PXD011139

PXD011139 is an original dataset announced via ProteomeXchange.

Title	Myh10 deficiency leads to defective extracellular matrix remodeling and pulmonary disease
Description	Impaired alveolar formation and maintenance are features of many pulmonary diseases that are associated with significant morbidity and mortality. In a forward genetic screen for modulators of mouse lung development, we identified the non-muscle myosin II heavy chain gene, Myh10. Myh10 mutant pups exhibit cyanosis and respiratory distress, and die shortly after birth from differentiation defects in alveolar epithelium and mesenchyme. From omics analyses and follow up studies, we find decreased Thrombospondin expression accompanied with increased matrix metalloproteinase activity in both mutant lungs and cultured mutant fibroblasts, as well as disrupted extracellular matrix (ECM) remodeling. Loss of Myh10 specifically in mesenchymal cells results in ECM deposition defects and alveolar simplification. Notably, MYH10 expression is down-regulated in the lung of emphysema patients. Altogether, our findings reveal critical roles for Myh10 in alveologenesis at least in part via the regulation of ECM remodeling, which may contribute to the pathogenesis of emphysema.
HostingRepository	PRIDE
AnnounceDate	2018-09-19
AnnouncementXML	Submission_2018-11-26_08:58:03.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Anna Sokol
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	monohydroxylated residue; acetylated residue; iodoacetamide derivatized residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2018-09-19 00:48:45	ID requested
1	2018-09-19 04:23:29	announced
⏵ 2	2018-11-26 08:58:05	announced	Updated publication reference for PubMed record(s): 30389913.

Kim HT, Yin W, Jin YJ, Panza P, Gunawan F, Grohmann B, Buettner C, Sokol AM, Preussner J, Guenther S, Kostin S, Ruppert C, Bhagwat AM, Ma X, Graumann J, Looso M, Guenther A, Adelstein RS, Offermanns S, Stainier DYR, Myh10 deficiency leads to defective extracellular matrix remodeling and pulmonary disease. Nat Commun, 9(1):4600(2018) [pubmed]

curator keyword: Biomedical

submitter keyword: Myh10/NM IIB

ECM remodeling

MMP

THBS

lung development

emphysema

Didier Y.R. Stainier
contact affiliation	Department of Developmental Genetics, Max Planck Institute for Heart and Lung Research, Bad Nauheim, Germany
contact email	didier.stainier@mpi-bn.mpg.de
lab head
Anna Sokol
contact affiliation	Biomolecular Mass Spectrometry, Max Planck Institute for Heart and Lung Research, Bad Nauheim, Germany
contact email	anna.sokol@mpi-bn.mpg.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/2018/09/PXD011139

PRIDE project URI

• PRIDE
  1. PXD011139
     1. Label: PRIDE project
     2. Name: Myh10 deficiency leads to defective extracellular matrix remodeling and pulmonary disease