Search Results - "Kida, Sachiho"
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Evaluation of cerebrospinal fluid heparan sulfate as a biomarker of neuropathology in a murine model of mucopolysaccharidosis type II using high-sensitivity LC/MS/MS
Published in Molecular genetics and metabolism (01-09-2018)“…Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a lysosomal storage disorder caused by a deficiency of iduronate-2-sulfatase (IDS), an enzyme that…”
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Clearance of heparan sulfate in the brain prevents neurodegeneration and neurocognitive impairment in MPS II mice
Published in Molecular therapy (05-05-2021)“…Mucopolysaccharidosis II (MPS II), a lysosomal storage disease caused by mutations in iduronate-2-sulfatase (IDS), is characterized by a wide variety of…”
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Autophagy in the Central Nervous System and Effects of Chloroquine in Mucopolysaccharidosis Type II Mice
Published in International journal of molecular sciences (20-11-2019)“…Mucopolysaccharidosis type II (MPS II) is a rare lysosomal storage disease (LSD) involving a genetic error in iduronic acid-2-sulfatase (IDS) metabolism that…”
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Enzyme replacement with transferrin receptor-targeted α-L-iduronidase rescues brain pathology in mucopolysaccharidosis I mice
Published in Molecular therapy. Methods & clinical development (08-06-2023)“…Mucopolysaccharidosis I (MPS I), a lysosomal storage disease caused by dysfunction of α-L-iduronidase (IDUA), is characterized by the deposition of dermatan…”
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Ectopic expression of N-acetylglucosaminyltransferase V accelerates hepatic triglyceride synthesis
Published in Hepatology research (01-03-2016)“…Aim Glycosylation changes induce various types of biological phenomena in human diseases. N‐Acetylglucosaminyltransferase V (GnT‐V) is one of the most…”
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Fetuin-A negatively correlates with liver and vascular fibrosis in nonalcoholic fatty liver disease subjects
Published in Liver international (01-03-2015)“…Background & Aims Fetuin‐A (α2HS‐glycoprotein), a liver secretory glycoprotein, is known as a transforming growth factor (TGF)‐β1 signalling inhibitor. Serum…”
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Non-clinical evaluation of a blood-brain barrier-penetrating enzyme for the treatment of mucopolysaccharidosis type I
Published in Molecular genetics and metabolism (01-02-2019)“…Treatment of central nervous system (CNS) disorders in patients with lysosomal storage diseases by intravenous enzyme replacement therapy is still challenging…”
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Serum Mac-2 binding protein levels as a novel diagnostic biomarker for prediction of disease severity and nonalcoholic steatohepatitis
Published in Proteomics. Clinical applications (01-10-2013)“…Purpose Mac‐2 binding protein (Mac‐2bp) is one of the major fucosylated glycoproteins, which we identified with glycol‐proteomic analyses. We previously…”
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Hepatic aberrant glycosylation by N-acetylglucosaminyltransferase V accelerates HDL assembly
Published in American journal of physiology: Gastrointestinal and liver physiology (01-11-2016)“…Glycosylation is involved in various pathophysiological conditions. N-Acetylglucosaminyltransferase V (GnT-V), catalyzing β1-6 branching in asparagine-linked…”
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N-Acetylglucosaminyltransferase V exacerbates concanavalin A-induced hepatitis in mice
Published in Molecular medicine reports (01-05-2015)“…N-Acetylglucosaminyltransferase V (GnT-V) catalyzes β1-6 branching in asparagine-linked oligosaccharides and is one of the most important glycosyltransferases…”
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