PKP2 (plakophilin 2)

2012-03-01  

Identity

HGNC
LOCATION
12p11.21
LOCUSID
ALIAS
ARVD9
FUSION GENES

Other Information

Locus ID:

NCBI: 5318
MIM: 602861
HGNC: 9024
Ensembl: ENSG00000057294

Variants:

dbSNP: 5318
ClinVar: 5318
TCGA: ENSG00000057294
COSMIC: PKP2

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000057294ENST00000070846Q99959
ENSG00000057294ENST00000340811Q99959
ENSG00000057294ENST00000546741A0A087WXY2

Expression (GTEx)

0
10
20
30
40
50
60
70

Pathways

PathwaySourceExternal ID
Arrhythmogenic right ventricular cardiomyopathy (ARVC)KEGGko05412
Arrhythmogenic right ventricular cardiomyopathy (ARVC)KEGGhsa05412
Developmental BiologyREACTOMER-HSA-1266738
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
PA27505DSPGeneDataAnnotationassociated
PA446847Arrhythmogenic Right Ventricular DysplasiaDiseaseDataAnnotation, Literature, MultilinkAnnotationassociated23788249

References

Pubmed IDYearTitleCitations
377261682024Downregulated expression of plakophilin-2 gene in patients with colon adenocarcinoma predicts an unfavorable prognosis and immune infiltrate.0
380500582024Biallelic PKP2 loss of function variants are associated with a lethal perinatal-onset biventricular dilated cardiomyopathy with excessive trabeculations and ventricular septal defects.0
377261682024Downregulated expression of plakophilin-2 gene in patients with colon adenocarcinoma predicts an unfavorable prognosis and immune infiltrate.0
380500582024Biallelic PKP2 loss of function variants are associated with a lethal perinatal-onset biventricular dilated cardiomyopathy with excessive trabeculations and ventricular septal defects.0
362809012023Plakophilin 2 regulates intestinal barrier function by modulating protein kinase C activity in vitro.5
375103722023The Novel Variant NP_00454563.2 (p.Glu259Glyfs*77) in Gene PKP2 Associated with Arrhythmogenic Cardiomyopathy in 8 Families from Malaga, Spain.1
376079542023Impaired Plakophilin-2 in obesity breaks cell cycle dynamics to breed adipocyte senescence.2
362809012023Plakophilin 2 regulates intestinal barrier function by modulating protein kinase C activity in vitro.5
375103722023The Novel Variant NP_00454563.2 (p.Glu259Glyfs*77) in Gene PKP2 Associated with Arrhythmogenic Cardiomyopathy in 8 Families from Malaga, Spain.1
376079542023Impaired Plakophilin-2 in obesity breaks cell cycle dynamics to breed adipocyte senescence.2
349491592022Long non-coding RNA HCG11 enhances osteosarcoma phenotypes by sponging miR-1245b-5p that directly inhibits plakophilin 2.6
350631302022Modeling reduced contractility and impaired desmosome assembly due to plakophilin-2 deficiency using isogenic iPS cell-derived cardiomyocytes.14
355362392022Population Prevalence of Premature Truncating Variants in Plakophilin-2 and Association With Arrhythmogenic Right Ventricular Cardiomyopathy: A UK Biobank Analysis.2
356283492022Decreased Expression of Plakophilin-2 and αT-Catenin in Arrhythmogenic Right Ventricular Cardiomyopathy: Potential Markers for Diagnosis.1
359596572022Loss of Nuclear Envelope Integrity and Increased Oxidant Production Cause DNA Damage in Adult Hearts Deficient in PKP2: A Molecular Substrate of ARVC.12

Citation

Dessen P

PKP2 (plakophilin 2)

Atlas Genet Cytogenet Oncol Haematol. 2012-03-01

Online version: http://atlasgeneticsoncology.org/gene/52640/pkp2-(plakophilin-2)