Zobrazeno 1 - 7
of 7
pro vyhledávání: '"Melania Scarcella"'
Autor:
Melania Scarcella, Gianluca Scerra, Mariangela Ciampa, Marianna Caterino, Michele Costanzo, Laura Rinaldi, Antonio Feliciello, Serenella Anzilotti, Chiara Fiorentino, Maurizio Renna, Margherita Ruoppolo, Luigi Michele Pavone, Massimo D’Agostino, Valeria De Pasquale
Publikováno v:
iScience, Vol 27, Iss 3, Pp 108959- (2024)
Summary: Mucopolysaccharidoses (MPSs) are lysosomal disorders with neurological involvement for which no cure exists. Here, we show that recombinant NK1 fragment of hepatocyte growth factor rescues substrate accumulation and lysosomal defects in MPS
Externí odkaz:
https://doaj.org/article/b7dab594af994d1eb4614b3cad83d1e6
Autor:
Gianluca Scerra, Valeria De Pasquale, Melania Scarcella, Maria Gabriella Caporaso, Luigi Michele Pavone, Massimo D'Agostino
Publikováno v:
Open Biology, Vol 12, Iss 10 (2022)
Lysosomal storage diseases (LSDs) comprise a group of inherited monogenic disorders characterized by lysosomal dysfunctions due to undegraded substrate accumulation. They are caused by a deficiency in specific lysosomal hydrolases involved in cellula
Externí odkaz:
https://doaj.org/article/9d4a979dd1304966aef37c94b08be1a3
Publikováno v:
Biomedicines, Vol 10, Iss 4, p 922 (2022)
Lysosomal storage diseases (LSDs) are a group of metabolic diseases caused by inborn mutations of lysosomal enzymes, which lead to lysosome substrate accumulation in various cell types [...]
Externí odkaz:
https://doaj.org/article/f65c73ee75dc4d78a469fa47bc509410
Autor:
Valeria De Pasquale, Anna Esposito, Gianluca Scerra, Melania Scarcella, Mariangela Ciampa, Antonietta Luongo, Daniele D’Alonzo, Annalisa Guaragna, Massimo D’Agostino, Luigi Michele Pavone
Sanfilippo syndrome comprises a group of four genetic diseases due to the lack or decreased activity of enzymes involved in heparan sulfate (HS) catabolism. HS accumulation in lysosomes and other cellular compartments results in tissue and organ dysf
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::16844532e3504532b734a61312cce9ca
https://hdl.handle.net/11588/913438
https://hdl.handle.net/11588/913438
Autor:
null Gianluca Scerra, null Valeria De Pasquale, null Melania Scarcella, null Maria Gabriella Caporaso, null Luigi Michele Pavone, null Massimo D'Agostino
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2b269153addd0b39790b65ff36566d1f
https://doi.org/10.1098/rsob.220155/v3/response1
https://doi.org/10.1098/rsob.220155/v3/response1
Autor:
Melania Scarcella, Danila d’Angelo, Mariangela Ciampa, Simona Tafuri, Luigi Avallone, Luigi Michele Pavone, Valeria De Pasquale
Publikováno v:
International journal of molecular sciences. 23(16)
Cathepsins encompass a family of lysosomal proteases that mediate protein degradation and turnover. Although mainly localized in the endolysosomal compartment, cathepsins are also found in the cytoplasm, nucleus, and extracellular space, where they a
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bf9fd65cafba218805accc85da9689a0
https://pubmed.ncbi.nlm.nih.gov/36012353
https://pubmed.ncbi.nlm.nih.gov/36012353
Autor:
Melania Scarcella, Luigi Michele Pavone, Gianluca Scerra, Massimo D'Agostino, Valeria De Pasquale
Publikováno v:
Biochimica et Biophysica Acta (BBA) - Molecular Cell Research. 1868:119113
Mucopolysaccharidoses (MPSs) are a group of inherited lysosomal storage disorders associated with the deficiency of lysosomal enzymes involved in glycosaminoglycan (GAG) degradation. The resulting cellular accumulation of GAGs is responsible for wide
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c18174c6e989b831bf07a0656b442d17
https://doi.org/10.1016/j.bbamcr.2021.119113
https://doi.org/10.1016/j.bbamcr.2021.119113