PXD036222

PXD036222 is an original dataset announced via ProteomeXchange.

Title	Understanding the role of TGFbeta signaling on aortic aneurysm and rupture using Marfan syndrome mouse models
Description	Although abnormal TGFbeta signaling is observed in several heritable forms of thoracic aortic aneurysms and dissections including Marfan syndrome, the precise role of TGFbeta signaling in aortic disease progression is still disputed. Using a mouse genetic approach and quantitative isobaric labeling proteomics, we sought to investigate the role of TGFbeta signaling in molecular pathways of pathogenesis associated with development of aortic aneurysm and aortic rupture. This study reports an isoform-specific effect of TGFbeta in MFS aortic disease and the effects of deleting the first hybrid domain of fibrillin-1 on TGFbeta signaling. Distinct molecular differences in mouse models of aneurysm (Fbn_GT-8_plus), of aneurysm and rupture (Fbn1_GT-8_H1delta), and of microdissection (Fbn1_H1delta_plus) were identified, which associated with TGFbeta signaling and extracellular matrix composition, possibly contributing to the development of dissection and rupture. These findings offer new insights into the pathophysiological mechanisms that potentially drive initiation of aortic dissection and could pave the way for development of new treatment targets of aortic disease.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_06:05:18.319.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Phillip Wilmarth
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	TMT6plex-126 reporter+balance reagent acylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Orbitrap Fusion

Revision	Datetime	Status	ChangeLog Entry
0	2022-08-22 03:47:18	ID requested
1	2023-10-09 11:22:31	announced
2	2023-11-14 09:05:56	announced	2023-11-14: Updated project metadata.
⏵ 3	2024-10-22 06:05:20	announced	2024-10-22: Updated project metadata.

Deleeuw V, Carlson E, Renard M, Zientek KD, Wilmarth PA, Reddy AP, Manalo EC, Tufa SF, Keene DR, Olbinado M, Stampanoni M, Kanki S, Yanagisawa H, Mosquera LM, Sips P, De Backer J, Sakai LY, signaling in thoracic aortic aneurysm and dissection using Fbn1 mutant mouse models. Matrix Biol, 123():17-33(2023) [pubmed]

10.1016/j.matbio.2023.09.001;

submitter keyword: mouse models, Marfan syndrome, PSR-OHSU, TGFbeta signaling,Fibrillin, quantitative proteomics, aortic aneurysm and dissection, tandem mass tags

Lynn Y. Sakai, Ph.D.
contact affiliation	Department of Molecular & Medical Genetics Oregon Health & Science University 3181 SW Sam Jackson Park Rd. Portland, Oregon 97239, USA
contact email	shlys@ohsu.edu
lab head
Phillip Wilmarth
contact affiliation	OHSU
contact email	wilmarth@ohsu.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/2023/10/PXD036222

PRIDE project URI

• PRIDE
  1. PXD036222
     1. Label: PRIDE project
     2. Name: Understanding the role of TGFbeta signaling on aortic aneurysm and rupture using Marfan syndrome mouse models