Oxidative Stress in Mucopolysaccharidoses: Pharmacological Implications

Gene Expression-Related Changes in Morphologies of Organelles and Cellular Component Organization in Mucopolysaccharidoses

(2021) Lidia Gaffke et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Update in the Mucopolysaccharidoses

(2021) Kim L. McBride et al.   Seminars in Pediatric Neurology

Molecular Mechanism of Neuroprotective Effect of Melatonin on Morphine Addiction and Analgesic Tolerance: an Update

(2021) Ling-Yan Su et al.   MOLECULAR NEUROBIOLOGY

Expression of genes involved in apoptosis is dysregulated in mucopolysaccharidoses as revealed by pilot transcriptomic analyses

(2020) Joanna Brokowska et al.   CELL BIOLOGY INTERNATIONAL

Genetic Base of Behavioral Disorders in Mucopolysaccharidoses: Transcriptomic Studies

(2020) Karolina Pierzynowska et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Underestimated Aspect of Mucopolysaccharidosis Pathogenesis: Global Changes in Cellular Processes Revealed by Transcriptomic Studies

(2020) Lidia Gaffke et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Pathogenesis of Mucopolysaccharidoses, an Update

(2020) Simona Fecarotta et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Transcriptomic Changes Related to Cellular Processes with Particular Emphasis on Cell Activation in Lysosomal Storage Diseases from the Group of Mucopolysaccharidoses

(2020) Estera Rintz et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Melatonin attenuates hypertension and oxidative stress in a rat model of L-NAME-induced gestational hypertension

(2020) Junfang Zuo et al.   VASCULAR MEDICINE

Changes in expressions of genes involved in the regulation of cellular processes in mucopolysaccharidoses as assessed by fibroblast culture-based transcriptomic analyses

(2020) Lidia Gaffke et al.   METABOLIC BRAIN DISEASE

Proteasome Composition and Activity Changes in Cultured Fibroblasts Derived From Mucopolysaccharidoses Patients and Their Modulation by Genistein

(2020) Karolina Pierzynowska et al.   Frontiers in Cell and Developmental Biology

Has resveratrol a potential for mucopolysaccharidosis treatment?

(2020) Estera Rintz et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

The Role of Dimethyl Sulfoxide (DMSO) in Gene Expression Modulation and Glycosaminoglycan Metabolism in Lysosomal Storage Disorders on an Example of Mucopolysaccharidosis

(2019) Marta Moskot et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Plasma coenzyme Q10 status is impaired in selected genetic conditions

(2019) Raquel Montero et al.   Scientific Reports

Oxidative stress assessment by glutathione peroxidase activity and glutathione levels in response to selenium supplementation in patients with Mucopolysaccharidosis I, II and VI

(2019) José Araújo de Oliveira-Silva et al.   GENETICS AND MOLECULAR BIOLOGY

Changes in cellular processes occurring in mucopolysaccharidoses as underestimated pathomechanisms of these diseases

(2019) Lidia Gaffke et al.   CELL BIOLOGY INTERNATIONAL

50 Years Ago in T J P

(2019) Adriana M. Montaño   JOURNAL OF PEDIATRICS

Oxidative stress, aging, and diseases

(2018) Ilaria Liguori et al.   Clinical Interventions in Aging

Hunter syndrome: Long-term idursulfase treatment does not protect patients against DNA oxidation and cytogenetic damage

(2018) Carlos Eduardo Diaz Jacques et al.   MUTATION RESEARCH-GENETIC TOXICOLOGY AND ENVIRONMENTAL MUTAGENESIS

Neuromuscular degeneration and locomotor deficit in a Drosophila model of mucopolysaccharidosis VII is attenuated by treatment with resveratrol

(2018) Sudipta Bar et al.   Disease Models & Mechanisms

Linking mitochondrial dysfunction to neurodegeneration in lysosomal storage diseases

(2017) Afshin Saffari et al.   JOURNAL OF INHERITED METABOLIC DISEASE

How close are we to therapies for Sanfilippo disease?

(2017) Lidia Gaffke et al.   METABOLIC BRAIN DISEASE

Clinical outcomes in a subpopulation of adults with Morquio A syndrome: results from a long-term extension study of elosulfase alfa

(2017) D. Hughes et al.   Orphanet Journal of Rare Diseases

Oxidative and nitrative stress and pro-inflammatory cytokines in Mucopolysaccharidosis type II patients: effect of long-term enzyme replacement therapy and relation with glycosaminoglycan accumulation

(2016) Carlos Eduardo Diaz Jacques et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Biomolecules damage and redox status abnormalities in Fabry patients before and during enzyme replacement therapy

(2016) Giovana Brondani Biancini et al.   CLINICA CHIMICA ACTA

Cell cycle is disturbed in mucopolysaccharidosis type II fibroblasts, and can be improved by genistein

(2016) Marta Moskot et al.   GENE

A Preclinical Study Evaluating AAVrh10-Based Gene Therapy for Sanfilippo Syndrome

(2016) Leanne K. Winner et al.   HUMAN GENE THERAPY

Therapeutic Potential of Hydroxypropyl-β-Cyclodextrin-Based Extract of Medicago sativa in the Treatment of Mucopolysaccharidoses

(2016) Barbara Fumić et al.   PLANTA MEDICA

Glial degeneration with oxidative damage drives neuronal demise in MPSII disease

(2016) Cristina Zalfa et al.   Cell Death & Disease

Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model

(2015) Carla Martins et al.   BRAIN

Genistein Improves 3-NPA-Induced Memory Impairment in Ovariectomized Rats: Impact of Its Antioxidant, Anti-Inflammatory and Acetylcholinesterase Modulatory Properties

(2015) Esther T. Menze et al.   PLoS One

Glycosaminoglycans can be associated with oxidative damage in mucopolysaccharidosis II patients submitted to enzyme replacement therapy

(2014) G. W. Negretto et al.   CELL BIOLOGY AND TOXICOLOGY

Intracerebral Administration of Adeno-Associated Viral Vector Serotype rh.10 Carrying Human SGSH and SUMF1 cDNAs in Children with Mucopolysaccharidosis Type IIIA Disease: Results of a Phase I/II Trial

(2014) Marc Tardieu et al.   HUMAN GENE THERAPY

The Phytoestrogen Genistein Modulates Lysosomal Metabolism and Transcription Factor EB (TFEB) Activation

(2014) Marta Moskot et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Oxidative stress is independent of inflammation in the neurodegenerative sanfilippo syndrome type B

(2014) Stéphanie Trudel et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Assessment of Sleep in Children with Mucopolysaccharidosis Type III

(2014) Louise Victoria Mahon et al.   PLoS One

Liver-directed gene therapy corrects cardiovascular lesions in feline mucopolysaccharidosis type I

(2014) C. Hinderer et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Treatment effect of coenzyme Q10 and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease

(2013) Leslie Matalonga et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Unfolded protein response is not activated in the mucopolysaccharidoses but protein disulfide isomerase 5 is deregulated

(2011) Guglielmo R. D. Villani et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Neuroinflammatory and oxidative stress phenomena in MPS IIIA mouse model: The positive effect of long-term aspirin treatment

(2011) Audrey Arfi et al.   MOLECULAR GENETICS AND METABOLISM

Oxidative stress in patients with mucopolysaccharidosis type II before and during enzyme replacement therapy

(2011) Letícia Filippon et al.   MOLECULAR GENETICS AND METABOLISM

DNA damage in leukocytes from pretreatment mucopolysaccharidosis type II patients; protective effect of enzyme replacement therapy

(2011) Letícia Filippon et al.   MUTATION RESEARCH-GENETIC TOXICOLOGY AND ENVIRONMENTAL MUTAGENESIS

Long-term Amelioration of Feline Mucopolysaccharidosis VI After AAV-mediated Liver Gene Transfer

(2010) Gabriella Cotugno et al.   MOLECULAR THERAPY

Genistein ameliorates learning and memory deficits in amyloid β(1–40) rat model of Alzheimer’s disease

(2010) Maryam Bagheri et al.   NEUROBIOLOGY OF LEARNING AND MEMORY

Genistein Improves Neuropathology and Corrects Behaviour in a Mouse Model of Neurodegenerative Metabolic Disease

(2010) Marcelina Malinowska et al.   PLoS One

Intracranial gene delivery of LV-NAGLU vector corrects neuropathology in murine MPS IIIB

(2009) Carmela Di Domenico et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Mucopolysaccharidosis IIIB: Oxidative damage and cytotoxic cell involvement in the neuronal pathogenesis

(2009) Guglielmo R.D. Villani et al.   BRAIN RESEARCH

Alterations in Oxidative Markers in the Cerebellum and Peripheral Organs in MPS I Mice

(2009) Gustavo Kellermann Reolon et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Oxidative Stress and Neurodegenerative Diseases: A Review of Upstream and Downstream Antioxidant Therapeutic Options

(2009) Bayani Uttara et al.   Current Neuropharmacology

Genistein reduces lysosomal storage in peripheral tissues of mucopolysaccharide IIIB mice

(2009) Marcelina Malinowska et al.   MOLECULAR GENETICS AND METABOLISM

Mutational and oxidative stress analysis in patients with mucopolysaccharidosis type I undergoing enzyme replacement therapy

(2007) Vanessa Gonçalves Pereira et al.   CLINICA CHIMICA ACTA