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PXD000978

PXD000978 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleProteomic profiling of MDC1A muscle using TMT quantitation, Bruno Oliveira
DescriptionSUMMARY 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.
HostingRepositoryPRIDE
AnnounceDate2014-07-24
AnnouncementXMLSubmission_2014-07-24_05:17:57.xml
DigitalObjectIdentifier
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterCarina Sihlbom
SpeciesList scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationListL-cysteine methyl disulfide; TMT6plex-126 reporter+balance reagent acylated residue
InstrumentLTQ Orbitrap
Dataset History
RevisionDatetimeStatusChangeLog Entry
02014-05-15 03:37:57ID requested
12014-07-08 06:02:22announced
22014-07-24 05:17:58announcedUpdated project metadata.
Publication List
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]
Keyword List
curator keyword: Biomedical
submitter keyword: laminin α2 chain deficient muscle, metabolic alteration, calcium dysregulation, extracellular matrix proteins
Contact List
Carina Sihlbom
contact affiliationProteomics Core Facility, Sahlgrenska Academy Gothenburg University
contact emailcarina.sihlbom@gu.se
lab head
Carina Sihlbom
contact affiliationProteomics Core Facility / University of Gothenburg
contact emailcarina.sihlbom@gu.se
dataset submitter
Full Dataset Link List
Dataset FTP location
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