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Cited by 23Lysosomal Storage Disorders ResearchGlycogen Storage Diseases and MyoclonusGlycosylation and Glycoproteins Research

Structure of the murine lysosomal multienzyme complex core

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Gorelik, Alexei, Illes, Katalin, Hasan, S. M. Naimul, Nagar, Bhushan, Mazhab-Jafari, Mohammad T.·2021·Science Advances
DOI 10.1126/sciadv.abf4155PMC PMC8115914
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Abstract

The 3D structure of the complex formed by the enzymes GLB1 and CTSA furthers our understanding of lysosomal storage diseases.

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  • The CTSA homodimer remains intact upon association with NEU1, as is the case in the complex of CTSA with GLB1.

    “These results demonstrate that the CTSA homodimer remains intact upon association with NEU1, as is the case in the complex of CTSA with another lysosomal glycosidase, GLB1 (79).”

    From Structure of the immunoregulatory sialidase NEU1 (2023) · cites this paper as [79]

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Authors
Gorelik, A., Illes, K., Hasan, S. M. N., Nagar, B., & Mazhab-Jafari, M. T.
Affiliations
  • University Health Network (CA)
  • McGill University (CA)
  • Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada.
Corresponding author
Nagar, Bhushan
ORCID
Venue
Science Advances
Publisher
American Association for the Advancement of Science (AAAS)
Published
2021-05-14
Volume
7
Issue
20
ISSN
2375-2548 (ISSN-L)
DOI
10.1126/sciadv.abf4155
PMC
PMC8115914
OpenAlex
W3163623036
Cited by
23
References
0
Citations by year
2026: 7 · 2025: 3 · 2024: 5 · 2023: 3 · 2022: 3 · 2021: 2
FWCI
1.99
Type
journal article
Language
en
Open access
Licence
Locations
  • Science Advances (publishedVersion, cc-by-nc)
  • PubMed Central (publishedVersion, cc-by-nc)
  • PubMed (publishedVersion)
Funders
  • Princess Margaret Cancer Foundation
  • Ontario Research Foundation
  • Canadian Institutes of Health Research (MFE-164773, MOP-133535, MOP-419240)
  • Canada Foundation for Innovation
  • Fonds de Recherche du Québec - Santé (277129)
  • Natural Sciences and Engineering Research Council of Canada (RGPIN-2018-06070)
Topics
  • Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
  • Health Sciences › Medicine › Rheumatology › Glycogen Storage Diseases and Myoclonus
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Glycosylation and Glycoproteins Research
Keywords
beta-galactosidase, GLB1, NEU1, Clinical and Translational Science Awards, GM1 gangliosidosis, sialidosis, galactosialidosis, lysosomal storage disease, protein complex structure, polar interfaces, glycoprotein degradation, lysosomes

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