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ProteomicsOverexpression of the human mutation P209L of cochaperone BAG3 in mouse
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. E...
ORGANISM(S): Mus musculus (Mouse) 
2021-04-13 | PXD021165 | Pride
Heart Human bag3 p209l Mouse 
UnknownP209L mutation in Bag3 does not cause cardiomyopathy in mice.
Not available
S-EPMC6397380 | biostudies-literature
UnknownOverexpression of human BAG3P209L in mice causes restrictive cardiomyopathy.
Not available
S-EPMC8196106 | biostudies-literature
ProteomicsComparison of the skeletal muscle proteomes in wt and hBAG3P209L overexpressing mice
Myofibrillar myopathy-6 (MFM6) is rare, autosomal dominant neuromuscular disease that is characterised by devastating childhood muscular dystrophy, restrictive cardiomyopathy, and respiratory insufficiency. MFM-6 is caused by single base mutation in the gene encoding the co-chaperone BAG3 (Bcl-2 ass...
ORGANISM(S): Mus musculus (Mouse) 
2025-12-11 | PXD047942 | Pride
Myofibrillar myopathy-6  mouse model  proteome  skeletal muscle Bag3p209l Bag3 
TranscriptomicsOverexpression of human BAG3P209L in mice causes restrictive cardiomyopathy due to sarcomere disruption and protein aggregate formation
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 human disease in mouse and gain insight into its mechanisms, we have generated humanized transgenic mouse mod...
ORGANISM(S): Mus musculus 
2021-04-13 | GSE166862 | GEO
UnknownMitophagy Facilitates Cytosolic Proteostasis to Preserve Cardiac Function.
Not available
S-EPMC11623534 | biostudies-literature
GenomicsOverexpression of human BAG3P209L in mice causes restrictive cardiomyopathy due to sarcomere disruption and protein aggregate formation
Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy due to sarcomere disruption and protein aggregate formation
PRJNA702141 | ENA
Genomics
Transcriptomic analysis of skeletal muscle from CAG-BAG3P209L-mice
PRJNA1082302 | ENA
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