Elosulfase alfa for mucopolysaccharidosis type IVA: Real-world experience in 7 patients from the Spanish Morquio-A early access program
Sign Up to like & getrecommendations! Published in 2018 at "Molecular Genetics and Metabolism Reports"
DOI: 10.1016/j.ymgmr.2018.03.009
Abstract: There is a growing interest in evaluating the effectiveness of enzyme replacement therapy (ERT) with elosulfase alfa in patients with mucopolysaccharidosis type IVA (MPS-IVA) under real-world conditions. We present the experience of seven pediatric MPS-IVA… read more here.
Keywords: iva real; mucopolysaccharidosis type; real world; type iva ... See more keywords
PilY1 and minor pilins form a complex priming the type IVa pilus in Myxococcus xanthus
Sign Up to like & getrecommendations! Published in 2020 at "Nature Communications"
DOI: 10.1038/s41467-020-18803-z
Abstract: Type IVa pili are ubiquitous and versatile bacterial cell surface filaments that undergo cycles of extension, adhesion and retraction powered by the cell-envelope spanning type IVa pilus machine (T4aPM). The overall architecture of the T4aPM… read more here.
Keywords: minor pilins; type iva; myxococcus xanthus; iva pilus ... See more keywords
A noncanonical cytochrome c stimulates calcium binding by PilY1 for type IVa pili formation
Sign Up to like & getrecommendations! Published in 2022 at "Proceedings of the National Academy of Sciences of the United States of America"
DOI: 10.1073/pnas.2115061119
Abstract: Significance Type IVa pili (T4aP) are bacterial surface structures that function under different environmental conditions. In the machine for T4aP formation, a complex of minor pilins and PilY1 primes T4aP formation and is also present… read more here.
Keywords: type iva; calcium binding; calcium; iva pili ... See more keywords
Mucopolysaccharidosis type IVA (Morquio A): a close differential diagnosis of spondylo-epiphyseal dysplasia
Sign Up to like & getrecommendations! Published in 2017 at "BMJ Case Reports"
DOI: 10.1136/bcr-2017-221156
Abstract: Patients with mucopolysaccharidoses (MPS) have a plethora of multisystemic manifestations depending on the particular type, and atypical presentations are not uncommon. MPS type IVA (Morquio A syndrome) has predominant musculoskeletal system involvement and corneal clouding… read more here.
Keywords: epiphyseal dysplasia; diagnosis; type iva; iva morquio ... See more keywords
PilT and PilU are homohexameric ATPases that coordinate to retract type IVa pili
Sign Up to like & getrecommendations! Published in 2019 at "PLoS Genetics"
DOI: 10.1371/journal.pgen.1008448
Abstract: Bacterial type IV pili are critical for diverse biological processes including horizontal gene transfer, surface sensing, biofilm formation, adherence, motility, and virulence. These dynamic appendages extend and retract from the cell surface. In many type… read more here.
Keywords: retraction; type iva; pilu; pilt ... See more keywords