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10.3390/biom10030355
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Article

Proteomic Analysis of Mucopolysaccharidosis IIIB Mouse Brain

by
Valeria De Pasquale
1,†,
Michele Costanzo
1,2,†,
Rosa Anna Siciliano
3,
Maria Fiorella Mazzeo
3,
Valeria Pistorio
1,
Laura Bianchi
4,
Emanuela Marchese
2,5,
Margherita Ruoppolo
1,2,
Luigi Michele Pavone
1,* and
Marianna Caterino
1,2
1
Department of Molecular Medicine and Medical Biotechnology, School of Medicine, University of Naples Federico II, 80131 Naples, Italy
2
CEINGE-Biotecnologie Avanzate scarl, 80145 Naples, Italy
3
Institute of Food Sciences, CNR, 83100 Avellino, Italy
4
Laboratory of Functional Proteomics, Department of Life Sciences, University of Siena, 53100 Siena, Italy
5
Department of Mental Health and Preventive Medicine, University of Campania Luigi Vanvitelli, 80138 Naples, Italy
*
Author to whom correspondence should be addressed.
†
These authors equally contributed to this work.
Biomolecules 2020, 10(3), 355; https://doi.org/10.3390/biom10030355
Submission received: 24 November 2019 / Revised: 17 February 2020 / Accepted: 24 February 2020 / Published: 26 February 2020
(This article belongs to the Special Issue Lysosomal Storage Disorders: Molecular Basis and Therapeutic Approaches)
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Abstract

Mucopolysaccharidosis IIIB (MPS IIIB) is an inherited metabolic disease due to deficiency of α-N-Acetylglucosaminidase (NAGLU) enzyme with subsequent storage of undegraded heparan sulfate (HS). The main clinical manifestations of the disease are profound intellectual disability and neurodegeneration. A label-free quantitative proteomic approach was applied to compare the proteome profile of brains from MPS IIIB and control mice to identify altered neuropathological pathways of MPS IIIB. Proteins were identified through a bottom up analysis and 130 were significantly under-represented and 74 over-represented in MPS IIIB mouse brains compared to wild type (WT). Multiple bioinformatic analyses allowed to identify three major clusters of the differentially abundant proteins: proteins involved in cytoskeletal regulation, synaptic vesicle trafficking, and energy metabolism. The proteome profile of NAGLU−/− mouse brain could pave the way for further studies aimed at identifying novel therapeutic targets for the MPS IIIB. Data are available via ProteomeXchange with the identifier PXD017363.
Keywords: mucopolysaccharidosis IIIB; quantitative proteomics; NAGLU; lysosomes mucopolysaccharidosis IIIB; quantitative proteomics; NAGLU; lysosomes

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MDPI and ACS Style

De Pasquale, V.; Costanzo, M.; Siciliano, R.A.; Mazzeo, M.F.; Pistorio, V.; Bianchi, L.; Marchese, E.; Ruoppolo, M.; Pavone, L.M.; Caterino, M. Proteomic Analysis of Mucopolysaccharidosis IIIB Mouse Brain. Biomolecules 2020, 10, 355. https://doi.org/10.3390/biom10030355

AMA Style

De Pasquale V, Costanzo M, Siciliano RA, Mazzeo MF, Pistorio V, Bianchi L, Marchese E, Ruoppolo M, Pavone LM, Caterino M. Proteomic Analysis of Mucopolysaccharidosis IIIB Mouse Brain. Biomolecules. 2020; 10(3):355. https://doi.org/10.3390/biom10030355

Chicago/Turabian Style

De Pasquale, Valeria, Michele Costanzo, Rosa Anna Siciliano, Maria Fiorella Mazzeo, Valeria Pistorio, Laura Bianchi, Emanuela Marchese, Margherita Ruoppolo, Luigi Michele Pavone, and Marianna Caterino. 2020. "Proteomic Analysis of Mucopolysaccharidosis IIIB Mouse Brain" Biomolecules 10, no. 3: 355. https://doi.org/10.3390/biom10030355

APA Style

De Pasquale, V., Costanzo, M., Siciliano, R. A., Mazzeo, M. F., Pistorio, V., Bianchi, L., Marchese, E., Ruoppolo, M., Pavone, L. M., & Caterino, M. (2020). Proteomic Analysis of Mucopolysaccharidosis IIIB Mouse Brain. Biomolecules, 10(3), 355. https://doi.org/10.3390/biom10030355

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