DSP (desmoplakin)

2007-02-01  

Identity

HGNC
LOCATION
6p24.3
LOCUSID
ALIAS
DCWHKTA,DP
FUSION GENES

Other Information

Locus ID:

NCBI: 1832
MIM: 125647
HGNC: 3052
Ensembl: ENSG00000096696

Variants:

dbSNP: 1832
ClinVar: 1832
TCGA: ENSG00000096696
COSMIC: DSP

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000096696ENST00000379802P15924
ENSG00000096696ENST00000418664P15924

Expression (GTEx)

0
500
1000
1500

Pathways

PathwaySourceExternal ID
Arrhythmogenic right ventricular cardiomyopathy (ARVC)KEGGko05412
Arrhythmogenic right ventricular cardiomyopathy (ARVC)KEGGhsa05412
Immune SystemREACTOMER-HSA-168256
Innate Immune SystemREACTOMER-HSA-168249
Programmed Cell DeathREACTOMER-HSA-5357801
ApoptosisREACTOMER-HSA-109581
Apoptotic execution phaseREACTOMER-HSA-75153
Apoptotic cleavage of cellular proteinsREACTOMER-HSA-111465
Apoptotic cleavage of cell adhesion proteinsREACTOMER-HSA-351906
Developmental BiologyREACTOMER-HSA-1266738
Neutrophil degranulationREACTOMER-HSA-6798695
KeratinizationREACTOMER-HSA-6805567
Formation of the cornified envelopeREACTOMER-HSA-6809371

Protein levels (Protein atlas)

Not detected
Low
Medium
High

PharmGKB

Entity IDNameTypeEvidenceAssociationPKPDPMIDs
PA134871907TMEM43GeneDataAnnotationassociated
PA27489DSC2GeneDataAnnotationassociated
PA27502DSG2GeneDataAnnotationassociated
PA33357PKP2GeneDataAnnotationassociated
PA446847Arrhythmogenic Right Ventricular DysplasiaDiseaseDataAnnotation, Literature, MultilinkAnnotationassociated23788249

References

Pubmed IDYearTitleCitations
385097112024Epidermal growth factor receptor inhibition leads to cellular phenotype correction of DSP-mutated keratinocytes.1
385097112024Epidermal growth factor receptor inhibition leads to cellular phenotype correction of DSP-mutated keratinocytes.1
365803162023Variant Location Is a Novel Risk Factor for Individuals With Arrhythmogenic Cardiomyopathy Due to a Desmoplakin (DSP) Truncating Variant.7
367688122023DSP-Related Cardiomyopathy as a Distinct Clinical Entity? Emerging Evidence from an Italian Cohort.6
365803162023Variant Location Is a Novel Risk Factor for Individuals With Arrhythmogenic Cardiomyopathy Due to a Desmoplakin (DSP) Truncating Variant.7
367688122023DSP-Related Cardiomyopathy as a Distinct Clinical Entity? Emerging Evidence from an Italian Cohort.6
350089562022Missense Mutations in Desmoplakin Plakin Repeat Domains Have Dramatic Effects on Domain Structure and Function.1
353487022022Analysis of incidental findings in Qatar genome participants reveals novel functional variants in LMNA and DSP.1
354454682022A novel desmoplakin mutation causes dilated cardiomyopathy with palmoplantar keratoderma as an early clinical sign.3
354701092022Clinical profile and long-term follow-up of a cohort of patients with desmoplakin cardiomyopathy.18
358164322022Colocalization of Gene Expression and DNA Methylation with Genetic Risk Variants Supports Functional Roles of MUC5B and DSP in Idiopathic Pulmonary Fibrosis.11
350089562022Missense Mutations in Desmoplakin Plakin Repeat Domains Have Dramatic Effects on Domain Structure and Function.1
353487022022Analysis of incidental findings in Qatar genome participants reveals novel functional variants in LMNA and DSP.1
354454682022A novel desmoplakin mutation causes dilated cardiomyopathy with palmoplantar keratoderma as an early clinical sign.3
354701092022Clinical profile and long-term follow-up of a cohort of patients with desmoplakin cardiomyopathy.18

Citation

Dessen P

DSP (desmoplakin)

Atlas Genet Cytogenet Oncol Haematol. 2007-02-01

Online version: http://atlasgeneticsoncology.org/gene/45936/dsp-(desmoplakin)