PXD017363

PXD017363 is an original dataset announced via ProteomeXchange.

Title	Proteomic analysis of mucopolysaccharidosis IIIB mouse brain
Description	Mucopolysaccharidosis IIIB (MPS IIIB) is an inherited metabolic disease due to deficiency of α-N-Acetylglucosaminidase (NAGLU) enzyme with subsequent storage of undegradedheparan sulfate (HS). The main clinical manifestations of the disease are profound intellectual disability and neurodegeneration. To identify potential biomarkers and novel neuropathological mechanisms of MPS IIIB, a label-free quantitative proteomic approach was applied to compare the proteome profile of brains from MPS IIIB and control mice. 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 of the differentially abundant proteins allowed to define three major clusters: proteins involved in cytoskeletal regulation, synaptic vesicle trafficking, and energy metabolism. The results highlight the involvement of these clustered proteins in the neuropathology of MPS IIIB disease. The proteins identified in this study would provide potential targets for diagnostic and therapeutic studies of MPS IIIB.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_04:59:26.172.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Michele Costanzo
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2020-02-03 08:00:45	ID requested
1	2020-02-26 17:03:40	announced
2	2020-04-30 03:48:54	announced	2020-04-30: Updated publication reference for PubMed record(s): 32111039.
⏵ 3	2024-10-22 04:59:30	announced	2024-10-22: Updated project metadata.

10.3390/biom10030355;

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, 10(3):(2020) [pubmed]

submitter keyword: Mucopolysaccharidosis IIIB

Quantitative proteomics

NAGLU

Lysosomes

Michele Costanzo
contact affiliation	Department of Molecular Medicine and Medical Biotechnology - University of Naples "Federico II"
contact email	michele.costanzo@unina.it
lab head
Michele Costanzo
contact affiliation	Department of Molecular Medicine and Medical Biotechnology - University of Naples Federico II
contact email	michele.costanzo@unina.it
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2020/02/PXD017363

PRIDE project URI

• PRIDE
  1. PXD017363
     1. Label: PRIDE project
     2. Name: Proteomic analysis of mucopolysaccharidosis IIIB mouse brain