Reactome | PKP2 [plasma membrane]

PKP2 [plasma membrane]

Stable Identifier

R-HSA-6814393

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

plasma membrane

Synonyms

Plakophilin-2, PKP2_HUMAN

Locations in the PathwayBrowser

Expand all

Developmental Biology (Homo sapiens)

- Keratinization (Homo sapiens)
  - Formation of the cornified envelope (Homo sapiens)
    - CASP14 cleaves filaggrin (Homo sapiens)
      - Late cornified envelope [plasma membrane] (Homo sapiens)
        
        Cornified envelope:CDSN [plasma membrane] (Homo sapiens)
        
        Cornified envelope [plasma membrane] (Homo sapiens)
        
        Early cornified envelope [plasma membrane] (Homo sapiens)
        
        FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
      - Mature cornified envelope [plasma membrane] (Homo sapiens)
        
        No FGN-cornified envelope:CDSN [plasma membrane] (Homo sapiens)
        
        No FGN-Cornified envelope [plasma membrane] (Homo sapiens)
        
        No FGN-late cornified envelope [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome network [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - CDSN binds the cornified envelope (Homo sapiens)
      - Cornified envelope [plasma membrane] (Homo sapiens)
        
        Early cornified envelope [plasma membrane] (Homo sapiens)
        
        FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
      - Cornified envelope:CDSN [plasma membrane] (Homo sapiens)
        
        Cornified envelope [plasma membrane] (Homo sapiens)
        
        Early cornified envelope [plasma membrane] (Homo sapiens)
        
        FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - Filaggrin binds Keratin tonofilament:Desmosome (Homo sapiens)
      - FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
      - Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - Fillagrin and keratin intermediate filaments polymerize forming a network (Homo sapiens)
      - FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
      - FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - Lamellar bodies bind the early cornified envelope (Homo sapiens)
      - Cornified envelope [plasma membrane] (Homo sapiens)
        
        Early cornified envelope [plasma membrane] (Homo sapiens)
        
        FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
      - Early cornified envelope [plasma membrane] (Homo sapiens)
        
        FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - Late envelope proteins bind cornified envelope:CDSN (Homo sapiens)
      - Cornified envelope:CDSN [plasma membrane] (Homo sapiens)
        
        Cornified envelope [plasma membrane] (Homo sapiens)
        
        Early cornified envelope [plasma membrane] (Homo sapiens)
        
        FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
      - Late cornified envelope [plasma membrane] (Homo sapiens)
        
        Cornified envelope:CDSN [plasma membrane] (Homo sapiens)
        
        Cornified envelope [plasma membrane] (Homo sapiens)
        
        Early cornified envelope [plasma membrane] (Homo sapiens)
        
        FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - PERP binds desmosomes (Homo sapiens)
      - Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
      - Desmosome:PERP [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - Plasma membrane resorption (Homo sapiens)
      - Mature cornified envelope [plasma membrane] (Homo sapiens)
        
        No FGN-cornified envelope:CDSN [plasma membrane] (Homo sapiens)
        
        No FGN-Cornified envelope [plasma membrane] (Homo sapiens)
        
        No FGN-late cornified envelope [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome network [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - Reinforcement of the Cornified Envelope (Homo sapiens)
      - Early cornified envelope [plasma membrane] (Homo sapiens)
        
        FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
      - FLG-Keratin filament network [plasma membrane] (Homo sapiens)
        
        FLG(?-?):Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
        
        Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
  - Keratin filaments bind cell-cell adhesion complexes (Homo sapiens)
    - Desmosome [plasma membrane] (Homo sapiens)
      - Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)
    - Keratin tonofilament:Desmosome [plasma membrane] (Homo sapiens)
      - Desmosome [plasma membrane] (Homo sapiens)
        
        Plakophilins [plasma membrane] (Homo sapiens)
        
        PKP2 [plasma membrane] (Homo sapiens)

External Reference Information

External Reference

UniProt:Q99959 PKP2

Gene Names

PKP2

Chain

chain:1-881

Reference Genes

BioGPS Gene:5318 PKP2

COSMIC (genes):PKP2 PKP2

CTD Gene:5318 PKP2

dbSNP Gene:5318 PKP2

ENSEMBL:ENSG00000057294 PKP2

HGNC:9024 PKP2

KEGG:hsa:5318 PKP2

Monarch:5318 PKP2

NCBI Gene:5318 PKP2

OMIM:602861 PKP2

UCSC:Q99959 PKP2

Reference Transcript

Participates

as a member of

Plakophilins [plasma membrane] (Homo sapiens)

Other forms of this molecule

PKP2 [cornified envelope]

Inferred To

pkp2 [plasma membrane] (Danio rerio)

pkp2 [plasma membrane] (Xenopus tropicalis)

PKP2 [plasma membrane] (Bos taurus)

PKP2 [plasma membrane] (Canis familiaris)

PKP2 [plasma membrane] (Gallus gallus)

PKP2 [plasma membrane] (Sus scrofa)

Pkp2 [plasma membrane] (Mus musculus)

Pkp2 [plasma membrane] (Rattus norvegicus)

jac-1 [plasma membrane] (Caenorhabditis elegans)

Cross References

ENSEMBL

ENST00000070846, ENSP00000070846, ENST00000340811, ENSP00000342800, ENSG00000057294

OpenTargets

ENSG00000057294

GeneCards

PKP2

HPA

ENSG00000057294-PKP2

PRO

Q99959

Pharos - Targets

Q99959

GlyGen

Q99959

Orphanet

PKP2

PDB

3TT9

Interactors (7)

Accession	#Entities	Entities	Confidence Score	Evidence (IntAct)
UniProt:P31947 SFN	1	SFN [cytosol] (R-HSA-1445105)	0.628	5
UniProt:P31946 YWHAB	1	YWHAB [cytosol] (R-HSA-48888)	0.615	4
UniProt:P63104 YWHAZ	2	YWHAZ [cytosol] (R-HSA-206099) YWHAZ [nucleoplasm] (R-HSA-450469)	0.615	3
UniProt:P62258 YWHAE	1	YWHAE [cytosol] (R-HSA-194368)	0.604	5
UniProt:P03431 PB1	4	PB1 [extracellular region] (R-FLU-169246) PB1 [endosome lumen] (R-FLU-189137) PB1 [cytosol] (R-FLU-195940) PB1 [nucleoplasm] (R-FLU-188843)	0.599	8
UniProt:P00533 EGFR	20	p-6Y-EGFR [clathrin-coated endocytic vesicle membrane] (R-HSA-8869138) p-5Y-EGFRvIII [plasma membrane] (R-HSA-1248649) EGFRvIII [plasma membrane] (R-HSA-1214162) p-6Y-EGFR E746_A750del;T790M [plasma membrane] (R-HSA-1182985) p-6Y-EGFR L858R;T790M [plasma membrane] (R-HSA-1182984) p-6Y-EGFR A767_V769dup [plasma membrane] (R-HSA-1996343) p-6Y-EGFR N771_H773dup [plasma membrane] (R-HSA-1996342) p-6Y-EGFR K739_I744dup [plasma membrane] (R-HSA-1996346) p-6Y-EGFR D770_N771insNPG [plasma membrane] (R-HSA-1996335) p-6Y-EGFR E746_A750del [plasma membrane] (R-HSA-1181214) p-6Y-EGFR L858R [plasma membrane] (R-HSA-1173211) p-6Y-EGFR G719S [plasma membrane] (R-HSA-1169417) p-6Y-EGFR L747_P753delinsS [plasma membrane] (R-HSA-1228038) p-6Y-EGFR L747_A750delinsP [plasma membrane] (R-HSA-1228044) p-6Y-EGFR L747_T751del [plasma membrane] (R-HSA-1228041) p-6Y-EGFR L747_S752del [plasma membrane] (R-HSA-1228040) p-6Y-EGFR L747_T751delinsP [plasma membrane] (R-HSA-1228042) p-6Y-EGFR E746_T751delinsA [plasma membrane] (R-HSA-1228037) p-6Y-EGFR E746_S752delinsV [plasma membrane] (R-HSA-1228039) p-6Y-EGFR L861Q [plasma membrane] (R-HSA-1177541)	0.483	3
UniProt:P13569 CFTR	17	misfolded CFTR [endoplasmic reticulum membrane] (R-HSA-8868730) CFTR [endoplasmic reticulum membrane] (R-HSA-8866837) CFTR [plasma membrane] (R-HSA-383188) CFTR N1303K [plasma membrane] (R-HSA-5678983) CFTR F508del [plasma membrane] (R-HSA-5678978) CFTR W1282* [plasma membrane] (R-HSA-5678976) CFTR G551D [plasma membrane] (R-HSA-5678977) CFTR G542* [plasma membrane] (R-HSA-5678986) ub-CFTR F508del [endoplasmic reticulum membrane] (R-HSA-8866842) ub-CFTR F508del [cytosol] (R-HSA-8866838) CFTR F508del [endoplasmic reticulum membrane] (R-HSA-8866834) PolyUb-CFTR [endosome membrane] (R-HSA-6782976) CFTR [endosome membrane] (R-HSA-6782965) CFTR [Golgi-associated vesicle membrane] (R-HSA-5627079) CFTR [lysosomal membrane] (R-HSA-5627279) PolyUb-CFTR [plasma membrane] (R-HSA-8867595) PolyUb-CFTR [clathrin-coated endocytic vesicle membrane] (R-HSA-8869104)	0.483	12

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