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

ENSEMBL:ENSG00000057294.17 PKP2

HGNC:9024 PKP2

KEGG Gene (Homo sapiens):5318 PKP2

Monarch:5318 PKP2

NCBI Gene:5318 PKP2

OMIM:602861 PKP2

UCSC:Q99959 PKP2

Reference Transcript

Other Identifiers

11726130_a_at

11726131_a_at

16763032

207717_PM_s_at

207717_s_at

214154_PM_s_at

214154_s_at

33892_at

3402551

3402552

3402554

3402555

3402557

3402558

3450240

3450241

3450242

3450243

3450244

3450245

3450246

3450247

3450248

3450250

3450252

3450253

3450254

3450256

3450257

3450258

3450261

3450263

3450264

3450265

3450267

3450268

5318

7962212

A_33_P3405728

GE60196

GO:0001533

GO:0002159

GO:0002934

GO:0003012

GO:0003013

GO:0005080

GO:0005515

GO:0005634

GO:0005654

GO:0005737

GO:0005856

GO:0005882

GO:0005886

GO:0005911

GO:0005912

GO:0007010

GO:0007043

GO:0007155

GO:0007507

GO:0010765

GO:0010810

GO:0014704

GO:0016020

GO:0017080

GO:0019215

GO:0023052

GO:0030054

GO:0030057

GO:0030154

GO:0034330

GO:0043226

GO:0044325

GO:0045110

GO:0045294

GO:0045296

GO:0048496

GO:0048856

GO:0055010

GO:0060090

GO:0070161

GO:0072659

GO:0086002

GO:0086005

GO:0086019

GO:0086064

GO:0086073

GO:0086083

GO:0086091

GO:0098609

GO:0098631

GO:0098772

GO:0098911

GO:1900024

HMNXSV003006629

Hs.25051.2.A1_3p_a_at

ILMN_2386973

PH_hs_0040266

TC12001377.hg

X97675_rna1_at

g4758931_3p_a_at

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] (Bos taurus)

PKP2 [plasma membrane] (Canis familiaris)

PKP2 [plasma membrane] (Gallus gallus)

PKP2 [plasma membrane] (Sus scrofa)

PKP2 [plasma membrane] (Xenopus tropicalis)

Pkp2 [plasma membrane] (Mus musculus)

Pkp2 [plasma membrane] (Rattus norvegicus)

Cross References

RefSeq

NP_004563.2, NP_001005242.2

OpenTargets

ENSG00000057294

HPA

ENSG00000057294-PKP2

GeneCards

Q99959

Ensembl

ENSP00000342800, ENST00000340811, ENSG00000057294, ENSP00000070846, ENST00000070846

PRO

Q99959

Pharos - Targets

Q99959

Orphanet

15104

PDB

3TT9

Interactors (3)

Accession	#Entities	Entities	Confidence Score	Evidence (IntAct)
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	PolyUb-CFTR [clathrin-coated endocytic vesicle membrane] (R-HSA-8869104) PolyUb-CFTR [plasma membrane] (R-HSA-8867595) CFTR [lysosomal membrane] (R-HSA-5627279) CFTR [Golgi-associated vesicle membrane] (R-HSA-5627079) CFTR [endosome membrane] (R-HSA-6782965) PolyUb-CFTR [endosome membrane] (R-HSA-6782976) CFTR F508del [endoplasmic reticulum membrane] (R-HSA-8866834) ub-CFTR F508del [cytosol] (R-HSA-8866838) ub-CFTR F508del [endoplasmic reticulum membrane] (R-HSA-8866842) CFTR G542* [plasma membrane] (R-HSA-5678986) CFTR G551D [plasma membrane] (R-HSA-5678977) CFTR W1282* [plasma membrane] (R-HSA-5678976) CFTR F508del [plasma membrane] (R-HSA-5678978) CFTR N1303K [plasma membrane] (R-HSA-5678983) CFTR [plasma membrane] (R-HSA-383188) CFTR [endoplasmic reticulum membrane] (R-HSA-8866837) misfolded CFTR [endoplasmic reticulum membrane] (R-HSA-8868730)	0.483	12

Cite Us!