A study of intrathecal enzyme replacement for cognitive decline in mucopolysaccharidosis I (original) (raw)

Safety Evaluation of Chronic Intrathecal Administration of Idursulfase-IT in Cynomolgus Monkeys

michael heartlein

Toxicologic Pathology, 2011

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Intrathecal idursulfase-IT in patients with neuronopathic mucopolysaccharidosis II: Results from a phase 2/3 randomized study

Matilde ruiz garcia

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Journal Pre-proof Long-term open-label extension study of the safety and efficacy of intrathecal idursulfase-IT in patients with neuronopathic mucopolysaccharidosis II

Matilde ruiz garcia

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A phase I/II study of intrathecal idursulfase-IT in children with severe mucopolysaccharidosis II

Christian Hendriksz

Genetics in medicine : official journal of the American College of Medical Genetics, 2015

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CNS Penetration of Intrathecal-Lumbar Idursulfase in the Monkey, Dog and Mouse: Implications for Neurological Outcomes of Lysosomal Storage Disorder

michael heartlein

PLoS ONE, 2012

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Biodistribution and pharmacodynamics of recombinant human alpha-l-iduronidase (rhIDU) in mucopolysaccharidosis type I-affected cats following multiple intrathecal administrations

Raquel Walton

Molecular Genetics and Metabolism, 2011

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Effect of systemic high dose enzyme replacement therapy on the improvement of CNS defects in a mouse model of mucopolysaccharidosis type II

Ah-ra Ko

Orphanet journal of rare diseases, 2015

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Uptake of recombinant iduronate-2-sulfatase into neuronal and glial cells in vitro

Aurora Daniele

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2002

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Intrathecal enzyme replacement therapy: Successful treatment of brain disease via the cerebrospinal fluid

Steven Le

Molecular Genetics and Metabolism, 2007

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Effect of neonatal administration of a retroviral vector expressing α-l-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice

Katherine Ponder

Molecular Genetics and Metabolism, 2007

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Effect of high dose, repeated intra-cerebrospinal fluid injection of sulphamidase on neuropathology in mucopolysaccharidosis type IIIA mice

Helen Beard

Genes, Brain and Behavior, 2008

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Intrathecal enzyme replacement therapy reverses cognitive decline in mucopolysaccharidosis type I

Alia Ahmed

American journal of medical genetics. Part A, 2017

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Replacing the Enzyme -L-Iduronidase at Birth Ameliorates Symptoms in the Brain and Periphery of Dogs with Mucopolysaccharidosis Type I

A. Fales-Williams

Science Translational Medicine, 2010

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Preclinical dose ranging studies for enzyme replacement therapy with idursulfase in a knock-out mouse model of MPS II

Antony Emmanuel Camacho Garcia

Molecular Genetics and Metabolism, 2007

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Profile of idursulfase for the treatment of Hunter syndrome

Ferdinando Ceravolo

Research and Reports in Endocrine Disorders, 2015

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Intravenous delivery of a chemically modified sulfamidase efficiently reduces heparan sulfate storage and brain pathology in mucopolysaccharidosis IIIA mice

Agneta Tjernberg

Molecular Genetics and Metabolism Reports, 2019

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Successful desensitization to idursulfase in a patient with type II mucopolysaccharidosis (Hunter syndrome)

Carlos Serrano

Journal of investigational allergology & clinical immunology, 2011

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High-dose enzyme replacement therapy attenuates cerebroventriculomegaly in a mouse model of mucopolysaccharidosis type II

Chihwa Kim

Journal of Human Genetics, 2013

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Intracerebroventricular dosing of N-sulfoglucosamine sulfohydrolase in mucopolysaccharidosis IIIA mice reduces markers of brain lysosomal dysfunction

Terri Christianson

Journal of Biological Chemistry, 2022

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Penetration, diffusion, and uptake of recombinant human α-l-iduronidase after intraventricular injection into the rat brain

Steven Jungles

Molecular Genetics and Metabolism, 2005

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α-L-iduronidase therapy for mucopolysaccharidosis type I

Paul Orchard

Biologics: targets & therapy, 2008

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Intrathecal administration of AAV vectors for the treatment of lysosomal storage in the brains of MPS I mice

Gordon Watson

Gene Therapy, 2006

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A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome)

Roberto Giugliani

Genetics in Medicine, 2006

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The Effect of Recombinant Human Iduronate-2-Sulfatase (Idursulfase) on Growth in Young Patients with Mucopolysaccharidosis Type II

Anna Tylki-szymanska

PLoS ONE, 2014

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Glycosaminoglycan storage in neuroanatomical regions of mucopolysaccharidosis I dogs following intrathecal recombinant human iduronidase

Catalina Guerra

APMIS, 2011

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Comparative study of idursulfase beta and idursulfase in vitro and in vivo

Chihwa Kim

Journal of Human Genetics, 2016

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Correction of metabolic, craniofacial, and neurologic abnormalities in MPS I mice treated at birth with adeno-associated virus vector transducing the human alpha-L-iduronidase gene

Roland Gunther

Molecular therapy : the journal of the American Society of Gene Therapy, 2004

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Correction of Metabolic, Craniofacial, and Neurologic Abnormalities in MPS I Mice Treated at Birth with Adeno-associated Virus Vector Transducing the Human α-L-Iduronidase Gene

Walter Low

Molecular Therapy, 2004

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Whole body correction of mucopolysaccharidosis IIIA by intracerebrospinal fluid gene therapy

Sara Marcó, Sandra Motas, Luca Maggioni

Journal of Clinical Investigation, 2013

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Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled, multinational study of recombinant human α-L-iduronidase (laronidase)

Kenneth Berger

The Journal of Pediatrics, 2004

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An Improved Adeno-Associated Virus Vector for Neurological Correction of the Mouse Model of Mucopolysaccharidosis IIIA

Jessica Taylor

2019

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RTB lectin-mediated delivery of lysosomal α-l-iduronidase mitigates disease manifestations systemically including the central nervous system

Chester Whitley

Molecular genetics and metabolism, 2018

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