STRs in panel
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Congenital muscular dystrophy and congenital myopathy

Gene: HACD1

Green List (high evidence)

HACD1 (3-hydroxyacyl-CoA dehydratase 1)
EnsemblGeneIds (GRCh38): ENSG00000165996
EnsemblGeneIds (GRCh37): ENSG00000165996
OMIM: 610467, Gene2Phenotype
HACD1 is in 3 panels

9 reviews

Eleanor Williams (Genomics England Curator)

Green List (high evidence)

The rating of this gene has been updated to green following NHS Genomic Medicine Service approval.
Created: 1 Feb 2023, 5:10 p.m. | Last Modified: 1 Feb 2023, 5:10 p.m.
Panel Version: 3.14

Sarah Leigh (Genomics England Curator)

Green List (high evidence)

Tracy Lester (Genetics laboratory, Oxford UK)

Green List (high evidence)

Three publications showing 5 independent cases with a similar phenotype of congenital myopathy and homozygous variants in this gene. Also a homozygous labrador that shows myopathy. There is now sufficient evidence to rate this as green in regards to myopathy and hypotonia.
Created: 21 Jun 2022, 1:23 p.m. | Last Modified: 21 Jun 2022, 1:23 p.m.
Panel Version: 2.83

Mode of inheritance
BIALLELIC, autosomal or pseudoautosomal

Phenotypes
Hypotonia; muscle weakness; motor delay

Publications

Ivone Leong (Genomics England Curator)

Green List (high evidence)

This gene is associated with a relevant phenotype in Gene2Phenotype and not OMIM. There is enough evidence to support a gene-disease association. This gene should be rated Green at the next review.
Created: 30 Jun 2021, 2:17 p.m. | Last Modified: 30 Jun 2021, 2:17 p.m.
Panel Version: 2.43
Comment on publications: PMID: 33354762. Three additional cases.
Created: 30 Jun 2021, 2:15 p.m. | Last Modified: 30 Jun 2021, 2:15 p.m.
Panel Version: 2.42

Zornitza Stark (Australian Genomics)

Green List (high evidence)

Two unrelated families and a dog model.
Created: 6 Jul 2020, 7:50 a.m. | Last Modified: 6 Jul 2020, 7:50 a.m.
Panel Version: 2.5

Mode of inheritance
BIALLELIC, autosomal or pseudoautosomal

Phenotypes
Congenital myopathy

Publications

Louise Daugherty (Genomics England Curator)

I don't know

Gene rating and review submitted by Rachael Mein, Viapath Guy's Hospital February 2019 on on behalf of London South GLH for the GMS Neurology specialist test group.
Created: 30 Apr 2019, 10:09 a.m.

Rachael Mein (Viapath at Guy's Hospital)

Green List (high evidence)

Mode of inheritance
BIALLELIC, autosomal or pseudoautosomal

Phenotypes
congenital myopathy

Publications

Variants in this GENE are reported as part of current diagnostic practice

Helen Brittain (Genomics England Curator)

Comment when marking as ready: Reviewer contacted to see if they have further cases, amber and watchlist on current evidence.
Created: 7 Mar 2017, 2:52 p.m.
Comment on publications: Only one family
Created: 7 Mar 2017, 2:51 p.m.
Comment on list classification: 1 family in PMID 23933735. Reviewer contacted to see if they have further cases, amber and watchlist on current evidence.
Created: 7 Mar 2017, 2:51 p.m.

Anna Sarkozy (Great Ormond Street Hospital)

I don't know

variants were described in a single family in literature. no further confirmation to date
Created: 30 May 2019, 4:52 p.m.
it is going to be added in the new diagnostic panel
Created: 6 Mar 2017, 12:14 p.m.

Mode of inheritance
BIALLELIC, autosomal or pseudoautosomal

Phenotypes
congenital myopathy

Details

Mode of Inheritance
BIALLELIC, autosomal or pseudoautosomal
Sources
  • London South GLH
  • Expert Review Green
  • Expert Review
  • NHS GMS
Phenotypes
  • Myopathy, congenital, nonprogressive, OMIM:619967
OMIM
610467
Clinvar variants
Variants in HACD1
Penetrance
Complete
Publications
Panels with this gene

History Filter Activity

3 Feb 2023, Gel status: 3

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

Arina Puzriakova (Genomics England Curator)

gene: HACD1 was added gene: HACD1 was added to Congenital muscular dystrophy and congenital myopathy. Sources: NHS GMS,Expert Review,Expert Review Green,London South GLH Mode of inheritance for gene: HACD1 was set to BIALLELIC, autosomal or pseudoautosomal Publications for gene: HACD1 were set to 23933735; 15829503; 32426512; 33354762 Phenotypes for gene: HACD1 were set to Myopathy, congenital, nonprogressive, OMIM:619967 Penetrance for gene: HACD1 were set to Complete