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PXD006933-1

PXD006933 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleTHE PROTEOME SPECIATION OF AN IMMORTALIZED CYSTIC FIBROSIS CELL LINE: NEW PERSPECTIVES ON THE PATHOPHYSIOLOGY OF THE DISEASE.
DescriptionCystic Fibrosis (CF) is a recessively inherited disease caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR has a pivotal role in the onset of CF, and several proteins are directly or indirectly involved in its homeostasis. To study these CFTR interactors at protein species level, we used a functional proteomics approach combining 2D-DIGE, mass spectrometry and enrichment analysis. A human cystic fibrosis bronchial epithelial cell line (CFBE41o-) was used for analysis. 74 differentially expressed spots were identified and some were validated by western-blot. Enrichment analysis highlighted molecular pathways in which ezrin, HSP70, endoplasmin and lamin A/C, in addition to CFTR, were considered central hubs in CFTR homeostasis. These proteins acquire different functions through post-translational modifications, emphasizing the importance of studying the CF proteome at protein species level. Moreover, serpin H1, prelamin A/C, protein-SET and cystatin-B were associated for the first time to CF, demonstrating the importance of heat shock response, cross-talk between the cytoskeleton and signal transduction, chronic inflammation and alteration of CFTR gating in the pathophysiology of the disease. These results open new perspectives for the understanding of the proteostasis network, characteristic of CF pathology, and could provide a springboard for new therapeutic strategies.
HostingRepositoryPRIDE
AnnounceDate2022-03-01
AnnouncementXMLSubmission_2022-03-01_06:42:15.108.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterMichele Puglia
SpeciesList scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationListmonohydroxylated residue; iodoacetamide derivatized residue
InstrumentLCQ Classic
Dataset History
RevisionDatetimeStatusChangeLog Entry
02017-07-11 04:25:59ID requested
12022-03-01 06:42:15announced
22023-11-14 08:49:09announced2023-11-14: Updated project metadata.
Publication List
10.1016/j.jprot.2017.09.013;
Keyword List
curator keyword: Biological, Biomedical
submitter keyword: Cystic Fibrosis, LC-MS/MS, laminopathies, CFTR, 2D-DIGE
Contact List
Luca Bini
contact affiliationLaboratory of Functional Proteomics, Department of Life Sciences, University of Siena. Siena, Italy.
contact emailluca.bini@unisi.it
lab head
Michele Puglia
contact affiliationUniversity of Southern Denmark
contact emailmichelep@bmb.sdu.dk
dataset submitter
Full Dataset Link List
Dataset FTP location
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PRIDE project URI
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