Hironori Nagasaka Selected Research

4-phenylbutyric acid (Buphenyl)

2/2016	Successful treatment with 4-phenylbutyrate in a patient with benign recurrent intrahepatic cholestasis type 2 refractory to biliary drainage and bilirubin absorption.
7/2014	Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1.
5/2014	Improved liver function and relieved pruritus after 4-phenylbutyrate therapy in a patient with progressive familial intrahepatic cholestasis type 2.
6/2012	AP2 adaptor complex mediates bile salt export pump internalization and modulates its hepatocanalicular expression and transport function.
5/2012	4-Phenylbutyrate modulates ubiquitination of hepatocanalicular MRP2 and reduces serum total bilirubin concentration.
6/2010	Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children.

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Hironori Nagasaka Research Topics

Disease

6	Cholestasis 02/2016 - 07/2007
6	Liver Diseases (Liver Disease) 09/2013 - 04/2004
6	Ornithine Carbamoyltransferase Deficiency Disease 07/2013 - 08/2004
5	Hypoglycemia (Reactive Hypoglycemia) 01/2018 - 10/2007
5	progressive familial intrahepatic 1 Cholestasis 07/2014 - 03/2005
4	Hypercholesterolemia 01/2018 - 07/2007
4	Intrahepatic Cholestasis 02/2016 - 10/2004
4	Pruritus (Itching) 02/2016 - 01/2009
4	Phenylketonurias (Phenylketonuria) 06/2014 - 07/2011
4	Hepatolenticular Degeneration (Wilson's Disease) 11/2012 - 04/2004
3	Neonatal-onset citrullinemia type 2 01/2019 - 05/2009
3	Congenital Hyperinsulinism 01/2018 - 01/2011
3	Hyperammonemia 06/2010 - 09/2006
2	Heart Failure 11/2019 - 01/2014
2	Platelet Glycoprotein IV Deficiency 01/2018 - 06/2011
2	Atherosclerosis 01/2018 - 03/2005
2	Insulin Resistance 01/2018 - 02/2012
2	Cholesteryl Ester Transfer Protein Deficiency 01/2014 - 08/2009
2	Bone Resorption 12/2013 - 11/2011
2	Headache (Headaches) 07/2013 - 08/2004
2	Hyperinsulinism (Hyperinsulinemia) 06/2013 - 01/2011
2	Hepatorenal form of glycogen storage disease 01/2013 - 10/2007
2	Inflammation (Inflammations) 11/2012 - 04/2004
2	Type 2 Diabetes Mellitus (MODY) 10/2012 - 02/2012
2	Liver Failure 08/2009 - 05/2005
2	Citrullinemia 05/2009 - 03/2009
2	Inborn Urea Cycle Disorders 03/2009 - 01/2007
2	Dyslipidemias (Dyslipidemia) 01/2009 - 06/2008
2	Inborn Genetic Diseases (Disease, Hereditary) 01/2009 - 10/2006
2	Acute Liver Failure (Fulminant Hepatic Failure) 01/2009 - 10/2006
2	Alagille Syndrome (Alagille-Watson Syndrome) 06/2008 - 03/2005
2	Vomiting 09/2006 - 08/2004
1	Cardiovascular Diseases (Cardiovascular Disease) 08/2018
1	Myalgia 01/2018
1	Premature Birth (Birth, Premature) 01/2018
1	Adult-onset citrullinemia type 2 01/2018
1	Neoplasms (Cancer) 11/2016
1	Diabetes Mellitus 09/2014
1	Neutral Lipid Storage Disease with Myopathy 01/2014
1	Fatty Liver 11/2012

Drug/Important Bio-Agent (IBA)

9	Triglycerides (Triacylglycerol)IBA 08/2018 - 07/2007
7	Urea (Carbamide)FDA LinkGeneric 01/2019 - 08/2004
6	4-phenylbutyric acid (Buphenyl)FDA Link 02/2016 - 06/2010
6	Lipoproteins (Lipoprotein)IBA 09/2013 - 03/2005
4	CopperIBA 11/2012 - 04/2004
4	AntioxidantsIBA 11/2012 - 10/2006
4	Pharmaceutical PreparationsIBA 06/2012 - 01/2009
3	CarbohydratesIBA 01/2019 - 08/2009
3	Glucose (Dextrose)FDA LinkGeneric 01/2018 - 01/2013
3	LipidsIBA 01/2018 - 05/2005
3	Insulin (Novolin)FDA Link 01/2018 - 02/2012
3	Bile Acids and Salts (Bile Acids)IBA 02/2016 - 06/2008
3	Member 11 Subfamily B ATP Binding Cassette TransporterIBA 02/2016 - 06/2012
3	Cholesterol Ester Transfer ProteinsIBA 01/2014 - 07/2007
3	oxidized low density lipoproteinIBA 09/2013 - 07/2007
3	LDL Lipoproteins (beta Lipoproteins)IBA 09/2013 - 07/2007
3	Arginine (L-Arginine)FDA Link 07/2013 - 09/2006
3	Oxidants (Oxidizing Agents)IBA 11/2012 - 10/2006
2	citrinIBA 01/2019 - 01/2018
2	Amino Acids FDA Link 01/2019 - 09/2006
2	CholesterolIBA 01/2018 - 07/2017
2	Fatty Acids (Saturated Fatty Acids)IBA 01/2018 - 06/2011
2	Hypoglycemic Agents (Hypoglycemics)IBA 01/2018 - 06/2011
2	Growth Hormone (Somatotropin)IBA 07/2017 - 09/2006
2	Blood Glucose (Blood Sugar)IBA 09/2014 - 01/2013
2	Lipase (Acid Lipase)FDA Link 01/2014 - 07/2007
2	Vitamin D FDA LinkGeneric 12/2013 - 02/2013
2	Phenylalanine (L-Phenylalanine)FDA Link 12/2013 - 07/2011
2	Monoclonal AntibodiesIBA 09/2013 - 09/2012
2	KATP ChannelsIBA 06/2013 - 01/2011
2	Mannose (D-Mannose)IBA 01/2013 - 07/2010
2	Peroxisome Proliferator-Activated Receptors (PPAR)IBA 11/2012 - 10/2012
2	EnzymesIBA 11/2012 - 10/2012
2	CalciumIBA 11/2011 - 10/2004
2	Adenosine Triphosphate (ATP)IBA 01/2011 - 03/2009
2	Free RadicalsIBA 01/2009 - 10/2006
2	HDL LipoproteinsIBA 03/2008 - 07/2007
2	Nitric Oxide (Nitrogen Monoxide)FDA Link 08/2004 - 08/2002
1	Aspartic Acid (Aspartate)FDA Link 01/2019
1	Amino Acid Transport System X-AG (Glutamate Transporter)IBA 01/2019
1	Lactic Acid (Lactate)FDA LinkGeneric 01/2018
1	Nonesterified Fatty Acids (NEFA)IBA 01/2018
1	AmmoniaIBA 01/2018
1	Biomarkers (Surrogate Marker)IBA 01/2018
1	SterolsIBA 01/2018
1	AsparagineIBA 11/2016
1	alpha-1,6-mannosylglycoprotein beta 1,6-N-acetylglucosaminyltransferaseIBA 11/2016
1	OligosaccharidesIBA 11/2016
1	GlycosyltransferasesIBA 11/2016
1	BilirubinIBA 02/2016
1	Phenylalanine Hydroxylase (Phenylalanine 4 Monooxygenase)IBA 06/2014
1	Therapeutic UsesIBA 05/2014
1	MetalsIBA 09/2013
1	Diazoxide (Hyperstat)FDA Link 06/2013
1	Octreotide (Sandostatin)FDA LinkGeneric 06/2013
1	Glucose-6-PhosphateIBA 01/2013

Therapy/Procedure

6	Therapeutics 07/2017 - 06/2003
2	Heart Transplantation (Grafting, Heart) 11/2019 - 01/2014
2	Pancreatectomy 01/2018 - 01/2011
1	Carbohydrate-Restricted Diet (Low Carbohydrate Diet) 01/2018