PXD000978

PXD000978 is an original dataset announced via ProteomeXchange.

Title	Proteomic profiling of MDC1A muscle using TMT quantitation, Bruno Oliveira
Description	SUMMARY Congenital muscular dystrophy with laminin á2 chain-deficiency (MDC1A) is one of the most severe forms of muscular disease and is characterized by decreased muscle tone at birth, reduced movements and contractures. The genetic basis of MDC1A is well-known, yet the secondary mechanisms ultimately leading to muscle degeneration and subsequent connective tissue infiltration are not fully understood. In order to obtain new insights into the molecular mechanisms underlying MDC1A, we performed a comparative proteomic analysis of affected muscles (diaphragm and gastrocnemius) from laminin á2 chain-deficient dy3K/dy3K mice, using the multidimensional protein identification technology combined with tandem mass tags. Out of the around 700 identified proteins, 113 and 101 proteins, respectively, were differentially expressed in the diseased gastrocnemius and diaphragm muscles compared to normal muscles. A large portion of these proteins are involved in different metabolic processes, bind calcium or are expressed in the extracellular matrix. Our findings suggest that metabolic alterations and calcium dysregulation could be novel mechanisms that underlie MDC1A and might be targets that should be explored for therapy. Also, detailed knowledge of the composition of fibrotic tissue, rich in extracellular matrix proteins, in laminin á2 chain-deficient muscle may help to design future anti-fibrotic treatment.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_04:20:44.539.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Carina Sihlbom
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	TMT6plex-126 reporter+balance reagent acylated residue; L-cysteine methyl disulfide
Instrument	LTQ Orbitrap

Revision	Datetime	Status	ChangeLog Entry
0	2014-05-15 03:37:57	ID requested
1	2014-07-08 06:02:22	announced
2	2014-07-24 05:17:58	announced	Updated project metadata.
⏵ 3	2024-10-22 04:20:52	announced	2024-10-22: Updated project metadata.

de Oliveira BM, Matsumura CY, Fontes-Oliveira CC, Gawlik KI, Acosta H, Wernhoff P, Durbeej M, 2 chain-deficient muscle. Mol Cell Proteomics, 13(11):3001-13(2014) [pubmed]

curator keyword: Biomedical

submitter keyword: laminin α2 chain deficient muscle, metabolic alteration, extracellular matrix proteins, calcium dysregulation

Carina Sihlbom
contact affiliation	Proteomics Core Facility, Sahlgrenska Academy Gothenburg University
contact email	carina.sihlbom@gu.se
lab head
Carina Sihlbom
contact affiliation	Proteomics Core Facility / University of Gothenburg
contact email	carina.sihlbom@gu.se
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/2014/07/PXD000978

PRIDE project URI

• PRIDE
  1. PXD000978
     1. Label: PRIDE project
     2. Name: Proteomic profiling of MDC1A muscle using TMT quantitation, Bruno Oliveira