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Keiko Kobayashi Selected Research

Intrahepatic Cholestasis

11/2012Effects of supplementation on food intake, body weight and hepatic metabolites in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse model of human citrin deficiency.
12/2011Metabolomic analysis reveals hepatic metabolite perturbations in citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mice, a model of human citrin deficiency.
8/2007Citrin/mitochondrial glycerol-3-phosphate dehydrogenase double knock-out mice recapitulate features of human citrin deficiency.
12/2005Variant clinical courses of 2 patients with neonatal intrahepatic cholestasis who have a novel mutation of SLC25A13.
10/2005Metabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier.
1/2005Frequency and distribution in East Asia of 12 mutations identified in the SLC25A13 gene of Japanese patients with citrin deficiency.
11/2004Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients.
10/2004Effects of citrin deficiency in the perinatal period: feasibility of newborn mass screening for citrin deficiency.
5/2003A novel inborn error of metabolism detected by elevated methionine and/or galactose in newborn screening: neonatal intrahepatic cholestasis caused by citrin deficiency.

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Keiko Kobayashi Research Topics

Disease

35Citrullinemia
01/2013 - 01/2002
28Neonatal-onset citrullinemia type 2
01/2013 - 01/2002
24Adult-onset citrullinemia type 2
01/2017 - 01/2002
13Hyperammonemia
12/2011 - 11/2002
9Intrahepatic Cholestasis
11/2012 - 05/2003
9Fatty Liver
03/2011 - 09/2002
6Necrosis
01/2016 - 01/2010
5Neoplasms (Cancer)
04/2022 - 04/2004
5Gas Gangrene (Gangrene, Gas)
10/2020 - 09/2012
5Brain Diseases (Brain Disorder)
11/2010 - 03/2005
5Cholestasis
04/2010 - 03/2005
5Galactosemias (Galactosemia)
08/2009 - 10/2005
4Hepatitis
01/2012 - 01/2008
4Cardiomegaly (Heart Hypertrophy)
03/2011 - 09/2002
4Neonatal hemochromatosis
01/2005 - 01/2002
3Atherosclerosis
01/2016 - 01/2013
3Liver Diseases (Liver Disease)
04/2010 - 02/2006
3Type 2 Diabetes Mellitus (MODY)
01/2009 - 01/2008
3Hypoglycemia (Reactive Hypoglycemia)
08/2007 - 12/2002
3Jaundice (Icterus)
04/2006 - 11/2002
2Inflammation (Inflammations)
01/2019 - 01/2015
2Cognitive Dysfunction
01/2019 - 01/2019
2Enterotoxemia
12/2016 - 11/2016
2Alzheimer Disease (Alzheimer's Disease)
01/2016 - 01/2013
2Non-alcoholic Fatty Liver Disease
01/2016 - 11/2008
2Infections
01/2016 - 04/2005
2Body Weight (Weight, Body)
11/2012 - 06/2009
2Failure to Thrive
12/2010 - 03/2007
2Hepatic Encephalopathy
01/2010 - 04/2004
2Metabolic Diseases (Metabolic Disease)
08/2009 - 06/2007
2Acute Liver Failure (Fulminant Hepatic Failure)
06/2009 - 11/2004
2Hypertrophy
01/2009 - 02/2006
2Proteinuria
01/2008 - 01/2007
2Hepatocellular Carcinoma (Hepatoma)
12/2007 - 10/2003
2Brain Edema (Cerebral Edema)
02/2006 - 03/2005
2Hypoproteinemia
10/2005 - 11/2004
2Coma (Comas)
10/2005 - 01/2002
1Hypoxia (Hypoxemia)
04/2022
1Male Infertility (Male Sterility)
03/2021
1VLCAD deficiency
02/2021
1Rhabdomyolysis
02/2021

Drug/Important Bio-Agent (IBA)

32citrinIBA
01/2017 - 01/2002
15aspartate-glutamate carrierIBA
01/2013 - 01/2002
13Proteins (Proteins, Gene)FDA Link
04/2022 - 05/2003
13CitrullineIBA
10/2012 - 01/2002
11DNA (Deoxyribonucleic Acid)IBA
01/2013 - 01/2002
11Urea (Carbamide)FDA LinkGeneric
11/2010 - 12/2002
9EnzymesIBA
01/2016 - 12/2002
8AmmoniaIBA
11/2010 - 01/2002
7Carnitine (L-Carnitine)FDA LinkGeneric
03/2011 - 09/2002
6CarbohydratesIBA
11/2012 - 05/2003
6Argininosuccinate Synthase (Argininosuccinate Synthetase)IBA
10/2005 - 12/2002
5Glycerol (Glycerine)FDA LinkGeneric
01/2017 - 01/2002
5Protein Isoforms (Isoforms)IBA
01/2013 - 01/2002
5Pyruvic Acid (Pyruvate)IBA
11/2012 - 08/2007
5Alanine Transaminase (SGPT)IBA
10/2012 - 06/2009
5Messenger RNA (mRNA)IBA
03/2011 - 09/2002
5Methionine (L-Methionine)FDA Link
12/2007 - 05/2003
4Virulence Factors (Pathogenicity Factors)IBA
10/2020 - 09/2012
4Amino AcidsFDA Link
11/2012 - 05/2003
4SodiumIBA
11/2012 - 01/2010
4Aspartate Aminotransferases (Aspartate Transaminase)IBA
10/2012 - 06/2009
4Bile Acids and Salts (Bile Acids)IBA
10/2012 - 10/2004
4Threonine (L-Threonine)FDA Link
10/2012 - 10/2005
4Galactose (Galactopyranose)FDA LinkGeneric
08/2009 - 05/2003
3Fatty Acids (Saturated Fatty Acids)IBA
02/2021 - 08/2006
3Sucrose (Saccharose)IBA
01/2017 - 08/2007
3CeramidesIBA
01/2016 - 06/2009
3Glycerolphosphate Dehydrogenase (Glycerol 3 Phosphate Dehydrogenase)IBA
11/2012 - 08/2007
3Therapeutic UsesIBA
12/2011 - 04/2004
3Arginine (L-Arginine)FDA Link
11/2010 - 11/2002
3NAD (NADH)IBA
08/2007 - 12/2002
2Toll-Like Receptor 4IBA
01/2021 - 01/2017
2CytokinesIBA
01/2019 - 01/2015
2Clostridium perfringens delta-toxin (CPDT)IBA
02/2018 - 01/2016
2Type C PhospholipasesIBA
01/2015 - 05/2013
2oxidized low density lipoproteinIBA
10/2014 - 01/2009
2Clostridium perfringens alpha toxinIBA
05/2013 - 09/2012
2Complementary DNA (cDNA)IBA
01/2013 - 04/2004
2Triglycerides (Triacylglycerol)IBA
11/2012 - 02/2006
2Glucose (Dextrose)FDA LinkGeneric
10/2012 - 03/2011
2OrnithineIBA
08/2009 - 12/2002
2Insulin (Novolin)FDA Link
07/2008 - 01/2008
2Lactic Acid (Lactate)FDA LinkGeneric
08/2007 - 04/2006
2Mannitol (Osmitrol)FDA LinkGeneric
02/2006 - 03/2005
2Systemic carnitine deficiencyIBA
02/2006 - 12/2002
2Aspartic Acid (Aspartate)FDA Link
04/2004 - 01/2002
2malic acid (malate)IBA
04/2004 - 01/2002
2Solute Carrier ProteinsIBA
12/2002 - 01/2002
1Doxorubicin (Adriamycin)FDA LinkGeneric
04/2022
1Oxygen (Dioxygen)IBA
04/2022
1SterolsIBA
03/2021
1BezafibrateIBA
02/2021

Therapy/Procedure

14Therapeutics
01/2019 - 01/2002
8Liver Transplantation
12/2010 - 01/2002
3Protein-Restricted Diet (Diet, Protein Restricted)
01/2010 - 11/2002
2Palliative Care (Palliative Therapy)
11/2015 - 10/2003
2Oral Administration
12/2011 - 06/2009
2Carbohydrate-Restricted Diet (Low Carbohydrate Diet)
11/2010 - 05/2003
2High-Protein Low-Carbohydrate Diet (Atkins Diet)
03/2007 - 05/2003