Proteasome composition and activity changes in cultured fibroblasts derived from mucopolysaccharidoses patients and their modulation by genistein
K Pierzynowska, L Gaffke, E Jankowska… - Frontiers in Cell and …, 2020 - frontiersin.org
In this study, we have asked whether proteasome composition and function are affected in
cells derived from patients suffering from all types of mucopolysaccharidosis (MPS), an …
cells derived from patients suffering from all types of mucopolysaccharidosis (MPS), an …
Genistein-mediated inhibition of glycosaminoglycan synthesis, which corrects storage in cells of patients suffering from mucopolysaccharidoses, acts by influencing an …
J Jakóbkiewicz-Banecka, E Piotrowska… - Journal of Biomedical …, 2009 - Springer
Background Mucopolysaccharidoses (MPS) are inherited metabolic disorders caused by
mutations leading to dysfunction of one of enzymes involved in degradation of …
mutations leading to dysfunction of one of enzymes involved in degradation of …
Decreased levels of chaperones in mucopolysaccharidoses and their elevation as a putative auxiliary therapeutic approach
M Żabińska, L Gaffke, P Bielańska, M Podlacha… - Pharmaceutics, 2023 - mdpi.com
Mucopolysaccharidoses (MPS) are rare genetic disorders belonging to the lysosomal
storage diseases. They are caused by mutations in genes encoding lysosomal enzymes …
storage diseases. They are caused by mutations in genes encoding lysosomal enzymes …
Underestimated aspect of mucopolysaccharidosis pathogenesis: global changes in cellular processes revealed by transcriptomic studies
L Gaffke, K Pierzynowska, M Podlacha… - International Journal of …, 2020 - mdpi.com
Mucopolysaccharidoses (MPS), a group of inherited metabolic disorders caused by
deficiency in enzymes involved in degradation of glycosaminoglycans (GAGs), are …
deficiency in enzymes involved in degradation of glycosaminoglycans (GAGs), are …
Changes in cellular processes occurring in mucopolysaccharidoses as underestimated pathomechanisms of these diseases
L Gaffke, K Pierzynowska, M Podlacha… - Cell Biology …, 2021 - Wiley Online Library
Mucopolysaccharidoses (MPS) are a group of genetic disorders belonging to lysosomal
storage diseases. They are caused by genetic defects leading to a lack or severe deficiency …
storage diseases. They are caused by genetic defects leading to a lack or severe deficiency …
Mucopolysaccharidoses: cellular consequences of glycosaminoglycans accumulation and potential targets
Mucopolysaccharidoses (MPSs) constitute a heterogeneous group of lysosomal storage
disorders characterized by the lysosomal accumulation of glycosaminoglycans (GAGs) …
disorders characterized by the lysosomal accumulation of glycosaminoglycans (GAGs) …
Cell cycle is disturbed in mucopolysaccharidosis type II fibroblasts, and can be improved by genistein
M Moskot, M Gabig-Cimińska, J Jakóbkiewicz-Banecka… - Gene, 2016 - Elsevier
Mucopolysaccharidoses (MPSs) are inherited metabolic diseases caused by mutations
resulting in deficiency of one of enzymes involved in degradation of glycosaminoglycans …
resulting in deficiency of one of enzymes involved in degradation of glycosaminoglycans …
Genistein-mediated inhibition of glycosaminoglycan synthesis as a basis for gene expression-targeted isoflavone therapy for mucopolysaccharidoses
E Piotrowska, J Jakobkiewicz-Banecka… - European Journal of …, 2006 - nature.com
Mucopolysaccharidoses (MPS) are inherited, severe, progressive, metabolic disorders
caused by deficiencies in different enzymes involved in degradation of glycosaminoglycans …
caused by deficiencies in different enzymes involved in degradation of glycosaminoglycans …
Transcriptomic changes related to cellular processes with particular emphasis on cell activation in lysosomal storage diseases from the group of …
Although mucopolysaccharidoses (MPS), inherited metabolic diseases from the group of
lysosomal storage diseases (LSD), are monogenic disorders, recent studies indicated that …
lysosomal storage diseases (LSD), are monogenic disorders, recent studies indicated that …
[HTML][HTML] Contribution of vesicle trafficking dysregulation to the pathomechanism of mucopolysaccharidosis
L Gaffke, K Pierzynowska, Z Cyske, M Podlacha… - Biochemical and …, 2023 - Elsevier
Although mucopolysaccharidoses (MPS) are monogenic diseases, caused by mutations in
genes coding for enzymes involved in degradation of glycosaminoglycans (GAGs), recent …
genes coding for enzymes involved in degradation of glycosaminoglycans (GAGs), recent …