PXD021165

PXD021165 is an original dataset announced via ProteomeXchange.

Title	Overexpression of the human mutation P209L of cochaperone BAG3 in mouse
Description	An amino acid exchange (P209L) in the HSPB8 binding site of the human cochaperone Bcl2-associated athanogene 3 (BAG3) gives rise to severe dominant childhood cardiomyopathy. To phenocopy the disease in mouse and gain insight into its mechanisms, we have generated humanized transgenic mouse models. Expression of human BAG3P209L-eGFP in mice caused Z-disc disintegration and formation of protein aggregates containing BAG3, components of the Z-disc, and the protein quality control system in cardiomyocytes. This was accompanied by massive fibrosis resulting in a severe, early-onset restrictive cardiomyopathy with increased mortality, as observed in patients. Here we present the shotgun proteome data of mice expressing hBAG3P209L-eGFP compared to control.
HostingRepository	PRIDE
AnnounceDate	2021-04-13
AnnouncementXML	Submission_2021-04-13_01:45:56.624.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Pitter Huesgen
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationList	iodoacetamide derivatized residue
Instrument	maXis

Revision	Datetime	Status	ChangeLog Entry
0	2020-08-27 03:33:08	ID requested
⏵ 1	2021-04-13 01:45:56	announced

Dataset with its publication pending

submitter keyword: Heart, Mouse, human BAG3 P209L

Pitter F Huesgen
contact affiliation	ZEA-3 Analytics Forschungszentrum Jülich
contact email	p.huesgen@fz-juelich.de
lab head
Pitter Huesgen
contact affiliation	Forschungszentrum Jülich
contact email	p.huesgen@fz-juelich.de
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD021165
     1. Label: PRIDE project
     2. Name: Overexpression of the human mutation P209L of cochaperone BAG3 in mouse