBA 01/2023
1	Triose-Phosphate Isomerase (Triosephosphate Isomerase)IBA 01/2022
1	RNA (Ribonucleic Acid)IBA 01/2021
1	Triglycerides (Triacylglycerol)IBA 01/2020
1	globotriaosylceramide (globotriosylceramide)IBA 01/2020
1	globotriaosyl lysosphingolipidIBA 01/2020
1	Carvedilol (Coreg)FDA LinkGeneric 01/2020
1	Antihypertensive Agents (Antihypertensives)IBA 01/2020
1	GalactosidasesIBA 01/2020
1	Fenofibrate (CiL)FDA LinkGeneric 01/2020
1	Losartan (Cozaar)FDA LinkGeneric 01/2020
1	Therapeutic UsesIBA 01/2020
1	Fetal Proteins (Fetoprotein)IBA 12/2019
1	Tumor Biomarkers (Tumor Markers)IBA 12/2019
1	2- ((3,5- dimethyl- 4- (4'- hydroxy- 3'- isopropylbenzyl)phenoxy)methyl)- 4- (3- chlorophenyl)- 2- oxido(1,3,2)dioxaphosphonaneIBA 01/2019
1	Calcium OxalateIBA 11/2018
1	calcium phosphateIBA 11/2018
1	Arginine (L-Arginine)FDA Link 01/2018
1	Nonsense Codon (Nonsense Mutation)IBA 01/2018
1	MM Form Creatine KinaseIBA 01/2018
1	Glycine (Aminoacetic Acid)FDA LinkGeneric 01/2018
1	CarbohydratesIBA 01/2017
1	Insulin-Like Growth Factor I (IGF-1)IBA 05/2016
1	Zinc Finger NucleasesIBA 04/2016

Therapy/Procedure

24	Therapeutics 07/2023 - 11/2010
17	Enzyme Replacement Therapy 01/2020 - 11/2007
2	Injections 01/2021 - 02/2012
2	Liver Transplantation 09/2009 - 11/2007
1	Contraindications 01/2020