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Cited by 117Lysosomal Storage Disorders ResearchGlycogen Storage Diseases and MyoclonusCellular transport and secretion

Lysosomal multienzyme complex: pros and cons of working together

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Bonten, Erik J., Annunziata, Ida, d’Azzo, Alessandra·2014·Cellular and Molecular Life Sciences
DOI 10.1007/s00018-013-1538-3PMC PMC4037752
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Abstract

The ubiquitous distribution of lysosomes and their heterogeneous protein composition reflects the versatility of these organelles in maintaining cell homeostasis and their importance in tissue differentiation and remodeling. In lysosomes, the degradation of complex, macromolecular substrates requires the synergistic action of multiple hydrolases that usually work in a stepwise fashion. This catalytic machinery explains the existence of lysosomal enzyme complexes that can be dynamically assembled and disassembled to efficiently and quickly adapt to the pool of substrates to be processed or degraded, adding extra tiers to the regulation of the individual protein components. An example of such a complex is the one composed of three hydrolases that are ubiquitously but differentially expressed: the serine carboxypeptidase, protective protein/cathepsin A (PPCA), the sialidase, neuraminidase-1 (NEU1), and the glycosidase β-galactosidase (β-GAL). Next to this 'core' complex, the existence of sub-complexes, which may contain additional components, and function at the cell surface or extracellularly, suggests as yet unexplored functions of these enzymes. Here we review how studies of basic biological processes in the mouse models of three lysosomal storage disorders, galactosialidosis, sialidosis, and GM1-gangliosidosis, revealed new and unexpected roles for the three respective affected enzymes, Ppca, Neu1, and β-Gal, that go beyond their canonical degradative activities. These findings have broadened our perspective on their functions and may pave the way for the development of new therapies for these lysosomal storage disorders.

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  • In the absence of functional PPCA, NEU1 is retained in the endosomal compartment while the pool of β-GAL reaching lysosomes is rapidly degraded; formation of the PPCA/NEU1/β-GAL complex has a reciprocal advantage for the three enzymes, which acquire active and stable conformation in lysosomes.

    “In absence of functional PPCA, NEU1 is retained in the endosomal compartment while the pool of β-GAL reaching the lysosomes is rapidly degraded 36.”

    From Galactosialidosis: historic aspects and overview of investigated and emerging treatment options (2017) · cites this paper as [36]

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Authors
Bonten, E. J., Annunziata, I., & d’Azzo, A.
Affiliations
St. Jude Children's Research Hospital (US)
Corresponding author
d’Azzo, Alessandra
ORCID
Venue
Cellular and Molecular Life Sciences
Publisher
Springer Science and Business Media LLC
Published
2013-12-15
Volume
71
Issue
11
Pages
2017-2032
ISSN
  • 1420-682X (ISSN-L)
  • 1420-9071
DOI
10.1007/s00018-013-1538-3
PMC
PMC4037752
OpenAlex
W2067188727
Cited by
117
References
1
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0.90
Type
journal article
Language
en
Open access
Licence
www.springer.com/tdm (tdm)
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  • Cellular and Molecular Life Sciences (publishedVersion)
  • Europe PMC (PubMed Central) (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • National Institutes of Health (1r13gm060905-01)
  • National Cancer Institute (P30CA021765)
  • National Institute of General Medical Sciences (R01GM104981, GM60905, R01GM060950)
  • National Institute of Diabetes and Digestive and Kidney Diseases (DK-52025, Z01DK052025, R01DK052025)
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 › Cell Biology › Cellular transport and secretion
Keywords
lysosomal enzymes, cathepsin A, beta-galactosidase, lysosomal storage disease, galactosialidosis, sialidosis, GM1 gangliosidosis, lysosomal protein degradation
MeSH terms
Animals, beta-Galactosidase, Disease Models, Animal, Gene Expression Regulation, Humans, Lysosomes, Mucolipidoses, Multienzyme Complexes, Neuraminidase, Signal Transduction, Lysosomal Storage Diseases, Gangliosidosis, GM1, Mice, Knockout, Cathepsin A, Mice

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

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