Present specialized field
（Last updated : 2020-03-27 19:20:17）
Present specialized field
Pediatrics, Neurochemistry/Neuropharmacology, Structural biochemistry
Sphingolipid activator proteins, Experimental Glycoscience: glycobiology
Krabbe disease (Globoid cell leukodystrophy)., Lysosomal Storage Disorders.
Novel neuroblastoma amplified sequence (NBAS) mutations in a Japanese boy with fever-triggered recurrent acute liver failure.
Developmental defects and aberrant accumulation of endogenous psychosine in oligodendrocytes in a murine model of Krabbe disease.
A case of sitosterolemia due to compound heterozygous mutations in ABCG5: clinical features and treatment outcomes obtained with colestimide and ezetimibe
Numerous intertriginous xanthomas in infant: A diagnostic clue for sitosterolemia
The effects of chemically synthesized saposin C on glucosylceramide-beta-glucosidase
Mesotrypsin and caspase-14 participate in prosaposin processing: potential relevance to epidermal permeability barrier formation.
Improved high-fat diet-induced glucose intolerance by an oral administration of phytosphingosine.
Chemoenzymatic synthesis of hydrophobic glycoprotein: synthesis of saposin C carrying complex-type carbohydrate.
Primary polydipsia, but not accumulated ceramide, causes lethal renal damage in saposin D-deficient mice.
Endosomal sorting by Semaphorin 4A in retinal pigment epithelium supports photoreceptor survival.
High-performance thin-layer chromatography/mass spectrometry for the analysis of neutral glycosphingolipids.
Synthesis of the sphingolipid activator protein, saposin C, using an azido-protected O-acyl isopeptide as an aggregation-disrupting element.
A Mutation in the Saposin C Domain of the Sphingolipid Activator Protein (Prosaposin) Gene Causes Neurodegenerative Disease in Mice.
Developmental changes in glycolipids and synchronized expression of nutrient transporters in the mouse small intestine.
Regional expression of prosaposin in the wild-type and saposin D-deficient mouse brain detected by an anti-mouse prosaposin-specific antibody.
HPTLC-MS for rapid analysis of neutral glycosphingolipids.
The function of sphingolipids in the nervous system: Lessons learnt from mouse models of specific sphingolipid activator protein deficiencies.
GD3-and O-acetylated GD3-ganglopsides in the GM2 synthase-deficient mouse brain and their immunohistochemical localization.
Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking.
Hematopoietic cell transplantation ameliorates clinical phenotype and progression of the CNS pathology in the mouse model of late onset Krabbe disease.
Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease.
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.
Plasma adiponectine levels in newborns are higher than those in adults and positively correlated with birth weight.
Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the twitcher mouse model of globoid cell leukodystrophy.
Comparative Clinico-Pathological study of saposin-A deficient (SAP-A-/-) and twitcher mice.
Are animal models useful for understanding the pathophysiology of lysosomal storage disease? ,
Ghrelin concentration in cord and neonatal blood: relation to fetal growth and energy balance.
Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A- and prosaposin-deficient mice.
Thiamine-responsive pyruvate dehydrogenase deficiency in two patients caused by a point mutation (F205L and L216F) within the thiamine pyrophosphate binding region.
Dramatic phenotypic improvement during pregnancy in a genetic leukodystrophy: Estrogen appears to be a critical factor.
Expression of immune-related molecules is down-regulated in twitcher mice following bone marrow transplantation.
Psychosine is as potent an inducer of cell death as C6-ceramide in cultured fibroblasts and in MOCH cells.
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.
Infiltration of hematogenous lineage cells into the demyelinating central nervous system of twitcher mice.
Paradoxical influence of acid beta-galactosidase gene dosage on phenotype of the twitcher mouse (genetic galactosylceramidase deficiency).
Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide.
Twitcher mice with only a single active galactosylceramide synthase gene exhibit clearly detectable but therapeutically minor phenotypic improvements.
Developmental changes in long-form leptin receptor expression and localization in rat brain.
Dynamic changes in serum leptin concentrations during the fetal and neonatal periods.
Comparison of GAD and ICA512/IA-2 antibodies at and after the onset of IDDM.
Effect of growth hormone on the translocation of GLUT4 and its relation to insulin-like and anti-insulin action.
Serum leptin concentration in cord blood: relationship to birth weight and gender.
DNA diagnosis of pyruvate dehydrogenase deficiency in female patients with congenital lactic acidaemia.
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Oligomerization of prosaposin in the brain of saposin D mutant mice
Cross-talk between O-GlcNAcylation and sialic acid metabolism
Neuropathological analyses of saposin D mutant mice
Neuropathological analysis of saposin D mutant mouse
Improvement of abnormal differentiation and maturation in Krabbe disease mouse oligodendrocytes
Pathophysiological analysis and therapeutic approach for inherited leukodystrophy with defective myelin lipid metabolism.
Pathophysiological analysis of inherited leukodystrophy with defective myeline lipid metabolism and its therapeutic application to the demyelinating diseases.
Numerous intertriginous xanthomas in infant: a diagnostic clue for sitosterolemia.
Central nervous system pathology in the phytosphingolipid-deficient mouse.
Role of prosaposin in retinal degeneration.
Clinical anatomy dissection by student doctors
Histopathological analysis of retinas of prosaposin knockout mice
Overexpression of prosaposin causes severe retinal photoreceptor cell degeneration in mouse
Overexpression of prosaposin causes severe retinal degeneration in mouse.
2D-DIGE proteomic analysis in saposin D-deficient mice - Is the culprit carbonic anhydrase?
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