PXD006933-1

PXD006933 is an original dataset announced via ProteomeXchange.

Title	THE PROTEOME SPECIATION OF AN IMMORTALIZED CYSTIC FIBROSIS CELL LINE: NEW PERSPECTIVES ON THE PATHOPHYSIOLOGY OF THE DISEASE.
Description	Cystic 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.
HostingRepository	PRIDE
AnnounceDate	2022-03-01
AnnouncementXML	Submission_2022-03-01_06:42:15.108.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Michele Puglia
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	monohydroxylated residue; iodoacetamide derivatized residue
Instrument	LCQ Classic

Revision	Datetime	Status	ChangeLog Entry
0	2017-07-11 04:25:59	ID requested
⏵ 1	2022-03-01 06:42:15	announced
2	2023-11-14 08:49:09	announced	2023-11-14: Updated project metadata.

10.1016/j.jprot.2017.09.013;

curator keyword: Biological, Biomedical

submitter keyword: Cystic Fibrosis, LC-MS/MS, laminopathies, CFTR, 2D-DIGE

Luca Bini
contact affiliation	Laboratory of Functional Proteomics, Department of Life Sciences, University of Siena. Siena, Italy.
contact email	luca.bini@unisi.it
lab head
Michele Puglia
contact affiliation	University of Southern Denmark
contact email	michelep@bmb.sdu.dk
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD006933
     1. Label: PRIDE project
     2. Name: THE PROTEOME SPECIATION OF AN IMMORTALIZED CYSTIC FIBROSIS CELL LINE: NEW PERSPECTIVES ON THE PATHOPHYSIOLOGY OF THE DISEASE.