PXD027096

PXD027096 is an original dataset announced via ProteomeXchange.

Title	Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development
Description	Analysis of mouse Adamts6 and Adamts10 mutant embryos, lacking these homologous secreted metalloproteases individually and in combination, along with in vitro analysis of microfibrils, measurement of ADAMTS6-fibrillin affinities and N-terminomics determination of ADAMTS6-cleaved sites, demonstrates a transcriptionally adapted system for fibrillin-2 proteolysis that contributes to postnatal fibrillin-1 dominance. The lack of ADAMTS6, alone and in combination with ADAMTS10 led to excess fibrillin-2 in perichondrium, with impaired skeletal development. Although ADAMTS6 cleaves fibrillin-1 and fibrillin-2 as well as fibronectin, which provides the initial scaffold for microfibril assembly, primacy of the protease-substrate relationship between ADAMTS6 and fibrillin-2 was unequivocally established by reversal of these defects in Adamts6-/- embryos by genetic reduction of Fbn2, but not Fbn1.
HostingRepository	PRIDE
AnnounceDate	2024-10-07
AnnouncementXML	Submission_2024-10-07_13:41:11.739.xml
DigitalObjectIdentifier	https://dx.doi.org/10.6019/PXD027096
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Supported dataset by repository
PrimarySubmitter	Daniel Martin
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	dimethylated L-arginine; 2x(13)C,4x(2)H labeled dimethylated residue; monohydroxylated residue; deamidated residue
Instrument	Orbitrap Fusion Lumos

Revision	Datetime	Status	ChangeLog Entry
0	2021-07-05 02:06:40	ID requested
⏵ 1	2024-10-07 13:41:12	announced

10.7554/elife.71142;

10.6019/PXD027096;

Mead TJ, Martin DR, Wang LW, Cain SA, Gulec C, Cahill E, Mauch J, Reinhardt D, Lo C, Baldock C, Apte SS, Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development. Elife, 11():(2022) [pubmed]

submitter keyword: fibrillin, fibronectin, HEK293T,ADAMTS6, extracellular matrix

Suneel Apte
contact affiliation	Department of Biomedical engineering, Cleveland clinic lerner research institute
contact email	aptes@ccf.org
lab head
Daniel Martin
contact affiliation	Cleveland Clinic
contact email	martin32@mail.usf.edu
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/2022/05/PXD027096

PRIDE project URI

• PRIDE
  1. PXD027096
     1. Label: PRIDE project
     2. Name: Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development