PXD004020

PXD004020 is an original dataset announced via ProteomeXchange.

Title	Molecular signatures of membrane protein complexes underlying muscular dystrophy
Description	Mutations in genes encoding components of the sarcolemmal dystrophin-glycoprotein complex (DGC) are responsible for a large number of muscular dystrophies. As such, molecular dissection of the DGC is expected to both reveal pathological mechanisms, and provides a biological framework for validating new DGC components. Establishment of the molecular composition of plasma-membrane protein complexes has been hampered by a lack of suitable biochemical approaches. Here we present an analytical workflow based upon the principles of protein correlation profiling that has enabled us to model the molecular composition of plasma-membrane protein complexes in mouse skeletal muscle. We also report our analysis of protein complexes in mice harboring mutations in DGC components, which implicates cell-adhesion pathways directly targeted by NFκB in the pathophysiology of DGC-related muscular dystrophies. Our study suggests that inflammatory and compensatory mechanisms are activated in these diseases. Additionally, it provides a molecular framework that will facilitate refinement of our understanding of the DGC, identification of protein biomarkers of neuromuscular disease, and pharmacological interrogation of the DGC in adult skeletal muscle.
HostingRepository	PRIDE
AnnounceDate	2017-10-24
AnnouncementXML	Submission_2017-10-24_01:06:26.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Jordy Hsiao
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	ubiquitination signature dipeptidyl lysine; phosphorylated residue; monohydroxylated residue; iodoacetamide derivatized residue
Instrument	6520 Quadrupole Time-of-Flight LC/MS

Revision	Datetime	Status	ChangeLog Entry
0	2016-04-20 03:12:05	ID requested
1	2016-04-22 08:24:39	announced
2	2017-06-21 00:31:19	announced	Updated project metadata.
⏵ 3	2017-10-24 01:06:27	announced	Updated project metadata.

Turk R, Hsiao JJ, Smits MM, Ng BH, Pospisil TC, Jones KS, Campbell KP, Wright ME, Molecular Signatures of Membrane Protein Complexes Underlying Muscular Dystrophy. Mol Cell Proteomics, 15(6):2169-85(2016) [pubmed]

curator keyword: Biomedical

submitter keyword: Mouse, Skeletal Muscle, directed MS, glycoproteomics, sucrose fractionation

Michael Eugene Wright
contact affiliation	Molecular Physiology & Biophysics, Wright Lab, University of Iowa, USA
contact email	michael-e-wright@uiowa.edu
lab head
Jordy Hsiao
contact affiliation	University of Iowa
contact email	jordy-hsiao@uiowa.edu
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD004020
     1. Label: PRIDE project
     2. Name: Molecular signatures of membrane protein complexes underlying muscular dystrophy