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Congenital muscular dystrophy and congenital myopathy

Gene: DNAJB6

Red List (low evidence)

DNAJB6 (DnaJ heat shock protein family (Hsp40) member B6)
EnsemblGeneIds (GRCh38): ENSG00000105993
EnsemblGeneIds (GRCh37): ENSG00000105993
OMIM: 611332, Gene2Phenotype
DNAJB6 is in 4 panels

2 reviews

Anna Sarkozy (Great Ormond Street Hospital)

Red List (low evidence)

Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

Phenotypes
Myofibrillar Myopathy, Dominant

Helen Brittain (Genomics England Curator)

Red List (low evidence)

Comment when marking as ready: Not a congenital presentation. Onset from mid-childhood to adulthood. More appropriate for a muscular dystrophy-type panel.
Created: 3 Feb 2017, 11:53 a.m.
Not a congenital presentation. Onset from mid-childhood to adulthood. More appropriate for a muscular dystrophy-type panel.
Created: 30 Jan 2017, 3:26 p.m.

Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown

Phenotypes
Muscular dystrophy, limb-girdle, type 1E 603511

Details

Mode of Inheritance
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Sources
  • Expert Review Red
  • Illumina TruGenome Clinical Sequencing Services
Phenotypes
  • Muscular dystrophy, limb-girdle, autosomal dominant 1, OMIM:603511
OMIM
611332
Clinvar variants
Variants in DNAJB6
Penetrance
Complete
Panels with this gene

History Filter Activity

3 Feb 2023, Gel status: 1

Created, Added New Source, Set mode of inheritance, Set Phenotypes, Set penetrance

Arina Puzriakova (Genomics England Curator)

gene: DNAJB6 was added gene: DNAJB6 was added to Congenital muscular dystrophy and congenital myopathy. Sources: Illumina TruGenome Clinical Sequencing Services,Expert Review Red Mode of inheritance for gene: DNAJB6 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown Phenotypes for gene: DNAJB6 were set to Muscular dystrophy, limb-girdle, autosomal dominant 1, OMIM:603511 Penetrance for gene: DNAJB6 were set to Complete