Tento výsledek nelze pro nepřihlášené uživatele zobrazit.
K zobrazení výsledku je třeba se přihlásit.

THERMODYNAMICS OF INTERACTION BETWEEN RECOMBINANT HUMAN LYSOSOMAL alfa-GLUCOSIDASE AND PHARMACOLOGICAL CHAPERONES

Autor: DEL VECCHIO, POMPEA GIUSEPPINA GRAZIA, PARENTI, GIANCARLO, FERRARA, MARIA CARMINA, I. FOTTICCHIA, B. CORBUCCI PONZANO, MORACCI, Marco

Background : Glycogen storage disease type II or Pompe disease is a rare inherited metabolic. Pompe disease is caused by mutations in the gene that encodes the lysosomal hydrolase acid a-glucosidase (GAA). GAA deficiency results in lysosomal glycogen

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=od______3730::5623a2913f56a4a31dea49643e5a5a26
http://hdl.handle.net/11588/558739

Carbohydrate-Active enZYmes: functional and applicative aspects of glycoside hydrolases

Autor: Ferrara, Maria Carmina

Carbohydrates play an important role in a variety of biological and industrial processes. The elucidation of the structure/function relationship of glycoside hydrolases (GHs) can help us to understand their implication in various biological phenomena

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1b87e1bcd4b30e875eedebfdf4a7f5ad
http://www.fedoa.unina.it/9208/

Extremophilic (Hemi)cellulolytic Microorganisms and Enzymes.

Autor: Cobucci-Ponzano, Beatrice, Ionata, Elena, Cara, Francesco La, Morana, Alessandra, Ferrara, Maria Carmina, Maurelli, Luisa, Strazzulli, Andrea, Giglio, Rosa, Moracci, Marco

Publikováno v: Lignocellulose Conversion; 2013, p111-130, 20p

Structure of human lysosomal acid α-glucosidase–a guide for the treatment of Pompe disease

Autor: Véronique Roig-Zamboni, Beatrice Cobucci-Ponzano, Roberta Iacono, Maria Carmina Ferrara, Stanley Germany, Yves Bourne, Giancarlo Parenti, Marco Moracci, Gerlind Sulzenbacher

Publikováno v: Nature Communications
Nature Communications, Nature Publishing Group, 2017, 8, pp.1111. ⟨10.1038/s41467-017-01263-3⟩
'Nature Communications ', vol: 8, pages: 1111-1-1111-10 (2017)
Nature Communications, Nature Publishing Group, 2017, 8, pp.1111. 〈10.1038/s41467-017-01263-3〉
Nature Communications, Vol 8, Iss 1, Pp 1-10 (2017)
Nature Communications, Nature Publishing Group, 2017, ⟨10.1038/s41467-017-01263-3⟩
Nature communications 8 (2017). doi:10.1038/s41467-017-01263-3
info:cnr-pdr/source/autori:Roig-Zamboni V.; Cobucci-Ponzano B.; Iacono R.; Ferrara M.C.; Germany S.; Bourne Y.; Parenti G.; Moracci M.; Sulzenbacher G./titolo:Structure of human lysosomal acid alpha-glucosidase-A guide for the treatment of Pompe disease/doi:10.1038%2Fs41467-017-01263-3/rivista:Nature communications/anno:2017/pagina_da:/pagina_a:/intervallo_pagine:/volume:8
Nature Communications, 2017, ⟨10.1038/s41467-017-01263-3⟩
Nature Communications, 2017, 8, pp.1111. ⟨10.1038/s41467-017-01263-3⟩

Pompe disease, a rare lysosomal storage disease caused by deficiency of the lysosomal acid α-glucosidase (GAA), is characterized by glycogen accumulation, triggering severe secondary cellular damage and resulting in progressive motor handicap and pr

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5c82ffd7ffc81e6e645698e18bb52b05
https://hal.archives-ouvertes.fr/hal-01802847/file/s41467-017-01263-3.pdf