A review of bioactive plant polysaccharides: Biological activities, functionalization, and biomedical applications

J Liu, S Willför, C Xu - Bioactive Carbohydrates and Dietary Fibre, 2015 - Elsevier
Natural polysaccharides from different sources have long been studied and widely used in
different areas, such as food and feed, medicine and pharmaceutics, and in papermaking. In …

Pathogenesis of mucopolysaccharidoses, an update

S Fecarotta, A Tarallo, C Damiano, N Minopoli… - International Journal of …, 2020 - mdpi.com
The recent advancements in the knowledge of lysosomal biology and function have
translated into an improved understanding of the pathophysiology of …

[PDF][PDF] Mucopolysaccharidosis VI: pathophysiology, diagnosis and treatment

P Harmatz, R Shediac - Front Biosci (Landmark Ed), 2017 - article.imrpress.com
1. ABSTRACT Mucopolysaccharidosis VI (MPS VI), or Maroteaux-Lamy syndrome, is an
autosomal recessive lysosomal storage disorder caused by deficient activity of the enzyme …

Recommendations for the management of MPS IVA: systematic evidence-and consensus-based guidance

MU Akyol, TD Alden, H Amartino, J Ashworth… - Orphanet journal of rare …, 2019 - Springer
Abstract Introduction Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an
autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N …

Lysosomal storage diseases: current therapies and future alternatives

AF Leal, AJ Espejo-Mojica, OF Sánchez… - Journal of Molecular …, 2020 - Springer
Lysosomal storage disorders (LSDs) are a group of monogenic diseases characterized by
progressive accumulation of undegraded substrates into the lysosome, due to mutations in …

Development of idursulfase therapy for mucopolysaccharidosis type II (Hunter syndrome): the past, the present and the future

DAH Whiteman, A Kimura - Drug design, development and therapy, 2017 - Taylor & Francis
Mucopolysaccharidosis type II (MPS II; Hunter syndrome; OMIM 309900) is a rare,
multisystemic, progressive lysosomal storage disease caused by deficient activity of the …

Novel therapies for mucopolysaccharidosis type III

B Seker Yilmaz, J Davison, SA Jones… - Journal of inherited …, 2021 - Wiley Online Library
Abstract Mucopolysaccharidosis type III (MPS III) or Sanfilippo disease is an orphan
inherited lysosomal storage disease and one of the most common MPS subtypes. The …

Innate immunity in mucopolysaccharide diseases

O Mandolfo, H Parker, B Bigger - International Journal of Molecular …, 2022 - mdpi.com
Mucopolysaccharidoses are rare paediatric lysosomal storage disorders, characterised by
accumulation of glycosaminoglycans within lysosomes. This is caused by deficiencies in …

Therapies for the bone in mucopolysaccharidoses

S Tomatsu, CJ Alméciga-Díaz, AM Montaño… - Molecular genetics and …, 2015 - Elsevier
Patients with mucopolysaccharidoses (MPS) have accumulation of glycosaminoglycans in
multiple tissues which may cause coarse facial features, mental retardation, recurrent ear …

Natural history and galsulfase treatment in mucopolysaccharidosis VI (MPS VI, Maroteaux–Lamy syndrome)—10‐year follow‐up of patients who previously …

R Giugliani, C Lampe, N Guffon… - American journal of …, 2014 - Wiley Online Library
Mucopolysaccharidosis VI (MPS VI) is a clinically heterogeneous and progressive disorder
with multiorgan manifestations caused by deficient N‐acetylgalactosamine‐4‐sulfatase …