PXD011833

PXD011833 is an original dataset announced via ProteomeXchange.

Title	The Amino-Terminal Part of Human Filaggrin-2 is a Component of Cornified Envelopes
Description	Filaggrin-2 (FLG2), a member of the S100-fused type protein family, is a 250 kDa protein mainly expressed in differentiated keratinocytes of the epidermis (Wu et al., 2009; Hsu et al., 2011). Its expression is reduced in atopic dermatitis (Pellerin et al., 2013), and heterozygous non-sense mutations of its gene are associated with a more persistent form of the disease in Afro-American populations (Margolis et al., 2014). Homozygous mutations of its gene are responsible for a rare skin disease called peeling skin syndrome type A (Bolling et al., 2018; Mohamad et al., 2018). However, the exact role of FLG2, a multi-domain protein, is not well known. Its COOH-tail exhibits anti-microbial activity (Hansmann et al., 2015), whereas its B domain is suspected to be involved in the hydration of the upper part of the epidermis, the cornified layer. Here, on the basis of immunological, global proteomics and in vitro enzymatic analysis we demonstrated that its amino-terminal domain is cross-linked to the cornified envelope, a pericellular resistant structure that replaces the plasma membrane during the last step of keratinocyte differentiation. In addition, depletion of FLG2 in reconstructed 3D human epidermis induced a marked fragility of cornified envelopes. These data suggest that FLG2 is important for proper mechanical strength of the cornified layer. They may explain the epidermal fragility observed in peeling skin syndrome type A patients.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_04:50:49.176.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Carine Froment
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	monohydroxylated residue; iodoacetamide derivatized residue; deamidated residue
Instrument	LTQ Orbitrap Velos

Revision	Datetime	Status	ChangeLog Entry
0	2018-11-26 08:16:00	ID requested
1	2019-02-25 08:58:29	announced
⏵ 2	2024-10-22 04:50:55	announced	2024-10-22: Updated project metadata.

10.1016/j.jid.2018.11.022;

Alb, é, rola G, Schr, ö, der JM, Froment C, Simon M, The Amino-Terminal Part of Human FLG2 Is a Component of Cornified Envelopes. J Invest Dermatol, 139(6):1395-1397(2019) [pubmed]

curator keyword: Biological

submitter keyword: keratinocyte, proteomics,Corneocyte, transglutaminase, terminal differentiation, epidermis

Michel Simon
contact affiliation	UDEAR,INSERM U1056,University of Toulouse III,UPS,Toulouse,France
contact email	michel.simon@inserm.fr
lab head
Carine Froment
contact affiliation	IPBS-CNRS
contact email	carine.froment@ipbs.fr
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/2019/02/PXD011833

PRIDE project URI

• PRIDE
  1. PXD011833
     1. Label: PRIDE project
     2. Name: The Amino-Terminal Part of Human Filaggrin-2 is a Component of Cornified Envelopes