Cargando…

Murine Fibroblasts and Primary Hepatocytes as Tools When Studying the Efficacy of Potential Therapies for Mucopolysaccharidosis Type I

Mucopolysaccharidosis type I (MPS I) is a metabolic genetic disease caused by the deficiency of a lysosomal enzyme involved in glycosaminoglycans (GAGs) degradation. MPS I cells have a constant level of GAG synthesis, but disturbed degradation means that GAGs accumulate progressively, impairing cell...

Descripción completa

Detalles Bibliográficos
Autores principales:	Węsierska, Magdalena, Nowicka, Wioletta, Kloska, Anna, Jakóbkiewicz-Banecka, Joanna, Malinowska, Marcelina
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2022
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9820816/ https://www.ncbi.nlm.nih.gov/pubmed/36613977 http://dx.doi.org/10.3390/ijms24010534

Ejemplares similares

Cellular and Gene Expression Response to the Combination of Genistein and Kaempferol in the Treatment of Mucopolysaccharidosis Type I
por: Węsierska, Magdalena, et al.
Publicado: (2022)

Lipophagy and Lipolysis Status in Lipid Storage and Lipid Metabolism Diseases
por: Kloska, Anna, et al.
Publicado: (2020)

Lipids and Lipid Mediators Associated with the Risk and Pathology of Ischemic Stroke
por: Kloska, Anna, et al.
Publicado: (2020)

The Role of Dimethyl Sulfoxide (DMSO) in Gene Expression Modulation and Glycosaminoglycan Metabolism in Lysosomal Storage Disorders on an Example of Mucopolysaccharidosis
por: Moskot, Marta, et al.
Publicado: (2019)

Global Changes of 5-mC/5h-mC Ratio and Methylation of Adiponectin and Leptin Gene in Placenta Depending on Mode of Delivery
por: Słabuszewska-Jóźwiak, Aneta, et al.
Publicado: (2021)

Corrigendum: Modulation of expression of genes involved in glycosaminoglycan metabolism and lysosome biogenesis by flavonoids
por: Moskot, Marta, et al.
Publicado: (2016)

Modulation of expression of genes involved in glycosaminoglycan metabolism and lysosome biogenesis by flavonoids
por: Moskot, Marta, et al.
Publicado: (2015)

Corrigendum: Modulation of expression of genes involved in glycosaminoglycan metabolism and lysosome biogenesis by flavonoids
por: Moskot, Marta, et al.
Publicado: (2016)

Effects of flavonoids on glycosaminoglycan synthesis: implications for substrate reduction therapy in Sanfilippo disease and other mucopolysaccharidoses
por: Kloska, Anna, et al.
Publicado: (2011)

Atypical microbial infections of digestive tract may contribute to diarrhea in mucopolysaccharidosis patients: a MPS I case study
por: Węgrzyn, Grzegorz, et al.
Publicado: (2005)

Synthetic genistein derivatives as modulators of glycosaminoglycan storage
por: Kloska, Anna, et al.
Publicado: (2012)

Lysosome Alterations in the Human Epithelial Cell Line HaCaT and Skin Specimens: Relevance to Psoriasis
por: Bocheńska, Katarzyna, et al.
Publicado: (2019)

Correction: Bocheńska, K. et al. Lysosome Alterations in the Human Epithelial Cell Line HaCaT and Skin Specimens: Relevance to Psoriasis. Int. J. Mol. Sci. 2019, 20, 2255
por: Bocheńska, Katarzyna, et al.
Publicado: (2020)

Activities of genes controlling sphingolipid metabolism in human fibroblasts treated with flavonoids
por: Moskot, Marta, et al.
Publicado: (2015)

Dosage Compensation in Females with X-Linked Metabolic Disorders
por: Juchniewicz, Patrycja, et al.
Publicado: (2021)

Effects of flavonoids on expression of genes involved in cell cycle regulation and DNA replication in human fibroblasts
por: Moskot, Marta, et al.
Publicado: (2015)

X-chromosome inactivation patterns depend on age and tissue but not conception method in humans
por: Juchniewicz, Patrycja, et al.
Publicado: (2023)

Factors and processes modulating phenotypes in neuronopathic lysosomal storage diseases
por: Jakóbkiewicz-Banecka, Joanna, et al.
Publicado: (2013)

Genistein-mediated inhibition of glycosaminoglycan synthesis, which corrects storage in cells of patients suffering from mucopolysaccharidoses, acts by influencing an epidermal growth factor-dependent pathway
por: Jakóbkiewicz-Banecka, Joanna, et al.
Publicado: (2009)

Personalized prediction of the secondary oocytes number after ovarian stimulation: A machine learning model based on clinical and genetic data
por: Zieliński, Krystian, et al.
Publicado: (2023)

Models in the Research Process of Psoriasis
por: Bocheńska, Katarzyna, et al.
Publicado: (2017)

Abnormal Sphingolipid World in Inflammation Specific for Lysosomal Storage Diseases and Skin Disorders
por: Moskot, Marta, et al.
Publicado: (2018)

Effect of Silicone on the Collagen Fibrillogenesis and Stability
por: Kadziński, Leszek, et al.
Publicado: (2015)

The model homologue of the partially defective human 5,10-methylenetetrahydrofolate reductase, considered as a risk factor for stroke due to increased homocysteine level, can be protected and reactivated by heat shock proteins
por: Grabowski, Michał, et al.
Publicado: (2016)

Sexual behaviour in a murine model of mucopolysaccharidosis type I (MPS I)
por: Barbosa Mendes, Ana, et al.
Publicado: (2019)

Prevalence of polymorphisms in OPG, RANKL and RANK as potential markers for Charcot arthropathy development
por: Bruhn-Olszewska, Bożena, et al.
Publicado: (2017)

Mucopolysaccharidosis Type I
por: Kubaski, Francyne, et al.
Publicado: (2020)

Nonsteroidal anti-inflammatory drugs modulate cellular glycosaminoglycan synthesis by affecting EGFR and PI3K signaling pathways
por: Mozolewski, Paweł, et al.
Publicado: (2017)

Impact of isoflavone genistein on psoriasis in in vivo and in vitro investigations
por: Bocheńska, Katarzyna, et al.
Publicado: (2021)

Inhibition of iduronic acid biosynthesis by ebselen reduces glycosaminoglycan accumulation in mucopolysaccharidosis type I fibroblasts
por: Maccarana, Marco, et al.
Publicado: (2021)

Effects of Enzyme Replacement Therapy Started Late in a Murine Model of Mucopolysaccharidosis Type I
por: Pasqualim, Gabriela, et al.
Publicado: (2015)

Cathepsin B-associated Activation of Amyloidogenic Pathway in Murine Mucopolysaccharidosis Type I Brain Cortex
por: Viana, Gustavo Monteiro, et al.
Publicado: (2020)

Enhanced Efficiency of the Basal and Induced Apoptosis Process in Mucopolysaccharidosis IVA and IVB Human Fibroblasts
por: Brokowska, Joanna, et al.
Publicado: (2023)

Expression of Long Noncoding RNAs in Fibroblasts from Mucopolysaccharidosis Patients
por: Cyske, Zuzanna, et al.
Publicado: (2023)

Distribution of Heparan Sulfate Oligosaccharides in Murine Mucopolysaccharidosis Type IIIA
por: Mason, Kerryn, et al.
Publicado: (2014)

Neurocognitive testing in a murine model of mucopolysaccharidosis type IIIA
por: Pericleous, Kleopatra, et al.
Publicado: (2023)

A Humoral Immune Response Alters the Distribution of Enzyme Replacement Therapy in Murine Mucopolysaccharidosis Type I
por: Le, Steven Q., et al.
Publicado: (2017)

Molecular action of isoflavone genistein in the human epithelial cell line HaCaT
por: Smolińska, Elwira, et al.
Publicado: (2018)

Aortic Root Dilatation in Mucopolysaccharidosis I–VII
por: Bolourchi, Meena, et al.
Publicado: (2016)

Open issues in Mucopolysaccharidosis type I-Hurler
por: Parini, Rossella, et al.
Publicado: (2017)

Cannot write session to /tmp/vufind_sessions/sess_vq7ui94fv71n484qmemok0u5hh