Keratinization (Homo sapiens)

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59423810, 142, 811, 2819, 444, 20, 28, 3545161118, 5540, 41, 532631, 33, 57, 5825, 4924, 43, 50263420, 30214647311, 5212, 27, 3917451, 22, 322958465834, 5415cytosolcytosolcornified envelopeKLK5 LCE5A PPL PKP1 DSG4 FLG(?-?)DSG2 SPRR2B Keratin type I, hair follicle specific Keratin associatedproteinsKLK5 LCE3B PPL CAPNS1 TGM1 DSC1 KRTAP3s LCE3E PKP2 LCE1A FLG-Keratin filament network DSG3 SPRR2E SPRR2D EVPL Keratin tonofilament:Desmosome network LCE1A JUP SPRR2F SPRR1B DSC2 KRTAP26-1 SPRR1B PKP3 JUP LCE2A RCOOHKeratin type II, hair specific DSC1 DSC3 LCE1F SPRR1A LIPK SPRR2E CASP14(153-242) proKLK8SPRR2F TGM5 KRTAP11-1 KRTAP6s SPRR2A IVL PI3(61-117) LOR LCE3C Keratin type I, hair follicle specific SPRR1B Keratin heterodimerPRSS8(45-322) RPTN CELA2ALCE1B DSP KLK13 SPRR2A SPRR1A LCE1D LCE1F DSG2 KLK12:SPINK6DSG1 SPRR2A Keratin type II, hair specific KRTAP13s LCE3E DSG3 KRTAP8-1 LCE2C Keratin type II, hair follicle specific KRTAP12s Keratin type I, hair specific SPRR2F Keratin type II, hair specific KLK5 propeptideDSG4 LCE1E LCE3A CASP14(153-242) LCE2C CDSNSPINK5(490-624)PKP4 DAGsDSG2 KLK8 propeptideLate cornifiedenvelopePPL EVPL LCE6A LCE1F RPTN Keratin type I, hair specific SPRR3 RPTN Ca2+ EVPL SPRR2A LCE1B Keratin type I, epithelial KLK8KRTAP27-1 CDSN TAGsLCE6A PKP1 TGM1:Ca2+, TGM5:Ca2+SPRR1B CytoplasmicprofilaggrinprocessingproteasesSPINK5(490-624) CSTA DSG3 DSC1 LCE3D TGM1 SPRR2G SPRR2B Keratin type I, hair follicle specific KLK5:SPINK5Keratin type I, hair specific PKP2 EVPL TGM1 DSC3 PKP4 proCELA2ASPRR3 CDSN SPINK6LOR PKP3 DSG3 H2OCHOL SPINK5(490-624)KRTAP24-1 SPRR2A SPRR2B Cornified envelopefatty acid Cell surface SPINK6 SPINK6 H2OKeratin type II, hair follicle specific JUP KRTAP17-1 SPRR2E ceramide TGM1 LCE6A FURIN LCE4A TGM1 SPRR1A PPL LCE3A LCE1E CHOL LIPN SPRR1B DSG4 LELP1 KAZNSPRR2B KLK14:SPINK6PERPLCE2B proKLK5LCE2B Cell surface PPL LORDesmosome:PERPSPRR3 Keratin type II, hair specific Keratin type II, hair specific LCE3C SPRR2A LCE1A TCHH KAZN IVL SPRR1A Keratin filamentLIPM LCE2C FLG(?-?):Keratintonofilament:DesmosomeSPRR2D Early cornifiedenvelopeCell surface PKP4 CSTA Late envelopeproteinsCell surfaceDSC3 SPRR2G CDSN LCE2D MembraneprofilaggrinprocessingproteasesSPINK6 LIPJ LOR LCE3D PCSK6 PI3(61-117) LCE3B IVL CASP14(6-146) KLK12LCE1D Keratin tetramerLCE1A KLK5:SPINK6CHOL CSTA CHOL Cornifiedenvelope:CDSNTCHH DSC3 Keratintonofilament:DesmosomeLCE2C FLG-Keratin filament network TCHH LELP1 CHOL CDSN PKP1 fatty acid LCE1C PKP3 DSP SPRR1A H2OLOR TGM1 TGM1 H+CELA2A propeptideLOR IVL SPINK9 Lamellar bodyKLK5EVPL LCE2B LCE3D LCE3B fatty acid DesmosomeH2OSPRR1A CSTA RPTN PERP SPRR2D KLK14Keratin type I, hair specific LCE3A IVL:TGM1:CellsurfacePI3(61-117) DSG1 SPRR2E LCE1E SPRR3 SPRR2G fatty acid SPRR1B KRTAP2s TCHH LCE4A PPL TGM1 LOR LCE2D SPRR2E Minor CE proteinsDSG2 Keratin type II, epithelial CHOL PPL KLK13:SPINK6KLK14 IVL DSG1 TCHH SPRR2F ST14EVPL ceramide ceramide IVL LCE2D KRTAP21s SPRR1B Keratin type ISPRR2D EVPL Keratin tonofilamentSPRR3 Keratin type I, epithelial DSC1 Keratin type II, hair follicle specific SPINK6SPRR2D CSTA SPRR2A KAZN:PPLEVPL keratinocyte LIPsPKP2 LCE2D LCE1D KRTAP16-1 Keratin type I, hair follicle specific CSTA SPRR1A Keratin tonofilament DSP fatty acid LCE5A LCE5A LCE1F DSG4 SPRR3 Keratin type I, epithelial SPRR2E SPRsFLG-Keratin filamentnetworkPRSS8(33-44) IVL KLK5:SPINK9KRTAP10s ceramide KRTAP1s KRTAP4s LCE4A Keratin type II, epithelial LELP1 DSC2 RPTN Keratin type II, hair follicle specific PI3(61-117) KRTAP9s CASP14FLG(?-?) KLK12 Keratintonofilament:Keratin associated proteins polymerDSG1 LCE1B Cell surface KLK5 RPTN Cell surface LCE1B KRTAP19s KRTAP29-1 CAPN1 SPRR2F LCE4A TGM1 SPRR2D LCE3E KRTAP20s KRTAP25-1 LCE3E PKP3 SPRR2F SPRR2G LCE2A EVPL:PPL:CellsurfaceSPRR2G FLG-Keratin filament network fatty acid Cell surface SPRR2B PKP2 PPLSPRR2G KLK13CELA2A LCE1C LCE5A RPTN LCE2A TGM1Keratin tonofilament FLG(1-4061)TCHH DSP LCE1D Keratin type I, hair specific TCHH PI3(61-117) LELP1 SPRR2B LCE1C LCE2B EVPL LCE2A Keratin type II, epithelial LCE6A KLK5 Keratin unit lengthfilamentKeratin type I, epithelial PPL LCE3C IVLKeratin type I, epithelial LCE3D Mature cornifiedenvelopeKeratin type II, hair follicle specific SPRR2G ST14 SPRR2B LCE3C Cell surface PPL SPINK9JUP CASP14(6-146) EVPL:PPLKeratin tonofilament:Desmosome network LCE1E KRTAP5s CSTA IVL ceramide FLG-Keratin filament network LCE3A PKP4 Mature cornifiedenvelopeEVPL:PPL:IVL:TGM1:Cell surfaceSPINK6 Keratin type II, epithelial Cell surface PPL SPRR2E LCE3B PI3(61-117) PI3(61-117) DSC2 Keratin filamentunitsSPRR2F SPRR2D LCE1C Keratin type I, hair follicle specific EVPLKeratin type IIPKP1 DSC2 KLK5SPRR3 ceramide Keratin type II, epithelial Ca2+ 44462336116, 374518, 559, 134545514518, 552344457, 569, 1336517, 5618, 5544466, 3745486, 3723456, 376, 37456, 375


Keratins are the major structural protein of vertebrate epidermis, constituting up to 85% of a fully differentiated keratinocyte (Fuchs 1995). Keratins belong to a superfamily of intermediate filament (IF) proteins that form alpha-helical coiled-coil dimers, which associate laterally and end-to-end to form approximately 10 nm diameter filaments. Keratin filaments are heteropolymeric, formed from equal amounts of acidic type I and basic /neutral type 2 keratins. Humans have 54 keratin genes (Schweitzer et al. 2006). They have highly specific expression patterns, related to the epithelial type and stage of differentiation. Roughly half of human keratins are specific to hair follicles (Langbein & Schweizer 2005). Keratin filaments bundle into tonofilaments that span the cytoplasm and bind to desmosomes and other cell membrane structures (Waschke 2008). This reflects their primary function, maintaining the mechanical stability of individual cells and epithelial tissues (Moll et al. 2008). View original pathway at:Reactome.


Pathway is converted from Reactome ID: 6805567
Reactome version: 66
Reactome Author 
Reactome Author: Jupe, Steve

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101521view11:38, 1 November 2018ReactomeTeamreactome version 66
101057view21:20, 31 October 2018ReactomeTeamreactome version 65
100588view19:54, 31 October 2018ReactomeTeamreactome version 64
100137view16:40, 31 October 2018ReactomeTeamreactome version 63
99687view15:09, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99277view12:45, 31 October 2018ReactomeTeamreactome version 62
93483view11:24, 9 August 2017ReactomeTeamNew pathway

External references


View all...
NameTypeDatabase referenceComment
CAPN1 ProteinP07384 (Uniprot-TrEMBL)
CAPNS1 ProteinP04632 (Uniprot-TrEMBL)
CASP14(153-242) ProteinP31944 (Uniprot-TrEMBL)
CASP14(6-146) ProteinP31944 (Uniprot-TrEMBL)
CASP14ComplexR-HSA-8847680 (Reactome)
CDSN ProteinQ15517 (Uniprot-TrEMBL)
CDSNProteinQ15517 (Uniprot-TrEMBL)
CELA2A ProteinP08217 (Uniprot-TrEMBL)
CELA2A propeptideProteinP08217 (Uniprot-TrEMBL)
CELA2AProteinP08217 (Uniprot-TrEMBL)
CHOL MetaboliteCHEBI:16113 (ChEBI)
CSTA ProteinP01040 (Uniprot-TrEMBL)
Ca2+ MetaboliteCHEBI:29108 (ChEBI)
Cell surface R-ALL-983438 (Reactome) This entity is intended to represent any molecule that might be at the outer cell surface of any cell, host or microbial.
Cell surfaceR-ALL-983438 (Reactome) This entity is intended to represent any molecule that might be at the outer cell surface of any cell, host or microbial.
Cornified envelope:CDSNComplexR-HSA-6814723 (Reactome)
Cornified envelopeComplexR-HSA-6814278 (Reactome)

profilaggrin processing

ComplexR-HSA-8934797 (Reactome)
DAGsMetaboliteCHEBI:18035 (ChEBI)
DSC1 ProteinQ08554 (Uniprot-TrEMBL)
DSC2 ProteinQ02487 (Uniprot-TrEMBL)
DSC3 ProteinQ14574 (Uniprot-TrEMBL)
DSG1 ProteinQ02413 (Uniprot-TrEMBL)
DSG2 ProteinQ14126 (Uniprot-TrEMBL)
DSG3 ProteinP32926 (Uniprot-TrEMBL)
DSG4 ProteinQ86SJ6 (Uniprot-TrEMBL)
DSP ProteinP15924 (Uniprot-TrEMBL)
Desmosome:PERPComplexR-HSA-8848046 (Reactome)
DesmosomeComplexR-HSA-6814388 (Reactome)
EVPL ProteinQ92817 (Uniprot-TrEMBL)
EVPL:PPL:Cell surfaceComplexR-HSA-6814188 (Reactome)
EVPL:PPL:IVL:TGM1:Cell surfaceComplexR-HSA-6814177 (Reactome)
EVPL:PPLComplexR-HSA-6814178 (Reactome)
EVPLProteinQ92817 (Uniprot-TrEMBL)
Early cornified envelopeComplexR-HSA-6814285 (Reactome)
FLG(1-4061)ProteinP20930 (Uniprot-TrEMBL)
FLG(?-?) ProteinP20930 (Uniprot-TrEMBL)
FLG(?-?):Keratin tonofilament:DesmosomeComplexR-HSA-8942225 (Reactome)
FLG(?-?)ProteinP20930 (Uniprot-TrEMBL)
FLG-Keratin filament networkR-HSA-6814323 (Reactome)
FLG-Keratin filament network R-HSA-6814323 (Reactome)
FURIN ProteinP09958 (Uniprot-TrEMBL)
H+MetaboliteCHEBI:15378 (ChEBI)
H2OMetaboliteCHEBI:15377 (ChEBI)
IVL ProteinP07476 (Uniprot-TrEMBL)
IVL:TGM1:Cell surfaceComplexR-HSA-6814746 (Reactome)
IVLProteinP07476 (Uniprot-TrEMBL)
JUP ProteinP14923 (Uniprot-TrEMBL)
KAZN ProteinQ674X7 (Uniprot-TrEMBL)
KAZN:PPLComplexR-HSA-6814328 (Reactome)
KAZNProteinQ674X7 (Uniprot-TrEMBL)
KLK12 ProteinQ9UKR0 (Uniprot-TrEMBL)
KLK12:SPINK6ComplexR-HSA-8850791 (Reactome)
KLK12ProteinQ9UKR0 (Uniprot-TrEMBL)
KLK13 ProteinQ9UKR3 (Uniprot-TrEMBL)
KLK13:SPINK6ComplexR-HSA-8850785 (Reactome)
KLK13ProteinQ9UKR3 (Uniprot-TrEMBL)
KLK14 ProteinQ9P0G3 (Uniprot-TrEMBL)
KLK14:SPINK6ComplexR-HSA-8850790 (Reactome)