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Akio Kihara Selected Research

Ichthyosis (Xeroderma)

4/2021Comprehensive stratum corneum ceramide profiling reveals reduced acylceramides in ichthyosis patient with CERS3 mutations.
1/2021Impaired production of the skin barrier lipid acylceramide by CYP4F22 ichthyosis mutations.
11/2020Skin permeability barrier formation by the ichthyosis-causative gene FATP4 through formation of the barrier lipid ω-O-acylceramide.
1/2020Severe Skin Permeability Barrier Dysfunction in Knockout Mice Deficient in a Fatty Acid ω-Hydroxylase Crucial to Acylceramide Production.
12/2019Reduced chain length in myelin sphingolipids and poorer motor coordination in mice deficient in the fatty acid elongase Elovl1.
1/2019The role of PNPLA1 in ω-O-acylceramide synthesis and skin barrier function.
1/2018Decreased Skin Barrier Lipid Acylceramide and Differentiation-Dependent Gene Expression in Ichthyosis Gene Nipal4-Knockout Mice.
11/2017Biallelic Mutations in KDSR Disrupt Ceramide Synthesis and Result in a Spectrum of Keratinization Disorders Associated with Thrombocytopenia.
1/2017PNPLA1 is a transacylase essential for the generation of the skin barrier lipid ω-O-acylceramide.
6/2015Essential role of the cytochrome P450 CYP4F22 in the production of acylceramide, the key lipid for skin permeability barrier formation.
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Akio Kihara Research Topics

Disease

11Ichthyosis (Xeroderma)
04/2021 - 02/2014
6Dry Eye Syndromes (Dry Eye Syndrome)
01/2022 - 01/2018
4Sjogren-Larsson Syndrome
01/2021 - 05/2012
3Neoplasms (Cancer)
01/2021 - 11/2006
3Muscular Diseases (Myopathy)
10/2015 - 12/2013
2Neurocutaneous Syndromes (Phakomatoses)
01/2021 - 12/2019
2Inflammation (Inflammations)
10/2020 - 11/2012
2Paraplegia (Spastic Paraplegia)
02/2020 - 12/2019
2Starvation
01/2020 - 08/2013
2Lymphopenia (Lymphocytopenia)
11/2018 - 09/2008
2Intellectual Disability (Idiocy)
02/2014 - 12/2013
2Macular Degeneration (Age-Related Maculopathy)
02/2014 - 11/2010
2Dehydration (Water Stress)
12/2013 - 05/2012
1Meibomian Gland Dysfunction
01/2021
1Neuroinflammatory Diseases
10/2020
1Brain Ischemia (Cerebral Ischemia)
10/2020
1Infarction (Infarctions)
10/2020
1Ischemia
10/2020
1Reperfusion Injury
10/2020
1Stroke (Strokes)
10/2020
1Neurologic Gait Disorders (Gait, Hemiplegic)
02/2020
1Fatty Acid Hydroxylase-Associated Neurodegeneration
02/2020
1Atopic Dermatitis (Atopic Eczema)
01/2020
1Deafness (Deaf Mutism)
12/2019
1Rupture
01/2019
1Nervous System Diseases (Neurological Disorders)
01/2019
1Chronic Obstructive Pulmonary Disease (COPD)
11/2018
1Emphysema
11/2018
1Pneumococcal Infections
11/2018
1Pulmonary Emphysema
11/2018
1Infections
01/2018
1Thrombocytopenia (Thrombopenia)
11/2017
1Hypersensitivity (Allergy)
01/2017
1Insulin Resistance
04/2016
1Sphingolipidoses (Sphingolipidosis)
04/2016
1Fibrosis (Cirrhosis)
01/2016
1Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)
03/2014
1Peroxisomal Disorders (Peroxisomal Disorder)
03/2014
1Experimental Autoimmune Encephalomyelitis (Encephalomyelitis, Autoimmune Experimental)
02/2014
1Demyelinating Diseases (Demyelinating Disease)
02/2014
1Stargardt disease 3
11/2010
1Neuroblastoma
05/2009
1Teratocarcinoma
09/2008
1Carcinoma (Carcinomatosis)
06/2005

Drug/Important Bio-Agent (IBA)

14LipidsIBA
01/2022 - 11/2006
8EnzymesIBA
01/2019 - 06/2005
6SphingolipidsIBA
01/2019 - 11/2012
5Fatty Acids (Saturated Fatty Acids)IBA
03/2014 - 05/2009
4Fatty Acid ElongasesIBA
01/2021 - 01/2018
4sphingosine 1-phosphate (sphingosine-1-phosphate)IBA
11/2018 - 11/2006
3long-chain-aldehyde dehydrogenaseIBA
01/2021 - 05/2016
3sphingosine kinaseIBA
09/2008 - 06/2005
2Proteins (Proteins, Gene)FDA Link
04/2021 - 01/2019
2CeramidesIBA
04/2021 - 11/2017
2Fingolimod Hydrochloride (FTY720)FDA Link
10/2020 - 09/2008
2CarbonIBA
01/2020 - 08/2013
2Glycerophospholipids (Phosphoglycerides)IBA
01/2019 - 11/2012
2Mutant Proteins (Protein, Mutant)IBA
06/2015 - 11/2010
2Membrane LipidsIBA
12/2013 - 11/2012
2Oxidoreductases (Dehydrogenase)IBA
12/2013 - 05/2012
1Fatty AlcoholsIBA
01/2022
1hexadecanal dehydrogenase (acylating) (fatty acyl-CoA reductase)IBA
01/2022
1Acyl Coenzyme A (Acyl CoA)IBA
05/2021
1AcyltransferasesIBA
05/2021
1Nonsense Codon (Nonsense Mutation)IBA
04/2021
1dihydroceramide desaturaseIBA
04/2021
1LigandsIBA
01/2021
1Monoclonal AntibodiesIBA
01/2021
1Ophthalmic Solutions (Eye Drops)IBA
01/2021
1Cholesterol Esters (Cholesteryl Esters)IBA
01/2021
1Mineral Oil (Liquid Paraffin)FDA LinkGeneric
01/2021
1amlexanox (Aphthasol)FDA Link
01/2021
1EstersIBA
01/2021
1N,N- diethyl- 2- (2- (4- (2- fluoroethoxy)phenyl)- 5,7- dimethylpyrazolo(1,5- a)pyrimidin- 3- yl)acetamideIBA
10/2020
1Tight Junction ProteinsIBA
10/2020
1IronIBA
02/2020
1AllergensIBA
01/2020
1DioxygenasesIBA
01/2020
1ceralifimodIBA
11/2018
1Pancreatic Elastase (Elastase)IBA
11/2018
1Sphingosine-1-Phosphate ReceptorsIBA
11/2018
1SmokeIBA
11/2018
1AM 251 (AM251)IBA
01/2016
1trierucateIBA
03/2014
1Lorenzo's oilIBA
03/2014
1IntegrinsIBA
02/2014
1Messenger RNA (mRNA)IBA
12/2013
1isoprocarbIBA
08/2013
1Sodium AzideIBA
08/2013
1Biological ProductsIBA
08/2013
1NitrogenIBA
08/2013
1Calcium Chelating AgentsIBA
08/2013
1Retinaldehyde (Retinal)IBA
11/2012
1Phosphoric Monoester Hydrolases (Phosphatases)IBA
05/2012
1PalmitatesIBA
05/2009
1octanoic acid (caprylic acid)IBA
05/2009
1Immunologic Factors (Immunomodulators)IBA
09/2008

Therapy/Procedure

1Immunotherapy
01/2021
1Thrombectomy
10/2020
1Canes (Cane)
02/2020