bag3 BCL2 associated athanogene 3 [Danio rerio (zebrafish)] - Gene

Summary

Official Symbol: bag3provided by ZNC
Official Full Name: BCL2 associated athanogene 3provided by ZNC
Primary source: ZFIN:ZDB-GENE-040801-40
See related: Ensembl:ENSDARG00000039486 AllianceGenome:ZFIN:ZDB-GENE-040801-40
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Danio rerio
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Actinopterygii; Neopterygii; Teleostei; Ostariophysi; Cypriniformes; Danionidae; Danioninae; Danio
Also known as: zgc:100859
Summary: Predicted to enable adenyl-nucleotide exchange factor activity and protein-folding chaperone binding activity. Acts upstream of or within several processes, including cardiac ventricle development; heart contraction; and skeletal muscle tissue development. Located in Z disc. Is expressed in fast muscle cell; heart; muscle; and slow muscle cell. Used to study cardiomyopathy; dilated cardiomyopathy; and myofibrillar myopathy 6. Human ortholog(s) of this gene implicated in dilated cardiomyopathy 1HH and myofibrillar myopathy 6. Orthologous to human BAG3 (BAG cochaperone 3). [provided by Alliance of Genome Resources, Jan 2025]
Orthologs: human mouse all
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Genomic context

Location:: chromosome: 13

Exon count:: 4

Annotation release	Status	Assembly	Chr	Location
RS_2024_08	current	GRCz11 (GCF_000002035.6)	13	NC_007124.7 (25486549..25499129)
105	previous assembly	GRCz10 (GCF_000002035.5)	13	NC_007124.6 (25356246..25368211)

Chromosome 13 - NC_007124.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

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Expression

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See details

Project title: Sequencing the Zebrafish transcriptome from a range of tissues and developmental stages
Description: Sequencing the Zebrafish transcriptome from a range of tissues and developmental stages
BioProject: PRJEB1986
Analysis date: Fri Dec 8 19:48:10 2017

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Metformin rescues muscle function in BAG3 myofibrillar myopathy models.
Title: Metformin rescues muscle function in BAG3 myofibrillar myopathy models.
Genetic compensation prevents myopathy and heart failure in an in vivo model of Bag3 deficiency.
Title: Genetic compensation prevents myopathy and heart failure in an in vivo model of Bag3 deficiency.
mtor is identified as a candidate therapeutic target gene for BAG3 cardiomyopathy.
Title: Haploinsufficiency of mechanistic target of rapamycin ameliorates bag3 cardiomyopathy in adult zebrafish.
expression of the co-chaperone BAG3 and other chaperone-assisted selective autophagy factors was analyzed in the cellular, zebrafish and ky/ky mouse models.
Title: Transcriptional upregulation of Bag3, a chaperone-assisted selective autophagy factor, in animal models of KY-deficient hereditary myopathy.
This study therefore identifies both BAG3 reduction and autophagy promotion as potential therapies for FLNC(W2710X) myofibrillar myopathy, and identifies protein insufficiency due to sequestration, compounded by impaired autophagy, as the cause.
Title: FLNC myofibrillar myopathy results from impaired autophagy and protein insufficiency.
findings showed that the P209L mutation causes BAG3 to aggregate; proposed that the gradual loss of available BAG3(wt) and BAG3(P209L) proteins results in insufficiency leading to myofibrillar disintegration
Title: Zebrafish models of BAG3 myofibrillar myopathy suggest a toxic gain of function leading to BAG3 insufficiency.

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General gene information

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Gene Ontology Provided by ZFIN

Function	Evidence Code	Pubs
enables adenyl-nucleotide exchange factor activity	IBA Inferred from Biological aspect of Ancestor more info
enables protein-folding chaperone binding	IBA Inferred from Biological aspect of Ancestor more info
enables protein-folding chaperone binding	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
acts_upstream_of_or_within cardiac ventricle development	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within heart contraction	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within locomotory behavior	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in muscle cell cellular homeostasis	IBA Inferred from Biological aspect of Ancestor more info
involved_in negative regulation of striated muscle cell apoptotic process	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein stabilization	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within skeletal muscle tissue development	IMP Inferred from Mutant Phenotype more info	PubMed

Component	Evidence Code	Pubs
located_in Z disc	IDA Inferred from Direct Assay more info	PubMed
is_active_in cytoplasm	IBA Inferred from Biological aspect of Ancestor more info
is_active_in cytosol	IBA Inferred from Biological aspect of Ancestor more info
is_active_in membrane	IBA Inferred from Biological aspect of Ancestor more info
is_active_in nucleus	IBA Inferred from Biological aspect of Ancestor more info

General protein information

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Preferred Names: BAG family molecular chaperone regulator 3

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.