PXD029834

PXD029834 is an original dataset announced via ProteomeXchange.

Title	Proteomic analysis of primary human microvascular endothelial cells (HMVECs) stimulated for 24 h with either 50 �g/mL recombinant Fibrillin-1 C-terminal fragment or 25 ng/ml VEGF-A compared to unstimulated cells.
Description	Mutations in the fibrillin-1 gene result in cardiovascular, ocular, and skeletal abnormalities in Marfan syndrome. Here we show that a fibrillin-1 mutation also alters the neovessel formation. In the mouse retina vascularization model, fibrillin-1 is detected at the basement membrane underlying the angiogenic front and around arterioles. In Fbn1C1041G/+ mice, a model of Marfan disease, the microvasculature is altered at these sites. At the front, endothelial tip-cell sprouting and branching are decreased, and we show that mutant fibrillin-1 delays angiogenesis by affecting Notch signalling. Supplying the growing vasculature of Fbn1C1041G/+ mice with a C-terminal fragment of fibrillin-1 corrects all defects. In vitro, the C-terminal fragment of fibrillin-1 also displays pro-angiogenic activities on microvascular endothelial cell. Our data establish that fibrillin-1 performs essential functions in microvessel formation and integrity and that mutant fibrillin-1-induced defects can be rescued pharmacologically.
HostingRepository	PRIDE
AnnounceDate	2023-11-14
AnnouncementXML	Submission_2023-11-14_08:48:27.784.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Dupuy Jean-William
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	acetylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	Orbitrap Fusion Lumos

Revision	Datetime	Status	ChangeLog Entry
0	2021-11-20 08:51:25	ID requested
1	2023-05-31 09:09:34	announced
⏵ 2	2023-11-14 08:48:36	announced	2023-11-14: Updated project metadata.

submitter keyword: Endothelial cell, quantitative proteome, angiogenesis

Elisabeth Genot
contact affiliation	Univ. Bordeaux, INSERM, Centre de Recherche cardio-thoracique de Bordeaux, U1045, F-33000 Bordeaux. France
contact email	elisabeth.genot@inserm.fr
lab head
Dupuy Jean-William
contact affiliation	Plateforme Proteome Bordeaux
contact email	jean-william.dupuy@u-bordeaux.fr
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD029834
     1. Label: PRIDE project
     2. Name: Proteomic analysis of primary human microvascular endothelial cells (HMVECs) stimulated for 24 h with either 50 �g/mL recombinant Fibrillin-1 C-terminal fragment or 25 ng/ml VEGF-A compared to unstimulated cells.