PXD008896

PXD008896 is an original dataset announced via ProteomeXchange.

Title	Protein interaction networks for Matrin 3 encoding mutations
Description	To understand how mutations in Matrin 3 (MATR3) cause amyotrophic lateral sclerosis (ALS) and distal myopathy, we used transcriptome and interactome analysis. We found over-expression of wild-type (WT) or F115C mutant MATR3 had little impact on gene expression in neuroglia cells. We identified ~123 proteins that bound MATR3, with proteins associated with stress granules and RNA processing/splicing being prominent. The interactome of myopathic S85C and ALS-variant F115C MATR3 were virtually identical to WT protein. Deletion of RNA recognition motif (RRM1) or Zn finger motifs (ZnF1 or ZnF2) diminished the binding of a subset of MATR3 interacting proteins. Remarkably, deletion of the RRM2 motif caused enhanced binding of >100 hundred proteins. In live cells, MATR3 lacking RRM2 (ΔRRM2) formed intranuclear spherical structures that fused over time into large structures. Our findings in the cell models used here suggest that MATR3 with disease-causing mutations is not dramatically different from WT protein in modulating gene regulation or in binding to normal interacting partners. The intra-nuclear localization and interaction network of MATR3 is strongly modulated by its RRM2 domain.
HostingRepository	PRIDE
AnnounceDate	2018-08-03
AnnouncementXML	Submission_2018-08-03_09:06:09.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Guilian Xu
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	2-pyrrolidone-5-carboxylic acid (Gln); ubiquitination signature dipeptidyl lysine; monohydroxylated residue; iodoacetamide derivatized residue; deamidated residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2018-02-08 01:38:31	ID requested
⏵ 1	2018-08-03 09:06:11	announced

Iradi MCG, Triplett JC, Thomas JD, Davila R, Crown AM, Brown H, Lewis J, Swanson MS, Xu G, Rodriguez-Lebron E, Borchelt DR, Characterization of gene regulation and protein interaction networks for Matrin 3 encoding mutations linked to amyotrophic lateral sclerosis and myopathy. Sci Rep, 8(1):4049(2018) [pubmed]

curator keyword: Biological

submitter keyword: Matrin 3, interaction

David R. Borchelt
contact affiliation	Department of Neuroscience Center of Translational Research on Neurodegenerative Disesae University of Florida
contact email	drb1@ufl.edu
lab head
Guilian Xu
contact affiliation	Neuroscience
contact email	xugl@ufl.edu
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD008896
     1. Label: PRIDE project
     2. Name: Protein interaction networks for Matrin 3 encoding mutations