Alessandra d'Azzo

Overview

Best Publications

• Lysosomal storage diseases

  Frances M Platt;Alessandra d'Azzo;Beverly L Davidson;Elizabeth F Neufeld

  1133
  Citations

• Molecular defect in combined beta-galactosidase and neuraminidase deficiency in man

  A. D'Azzo;A. Hoogeveen;A. J. J. Reuser;D. Robinson

  492
  Citations

• Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.

  E Bonten;A van der Spoel;M Fornerod;G Grosveld

  334
  Citations

• E3 ubiquitin ligases as regulators of membrane protein trafficking and degradation.

  Alessandra d'Azzo;Antonella Bongiovanni;Tommaso Nastasi

  318
  Citations

• GM1-ganglioside-mediated activation of the unfolded protein response causes neuronal death in a neurodegenerative gangliosidosis.

  Alessandra Tessitore;Maria del P. Martin;Renata Sano;Yanjun Ma

  315
  Citations

• GM1-Ganglioside Accumulation at the Mitochondria-Associated ER Membranes Links ER Stress to Ca2+-Dependent Mitochondrial Apoptosis

  Renata Sano;Ida Annunziata;Annette Patterson;Simon Moshiach

  311
  Citations

• Expression of cDNA encoding the human “protective protein≓ associated with lysosomal β-galactosidase and neuraminidase: Homology to yeast proteases

  Niels J. Galjart;Nynke Gillemans;Alan Harris;Gijsbertus T.J. van der Horst

  289
  Citations

• Cathepsin A regulates chaperone-mediated autophagy through cleavage of the lysosomal receptor

  Ana Maria Cuervo;Linda Mann;Erik J. Bonten;Alessandra d'Azzo

  265
  Citations

• Sialidases in Vertebrates : A Family Of Enzymes Tailored For Several Cell Functions*

  Eugenio Monti;Erik Bonten;Alessandra D'Azzo;Roberto Bresciani

  226
  Citations

• Human lysosomal protective protein has cathepsin A-like activity distinct from its protective function.

  N J Galjart;H Morreau;R Willemsen;N Gillemans

  225
  Citations

• Association of alpha- and beta-subunits during the biosynthesis of beta-hexosaminidase in cultured human fibroblasts.

  R L Proia;A d'Azzo;E F Neufeld

  219
  Citations

• Neuraminidase 1 is a negative regulator of lysosomal exocytosis.

  Gouri Yogalingam;Erik J. Bonten;Diantha van de Vlekkert;Huimin Hu

  207
  Citations

• Alternative splicing of beta-galactosidase mRNA generates the classic lysosomal enzyme and a beta-galactosidase-related protein.

  H. Morreau;N. J. Galjart;N. Gillemans;R. Willemsen

  183
  Citations

• Generalized CNS Disease and Massive GM1-Ganglioside Accumulation in Mice Defective in Lysosomal Acid β-galactosidase

  Christopher N. Hahn;Maria del Pilar Martin;Maria Schröder;Marie T. Vanier

  179
  Citations

• GM1 gangliosidosis and Morquio B disease: an update on genetic alterations and clinical findings

  Anna Caciotti;Scott C. Garman;Yadilette Rivera-Colón;Elena Procopio

  173
  Citations

• Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A

  Aarnoud van der Spoel;Erik Bonten;Alessandra d'Azzo

  172
  Citations

• Novel mutations in lysosomal neuraminidase identify functional domains and determine clinical severity in sialidosis

  Erik. J. Bonten;Willem F. Arts;Michael Beck;A. Covanis

  162
  Citations

• Lysosomal NEU1 deficiency affects amyloid precursor protein levels and amyloid-β secretion via deregulated lysosomal exocytosis.

  Ida Annunziata;Annette Patterson;Danielle Helton;Danielle Helton;Huimin Hu

  159
  Citations

• Broad and direct interaction between TLR and Siglec families of pattern recognition receptors and its regulation by Neu1.

  Guo Yun Chen;Nicholas K. Brown;Wei Wu;Zahra Khedri

  157
  Citations

• Expression Specificity of GFAP Transgenes

  Mu Su;Huimin Hu;Youngjin Lee;Alessandra d'Azzo

  153
  Citations