Mosaic skin disorders - deep sequencing

Gene: MVD

Green List (high evidence)

MVD (mevalonate diphosphate decarboxylase)
EnsemblGeneIds (GRCh38): ENSG00000167508
EnsemblGeneIds (GRCh37): ENSG00000167508
OMIM: 603236, Gene2Phenotype
MVD is in 3 panels

4 reviews

Achchuthan Shanmugasundram (Genomics England Curator)

Green List (high evidence)

The rating of this gene has been updated to green following NHS Genomic Medicine Service approval.
Created: 6 Dec 2024, 11:18 a.m. | Last Modified: 6 Dec 2024, 11:18 a.m.
Panel Version: 2.49

Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

Arina Puzriakova (Genomics England Curator)

Comment on list classification: At least 5 individuals reported (PMID: 30942823; 33491095) which meets the criteria for classifying this gene-disease association as Green at the next GMS panel update.
Created: 2 Apr 2024, 3:41 p.m. | Last Modified: 2 Apr 2024, 3:41 p.m.
Panel Version: 2.47
Porokeratoses are a heterogeneous group of keratinization disorders. For linear porokeratosis and disseminated superficial actinic porokeratosis, a heterozygous pathogenic germline variant in a mevalonate pathway gene and a postzygotic second hit mutation present in affected skin have been shown to be the pathogenetic mechanism for the development of the lesions.
Created: 2 Apr 2024, 3:37 p.m. | Last Modified: 2 Apr 2024, 3:37 p.m.
Panel Version: 2.46

Zornitza Stark (Australian Genomics)

Green List (high evidence)

Somatic second hit, at least 5 individuals reported.
Created: 18 Feb 2021, 8:16 a.m. | Last Modified: 18 Feb 2021, 8:16 a.m.
Panel Version: 1.5

Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

Phenotypes
Porokeratosis 7, multiple types, MIM# 614714

Publications

Tom Cullup (Great Ormond Street Hospital)

I don't know

Heterozygous germline variants + second somatic hit (point mutation or LOH) in affected tissues.
Created: 5 Dec 2019, 3:40 p.m. | Last Modified: 5 Dec 2019, 3:40 p.m.
Panel Version: 0.16

Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

Phenotypes
Linear porokeratosis

Publications

Details

Mode of Inheritance
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • NHS GMS
  • Expert Review Green
Phenotypes
  • Porokeratosis 7, multiple types, OMIM:614714
OMIM
603236
Clinvar variants
Variants in MVD
Penetrance
None
Publications
Panels with this gene

History Filter Activity

6 Dec 2024, Gel status: 3

Removed Tag

Achchuthan Shanmugasundram (Genomics England Curator)

Tag Q2_24_promote_green was removed from gene: MVD.

6 Dec 2024, Gel status: 3

Added New Source, Added New Source, Status Update

Achchuthan Shanmugasundram (Genomics England Curator)

Source Expert Review Green was added to MVD. Source NHS GMS was added to MVD. Rating Changed from Amber List (moderate evidence) to Green List (high evidence)

2 Apr 2024, Gel status: 2

Added Tag

Arina Puzriakova (Genomics England Curator)

Tag Q2_24_promote_green tag was added to gene: MVD.

2 Apr 2024, Gel status: 2

Entity classified by Genomics England curator

Arina Puzriakova (Genomics England Curator)

Gene: mvd has been classified as Amber List (Moderate Evidence).

2 Apr 2024, Gel status: 2

Set publications

Arina Puzriakova (Genomics England Curator)

Publications for gene: MVD were set to 30942823

2 Apr 2024, Gel status: 2

Set Phenotypes

Arina Puzriakova (Genomics England Curator)

Phenotypes for gene: MVD were changed from Linear porokeratosis to Porokeratosis 7, multiple types, OMIM:614714

5 Dec 2019, Gel status: 2

Entity classified by Genomics England curator

Catherine Snow (Genomics England)

Gene: mvd has been classified as Amber List (Moderate Evidence).

5 Dec 2019, Gel status: 1

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

Catherine Snow (Genomics England)

gene: MVD was added gene: MVD was added to Mosaic skin disorders - deep sequencing. Sources: Expert Review Red Mode of inheritance for gene: MVD was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted Publications for gene: MVD were set to 30942823 Phenotypes for gene: MVD were set to Linear porokeratosis