Gene

BAG3

BAG cochaperone 3

Cytogenetic location: 10q26.11
Coordinates: Chromosome 10: 119,651,380–119,677,819 (GRCh38)
Exons: 4 exons across 26.4 kb
Protein: 575 amino acids
MANE Select transcript: ENST00000369085.8NM_004281.4
Locus type: gene with protein product

Very high
Confident
Low
Very low

Reference genomeGRCh38hg38

Positions on gene and variant pages follow this choice, in this browser.

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Curated reports relevant to this gene

Dilated Cardiomyopathy
- Enlargement and weakening of the left ventricle not explained by coronary artery or valve disease; inherited forms involve TTN, LMNA, RBM20, FLNC, BAG3, DSP, MYH7, TNNT2, DES, PLN and many other genes, and include Barth syndrome and the Emery-Dreifuss muscular dystrophies.
  - ClinGen rates the link definitive
  - 4 GenCC submitters rate it definitive or strong
  - On the ACMG secondary findings list
  What knowing changes
  Knowing a genetic cause supports earlier heart monitoring and family screening, and for genes with a high arrhythmia risk such as LMNA, FLNC and RBM20 it informs decisions about rhythm monitoring and implantable defibrillators.
Myofibrillar Myopathies
- Myofibrillar myopathy
  A group of muscle disorders in which the contractile structure of muscle fibers breaks down, caused by genes such as DES, CRYAB, MYOT, LDB3, FLNC and BAG3.
  - ClinGen rates the link definitive
  - 5 GenCC submitters rate it definitive or strong
  - On the ACMG secondary findings list
  What knowing changes
  Because the heart is often involved, regular heart checks (such as ECG and echocardiography) are recommended even without symptoms.

How BAG3 is laid out

Coding sequence
Untranslated region
Intron
Direction of transcription
Known disease-causing sites
Stretches short reads find hard
Variants with a page

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 2.6 kb of the gene's 26.4 kb; 1,728 of those bases code for the protein.

How a genome test reads BAG3

116

positions in BAG3 where ClinVar classes a change as pathogenic or likely pathogenic

None of them falls in a region short reads find hard.

A whole genome covers all 26.4 kb of BAG3, introns included, not only the 2.6 kb of exons.

Variants with a page (14)

The BAG3 variants with a page here, most review stars first. Each page describes ClinVar's public record of the variant, never a result.

Variant	HGVS c.	Exon or intron	Position (GRCh38)	ClinVar classification	Review status
E455K	c.1363G>A	Exon 4	119,676,917	Pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
P209L	c.626C>T	Exon 3	119,672,373	Pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
P470S	c.1408C>T	Exon 4	119,676,962	Pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
Q251X	c.751C>T	Exon 3	119,672,498	Pathogenic/likely pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
R90X	c.268C>T	Exon 2	119,669,938	Pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
R121X	c.361C>T	Exon 2	119,670,031	Pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
R123X	c.367C>T	Exon 2	119,670,037	Pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
R309X	c.925C>T	Exon 4	119,676,479	Pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
R473X	c.1417C>T	Exon 4	119,676,971	Pathogenic/likely pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts
W26X	c.77G>A	Exon 1	119,651,752	Pathogenic/likely pathogenic	2 stars out of 4 criteria provided, multiple submitters, no conflicts

See all 14 BAG3 variants →

Traits linked to BAG3

The Human Phenotype Ontology lists these as features of the rare conditions BAG3 can cause. Each trait's page lists the other genes linked to it.

Abnormality of the musculoskeletal system

Show 16 more traits

Abnormality of limbs

Show 8 more traits

Abnormality of the nervous system

Show 6 more traits

Abnormality of the cardiovascular system

Show 2 more traits

Abnormality of the respiratory system

Laboratory abnormality

External links

Gene records

Transcript

Protein

Variants

Clinical

Where this comes from

HGNC: the gene's symbol, name, other symbols and location.
MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
The ACMG secondary findings list, SF v3.2.
ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). BAG3 gene: BAG cochaperone 3. Retrieved 16 October 2026, from https://dantelabs.com/genes/bag3

Vancouver

Dante Labs. BAG3 gene: BAG cochaperone 3 [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/bag3

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