PXD009896

PXD009896 is an original dataset announced via ProteomeXchange.

Title	ITGA3 neg. human keratinocytes
Description	Integrin alpha3beta1, a major epidermal adhesion receptor is critical for organization of the basement membrane during development and wound healing. Integrin alpha3 deficiency leads to interstitial lung disease, nephrotic syndrome and epidermolysis bullosa (ILNEB), an autosomal recessive multiorgan disease characterized by basement membrane abnormalities in skin, lung and kidney. The pathogenetic chains from ITGA3 mutation to tissue abnormalities are still unclear. Although integrin 3 was reported to regulate multiple extracellular proteins, the composition of the extracellular compartment of integrin alpha3-negative keratinocytes has not been resolved so far. In a comprehensive approach, quantitative proteomics of deposited extracellular matrix, conditioned cultured media as well as of the intracellular compartment of keratinocytes isolated from an ILNEB patient and from normal skin were performed. By mass spectrometry-based proteomics, 167 proteins corresponding to the GO terms “extracellular” and “cell adhesion”, or included in the “human matrisome” were identified in the deposited extracellular matrix, and 217 in the conditioned media of normal human keratinocytes. In the absence of integrin alpha3, 33% and 26% respectively were dysregulated. Dysregulated proteins were functionally related to integrin alpha3 or were known interaction partners. The results show that in the absence of integrin alpha3 ILNEB keratinocytes produce a fibronectin-rich microenvironment and make use of fibronectin-binding integrin subunits alphav and alpha5.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_04:12:20.056.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Joern Dengjel
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	LTQ Orbitrap

Revision	Datetime	Status	ChangeLog Entry
0	2018-05-25 01:43:00	ID requested
1	2018-11-27 00:09:16	announced
⏵ 2	2024-10-22 04:12:20	announced	2024-10-22: Updated project metadata.

10.1016/j.matbio.2018.07.001;

He Y, Thriene K, Boerries M, Hausser I, Franzke CW, Busch H, Dengjel J, Has C, 3 impacts the composition of the cellular microenvironment of ILNEB keratinocytes. Matrix Biol, 74():62-76(2018) [pubmed]

curator keyword: Biomedical

submitter keyword: keratinocyte, skin,human, LC-MS/MS

Joern Dengjel
contact affiliation	Department of Biology, University of Fribourg Chemin du Musée 10, 1700 Fribourg, Switzerland T +41 26 300 8631 F +41 26 300 9741 W http://www.unifr.ch/biology/research/dengjel/
contact email	joern.dengjel@unifr.ch
lab head
Joern Dengjel
contact affiliation	University of Fribourg
contact email	joern.dengjel@medizin.uni-freiburg.de
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/2018/11/PXD009896

PRIDE project URI

• PRIDE
  1. PXD009896
     1. Label: PRIDE project
     2. Name: ITGA3 neg. human keratinocytes