Faculty Information - Junko Matsuda

Present specialized field

BooK

Journal

Presentation

Winning science prize

（Last updated : 2024-04-17 11:45:06）

Junko Matsuda
Department Kawasaki Medical School Kawasaki Medical School, Department of Pathophysiology and Metabolism,

Position Professor

■ Present specialized field

Embryonic medicine and pediatrics, Neuroscience-general, Structural biochemistry

■ BooK

1.	2008		Sphingolipid activator proteins, Experimental Glycoscience: glycobiology
2.	2007/01		Krabbe disease (Globoid cell leukodystrophy)., Lysosomal Storage Disorders.

■ Journal

1.	2023/06/20	Genetic ablation of Saposin-D in Krabbe disease eliminates psychosine accumulation but does not significantly improve demyelination
2.	2023/04/01	Bifunctional DEGS2 has higher hydroxylase activity toward substrates with very-long-chain fatty acids in the production of phytosphingosine ceramides.
3.	2023/01/01	Mass Spectrometry of Neutral Glycosphingolipids
4.	2022/12	The Urinary Bladder is Rich in Glycosphingolipids Composed of Phytoceramides
5.	2021/05/24	Involvement of acid ceramidase in the degradation of bioactive N-acylethanolamines.
6.	2021/05/12	Prevalence of patients with lysosomal storage disorders and peroxisomal disorders: A nationwide survey in Japan
7.	2021/04/06	Reduction in miR-219 expression underlies cellular pathogenesis of oligodendrocytes in a mouse model of Krabbe disease
8.	2020/06/25	A Case of Adrenoleukodystrophy Diagnosed Based on Visual Disturbance
9.	2020	Variants in saposin D domain of prosaposin gene linked to Parkinson's disease.
10.	2019/01	Novel neuroblastoma amplified sequence (NBAS) mutations in a Japanese boy with fever-triggered recurrent acute liver failure.
11.	2018	Developmental defects and aberrant accumulation of endogenous psychosine in oligodendrocytes in a murine model of Krabbe disease.
12.	2017/01	A case of sitosterolemia due to compound heterozygous mutations in ABCG5: clinical features and treatment outcomes obtained with colestimide and ezetimibe
13.	2016/08	Numerous intertriginous xanthomas in infant: A diagnostic clue for sitosterolemia
14.	2015/06	The effects of chemically synthesized saposin C on glucosylceramide-beta-glucosidase
15.	2014/07	Mesotrypsin and caspase-14 participate in prosaposin processing: potential relevance to epidermal permeability barrier formation.
16.	2013/01	Improved high-fat diet-induced glucose intolerance by an oral administration of phytosphingosine.
17.	2012/11	Chemoenzymatic synthesis of hydrophobic glycoprotein: synthesis of saposin C carrying complex-type carbohydrate.
18.	2012/10	Primary polydipsia, but not accumulated ceramide, causes lethal renal damage in saposin D-deficient mice.
19.	2012/04	Endosomal sorting by Semaphorin 4A in retinal pigment epithelium supports photoreceptor survival.
20.	2011/11	High-performance thin-layer chromatography/mass spectrometry for the analysis of neutral glycosphingolipids.
21.	2011/02	Synthesis of the sphingolipid activator protein, saposin C, using an azido-protected O-acyl isopeptide as an aggregation-disrupting element.
22.	2010/08	A Mutation in the Saposin C Domain of the Sphingolipid Activator Protein (Prosaposin) Gene Causes Neurodegenerative Disease in Mice.
23.	2010/03	Developmental changes in glycolipids and synchronized expression of nutrient transporters in the mouse small intestine.
24.	2009/11	Regional expression of prosaposin in the wild-type and saposin D-deficient mouse brain detected by an anti-mouse prosaposin-specific antibody.
25.	2008/09	HPTLC-MS for rapid analysis of neutral glycosphingolipids.
26.	2007/11	The function of sphingolipids in the nervous system: Lessons learnt from mouse models of specific sphingolipid activator protein deficiencies.
27.	2006/06	GD3-and O-acetylated GD3-ganglopsides in the GM2 synthase-deficient mouse brain and their immunohistochemical localization.
28.	2006/04	Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking.
29.	2005/07	Hematopoietic cell transplantation ameliorates clinical phenotype and progression of the CNS pathology in the mouse model of late onset Krabbe disease.
30.	2005/01	Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease.
31.	2004/11	Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in the mouse.
32.	2004/10	Plasma adiponectine levels in newborns are higher than those in adults and positively correlated with birth weight.
33.	2004/08	Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the twitcher mouse model of globoid cell leukodystrophy.
34.	2004/07	Comparative Clinico-Pathological study of saposin-A deficient (SAP-A-/-) and twitcher mice.
35.	2003/12	Are animal models useful for understanding the pathophysiology of lysosomal storage disease? ,
36.	2003/11	Ghrelin concentration in cord and neonatal blood: relation to fetal growth and energy balance.
37.	2003/09	Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A- and prosaposin-deficient mice.
38.	2002/10	Thiamine-responsive pyruvate dehydrogenase deficiency in two patients caused by a point mutation (F205L and L216F) within the thiamine pyrophosphate binding region.
39.	2001/11	Dramatic phenotypic improvement during pregnancy in a genetic leukodystrophy: Estrogen appears to be a critical factor.
40.	2001/11	Expression of immune-related molecules is down-regulated in twitcher mice following bone marrow transplantation.
41.	2001/06	Psychosine is as potent an inducer of cell death as C6-ceramide in cultured fibroblasts and in MOCH cells.
42.	2001/05	A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse.
43.	2000/07	Infiltration of hematogenous lineage cells into the demyelinating central nervous system of twitcher mice.
44.	2000/07	Paradoxical influence of acid beta-galactosidase gene dosage on phenotype of the twitcher mouse (genetic galactosylceramidase deficiency).
45.	2000/01	Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide.
46.	2000/01	Twitcher mice with only a single active galactosylceramide synthase gene exhibit clearly detectable but therapeutically minor phenotypic improvements.
47.	1999/11	Developmental changes in long-form leptin receptor expression and localization in rat brain.
48.	1999/01	Dynamic changes in serum leptin concentrations during the fetal and neonatal periods.
49.	1998/01	Comparison of GAD and ICA512/IA-2 antibodies at and after the onset of IDDM.
50.	1998/01	Effect of growth hormone on the translocation of GLUT4 and its relation to insulin-like and anti-insulin action.
51.	1997/05	Serum leptin concentration in cord blood: relationship to birth weight and gender.
52.	1995/05	DNA diagnosis of pyruvate dehydrogenase deficiency in female patients with congenital lactic acidaemia.
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■ Presentation

1.	2023/11/03	A case of 17p13.3 microdeletion with breakpoint in the gene for CRK （Poster notice，General）
2.	2023/10/06	Selective Degeneration of Retinal Photoreceptor Cell in Prosaposin Transgenic Mice （Speech，General）
3.	2023/10/05	Quantitative Analysis of Sphingolipids in a mouse model of Krabbe Disease Using Mass Spectrometry （Speech，General）
4.	2023/07/06	Degeneration of Retinal Photoreceptor Cell in Prosaposin Transgenic Mice （Poster notice，General）
5.	2022/11/24	Establishment of cryopreserved neuronal cell bank -Analysis of Saposin D mutant neuron- （Poster notice，General）
6.	2022/11/24	Pathophysiological analysis of Saposin D-deficient Twitcher mice （Speech，General）
7.	2022/06/26	Acid ceramidase as a third N-acylethanolamine hydrolase in cells and tissues （Poster notice，General）
8.	2022/03/28	Hydrolysis of anti-inflammatory and anorexic N-acylethanolamines by acid ceramidase as revealed by using saposin D-deficient mice （Poster notice，General）
9.	2022/03/08	Hydrolytic activity of acid ceramidase on N-acylethanolamines, anti-inflammatory and anorexic lipid mediators: an analysis using saposin D-knockout mice （Poster notice，General）
10.	2021/11/03	Analysis of N-acylethanolamine-hydrolyzing activity using saposin D-deficient mice （Poster notice，General）
11.	2021/04/27	Role of acid ceramidase in the hydrolysis of anti-inflammatory and anorexic N-acylethanolamines （Poster notice，General）
12.	2020/09/16	Acid ceramidase hydrolyzes N-acylethanolamines, a group of anti-inflammatory and anorexic lipid mediators, in cells and tissues （Speech，General）
13.	2019/09/19	Oligomerization of prosaposin in the brain of saposin D mutant mice （Poster notice，General）
14.	2019/09/18	Cross-talk between O-GlcNAcylation and sialic acid metabolism （Speech，General）
15.	2019/09/18	Neuropathological analyses of saposin D mutant mice （Poster notice，General）
16.	2019/08/20	Neuropathological analysis of saposin D mutant mouse （Poster notice，General）
17.	2019/07/26	Improvement of abnormal differentiation and maturation in Krabbe disease mouse oligodendrocytes （Poster notice，General）
18.	2019/07/26	Pathophysiological analysis and therapeutic approach for inherited leukodystrophy with defective myelin lipid metabolism. （Speech，General）
19.	2018/09/06	Pathophysiological analysis of inherited leukodystrophy with defective myeline lipid metabolism and its therapeutic application to the demyelinating diseases. （Speech，General）
20.	2017/11/10	Frequency of Human Herpes Viruses 1-7, BV Virus and Human Parvovirus B19 in Patients with Febrile Neutropenia by Real-time PCR （Poster notice，General）
21.	2016/10/13	Numerous intertriginous xanthomas in infant: a diagnostic clue for sitosterolemia. （Speech，General）
22.	2015/08/26	Central nervous system pathology in the phytosphingolipid-deficient mouse. （Poster notice，General）
23.	2015/08/26	Role of prosaposin in retinal degeneration. （Poster notice，General）
24.	2015/07/04	Clinical anatomy dissection by student doctors （Speech，General）
25.	2014/10/18	Histopathological analysis of retinas of prosaposin knockout mice （Poster notice，General）
26.	2014/10/18	Overexpression of prosaposin causes severe retinal photoreceptor cell degeneration in mouse （Poster notice，General）
27.	2013/11/28	Overexpression of prosaposin causes severe retinal degeneration in mouse. （Poster notice，General）
28.	2013/09/11	2D-DIGE proteomic analysis in saposin D-deficient mice - Is the culprit carbonic anhydrase? （Poster notice，General）
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■ Winning science prize

2001/11

The National Organization for Rare Disorders, Inc. (NORD), Research Award.