KEGG   PATHWAY: hsa04382
Entry
hsa04382                    Pathway                                
Name
Cornified envelope formation - Homo sapiens (human)
Class
Organismal Systems; Development and regeneration
Pathway map
hsa04382  Cornified envelope formation
hsa04382

Network
nt06545  Cornified envelope formation
  Element
N01922  Localization of envoplakin and periplakin to the plasma membrane (PM)
N01923  Closslinking of envoplakin, periplakin and involucrin
N01924  Loricrin oligomerization with small proline-rich proteins and further stabilization
N01925  Ultra-long-chain omega-hydroxy fatty acyl-CoA synthesis
N01926  Sphinganine synthesis
N01927  Acylglucosylceramide synthesis
N01928  Transport of lipids into lamellar body (LB)
N01929  Peroxidation and deglycosylation of acylglucosylceramide
N01930  Processing of profilaggrin to filaggrin and binding to keratin filaments
N01931  Processing of filaggrin to amino acids
N01932  Histidine conversion to urocanic acid
Organism
Homo sapiens (human) [GN:hsa]
Gene
100132463  CLDN24; claudin 24 [KO:K06087]
100288814  CLDN34; claudin 34 [KO:K06087]
100506658  OCLN; occludin [KO:K06088]
100653049  [KO:K07604]
1041  CDSN; corneodesmosin [KO:K23457]
10558  SPTLC1; serine palmitoyltransferase long chain base subunit 1 [KO:K00654] [EC:2.3.1.50]
10686  CLDN16; claudin 16 [KO:K06087]
10999  SLC27A4; solute carrier family 27 member 4 [KO:K08745] [EC:6.2.1.-]
110806278  SPRR5; small proline rich protein 5 [KO:K28331]
11240  PADI2; peptidyl arginine deiminase 2 [KO:K01481] [EC:3.5.3.15]
112802  KRT71; keratin 71 [KO:K07605]
121214  SDR9C7; short chain dehydrogenase/reductase family 9C member 7 [KO:K24425]
121391  KRT74; keratin 74 [KO:K07605]
122526777  LCE7A; late cornified envelope 7A [KO:K28332]
124905679  [KO:K23757]
125115  KRT40; keratin 40 [KO:K07604]
126410  CYP4F22; cytochrome P450 family 4 subfamily F member 22 [KO:K17731] [EC:1.14.14.177]
126637  TCHHL1; trichohyalin like 1 [KO:K23769]
1282  COL4A1; collagen type IV alpha 1 chain [KO:K06237]
1284  COL4A2; collagen type IV alpha 2 chain [KO:K06237]
1285  COL4A3; collagen type IV alpha 3 chain [KO:K06237]
1286  COL4A4; collagen type IV alpha 4 chain [KO:K06237]
1287  COL4A5; collagen type IV alpha 5 chain [KO:K06237]
1288  COL4A6; collagen type IV alpha 6 chain [KO:K06237]
1294  COL7A1; collagen type VII alpha 1 chain [KO:K16628]
1308  COL17A1; collagen type XVII alpha 1 chain [KO:K07603]
1364  CLDN4; claudin 4 [KO:K06087]
1365  CLDN3; claudin 3 [KO:K06087]
1366  CLDN7; claudin 7 [KO:K06087]
137075  CLDN23; claudin 23 [KO:K06087]
140576  S100A16; S100 calcium binding protein A16 [KO:K23768]
140807  KRT72; keratin 72 [KO:K07605]
144501  KRT80; keratin 80 [KO:K07605]
147183  KRT25; keratin 25 [KO:K07604]
147409  DSG4; desmoglein 4 [KO:K07599]
149461  CLDN19; claudin 19 [KO:K06087]
151516  ASPRV1; aspartic peptidase retroviral like 1 [KO:K24552]
162605  KRT28; keratin 28 [KO:K07604]
163778  SPRR4; small proline rich protein 4 [KO:K28331]
1823  DSC1; desmocollin 1 [KO:K07600]
1828  DSG1; desmoglein 1 [KO:K07596]
1829  DSG2; desmoglein 2 [KO:K07597]
1830  DSG3; desmoglein 3 [KO:K07598]
1832  DSP; desmoplakin [KO:K10381]
192666  KRT24; keratin 24 [KO:K07604]
196374  KRT78; keratin 78 [KO:K07605]
199834  LCE4A; late cornified envelope 4A [KO:K28332]
204219  CERS3; ceramide synthase 3 [KO:K24622]
2053  EPHX2; epoxide hydrolase 2 [KO:K08726] [EC:3.3.2.10 3.1.3.76]
2125  EVPL; envoplakin [KO:K10383]
2312  FLG; filaggrin [KO:K10384]
23254  KAZN; kazrin, periplakin interacting protein [KO:K28344]
23562  CLDN14; claudin 14 [KO:K06087]
23581  CASP14; caspase 14 [KO:K04401] [EC:3.4.22.-]
24146  CLDN15; claudin 15 [KO:K06087]
242  ALOX12B; arachidonate 12-lipoxygenase, 12R type [KO:K08021] [EC:1.13.11.-]
2531  KDSR; 3-ketodihydrosphingosine reductase [KO:K04708] [EC:1.1.1.102]
254910  LCE5A; late cornified envelope 5A [KO:K28332]
25984  KRT23; keratin 23 [KO:K07604]
26154  ABCA12; ATP binding cassette subfamily A member 12 [KO:K05646]
26239  LCE2B; late cornified envelope 2B [KO:K28332]
26285  CLDN17; claudin 17 [KO:K06087]
2629  GBA1; glucosylceramidase beta 1 [KO:K01201] [EC:3.2.1.45]
28227  PPP2R3B; protein phosphatase 2 regulatory subunit B''beta [KO:K11583]
285848  PNPLA1; patatin like domain 1, omega-hydroxyceramide transacylase [KO:K16813] [EC:2.3.1.296]
286887  KRT6C; keratin 6C [KO:K07605]
29943  PADI1; peptidyl arginine deiminase 1 [KO:K01481] [EC:3.5.3.15]
3034  HAL; histidine ammonia-lyase [KO:K01745] [EC:4.3.1.3]
319101  KRT73; keratin 73 [KO:K07605]
338324  S100A7A; S100 calcium binding protein A7A [KO:K21126]
338785  KRT79; keratin 79 [KO:K07605]
342574  KRT27; keratin 27 [KO:K07604]
353131  LCE1A; late cornified envelope 1A [KO:K28332]
353132  LCE1B; late cornified envelope 1B [KO:K28332]
353133  LCE1C; late cornified envelope 1C [KO:K28332]
353134  LCE1D; late cornified envelope 1D [KO:K28332]
353135  LCE1E; late cornified envelope 1E [KO:K28332]
353137  LCE1F; late cornified envelope 1F [KO:K28332]
353139  LCE2A; late cornified envelope 2A [KO:K28332]
353140  LCE2C; late cornified envelope 2C [KO:K28332]
353141  LCE2D; late cornified envelope 2D [KO:K28332]
353142  LCE3A; late cornified envelope 3A [KO:K28332]
353143  LCE3B; late cornified envelope 3B [KO:K28332]
353144  LCE3C; late cornified envelope 3C [KO:K28332]
353145  LCE3E; late cornified envelope 3E [KO:K28332]
353238  PADI6; peptidyl arginine deiminase 6 [KO:K01481] [EC:3.5.3.15]
353288  KRT26; keratin 26 [KO:K07604]
3655  ITGA6; integrin subunit alpha 6 [KO:K06485]
3691  ITGB4; integrin subunit beta 4 [KO:K06525]
3713  IVL; involucrin [KO:K28330]
3728  JUP; junction plakoglobin [KO:K10056]
374454  KRT77; keratin 77 [KO:K07605]
376497  SLC27A1; solute carrier family 27 member 1 [KO:K08745] [EC:6.2.1.-]
3848  KRT1; keratin 1 [KO:K07605]
3849  KRT2; keratin 2 [KO:K07605]
3850  KRT3; keratin 3 [KO:K07605]
3851  KRT4; keratin 4 [KO:K07605]
3852  KRT5; keratin 5 [KO:K07605]
3853  KRT6A; keratin 6A [KO:K07605]
3854  KRT6B; keratin 6B [KO:K07605]
3855  KRT7; keratin 7 [KO:K07605]
3856  KRT8; keratin 8 [KO:K07605]
3857  KRT9; keratin 9 [KO:K07604]
3858  KRT10; keratin 10 [KO:K07604]
3859  KRT12; keratin 12 [KO:K07604]
3860  KRT13; keratin 13 [KO:K07604]
3861  KRT14; keratin 14 [KO:K07604]
3866  KRT15; keratin 15 [KO:K07604]
3868  KRT16; keratin 16 [KO:K07604]
3872  KRT17; keratin 17 [KO:K07604]
3875  KRT18; keratin 18 [KO:K07604]
3880  KRT19; keratin 19 [KO:K07604]
3881  KRT31; keratin 31 [KO:K07604]
3882  KRT32; keratin 32 [KO:K07604]
3883  KRT33A; keratin 33A [KO:K07604]
3884  KRT33B; keratin 33B [KO:K07604]
3885  KRT34; keratin 34 [KO:K07604]
3886  KRT35; keratin 35 [KO:K07604]
388697  HRNR; hornerin [KO:K23757]
388698  FLG2; filaggrin 2 [KO:K10384]
3887  KRT81; keratin 81 [KO:K07605]
3888  KRT82; keratin 82 [KO:K07605]
3889  KRT83; keratin 83 [KO:K07605]
3890  KRT84; keratin 84 [KO:K07605]
3891  KRT85; keratin 85 [KO:K07605]
3892  KRT86; keratin 86 [KO:K07605]
390792  KRT39; keratin 39 [KO:K07604]
3909  LAMA3; laminin subunit alpha 3 [KO:K06240]
3911  LAMA5; laminin subunit alpha 5 [KO:K06240]
3914  LAMB3; laminin subunit beta 3 [KO:K06244]
3918  LAMC2; laminin subunit gamma 2 [KO:K06246]
4014  LORICRIN; loricrin cornified envelope precursor protein [KO:K10385]
49861  CLDN20; claudin 20 [KO:K06087]
5010  CLDN11; claudin 11 [KO:K06087]
5045  FURIN; furin, paired basic amino acid cleaving enzyme [KO:K01349] [EC:3.4.21.75]
5046  PCSK6; proprotein convertase subtilisin/kexin type 6 [KO:K08672] [EC:3.4.21.-]
51099  ABHD5; abhydrolase domain containing 5, lysophosphatidic acid acyltransferase [KO:K13699] [EC:2.3.1.51]
51208  CLDN18; claudin 18 [KO:K06087]
51350  KRT76; keratin 76 [KO:K07605]
51702  PADI3; peptidyl arginine deiminase 3 [KO:K01481] [EC:3.5.3.15]
5317  PKP1; plakophilin 1 [KO:K10387]
5318  PKP2; plakophilin 2 [KO:K12642]
5339  PLEC; plectin [KO:K10388]
53842  CLDN22; claudin 22 [KO:K06087]
54474  KRT20; keratin 20 [KO:K07604]
5493  PPL; periplakin [KO:K10386]
55012  PPP2R3C; protein phosphatase 2 regulatory subunit B''gamma [KO:K11583]
5515  PPP2CA; protein phosphatase 2 catalytic subunit alpha [KO:K04382] [EC:3.1.3.16]
5516  PPP2CB; protein phosphatase 2 catalytic subunit beta [KO:K04382] [EC:3.1.3.16]
5518  PPP2R1A; protein phosphatase 2 scaffold subunit Aalpha [KO:K03456]
5519  PPP2R1B; protein phosphatase 2 scaffold subunit Abeta [KO:K03456]
5520  PPP2R2A; protein phosphatase 2 regulatory subunit Balpha [KO:K04354]
5521  PPP2R2B; protein phosphatase 2 regulatory subunit Bbeta [KO:K04354]
5522  PPP2R2C; protein phosphatase 2 regulatory subunit Bgamma [KO:K04354]
5523  PPP2R3A; protein phosphatase 2 regulatory subunit B''alpha [KO:K11583]
5525  PPP2R5A; protein phosphatase 2 regulatory subunit B'alpha [KO:K11584]
5526  PPP2R5B; protein phosphatase 2 regulatory subunit B'beta [KO:K11584]
5527  PPP2R5C; protein phosphatase 2 regulatory subunit B'gamma [KO:K11584]
5528  PPP2R5D; protein phosphatase 2 regulatory subunit B'delta [KO:K11584]
5529  PPP2R5E; protein phosphatase 2 regulatory subunit B'epsilon [KO:K11584]
55304  SPTLC3; serine palmitoyltransferase long chain base subunit 3 [KO:K00654] [EC:2.3.1.50]
55844  PPP2R2D; protein phosphatase 2 regulatory subunit Bdelta [KO:K04354]
5652  PRSS8; serine protease 8 [KO:K08664] [EC:3.4.21.-]
57402  S100A14; S100 calcium binding protein A14 [KO:K23767]
57704  GBA2; glucosylceramidase beta 2 [KO:K17108] [EC:3.2.1.45]
59344  ALOXE3; arachidonate epidermal lipoxygenase 3 [KO:K18684] [EC:4.2.1.152 5.4.4.7]
6271  S100A1; S100 calcium binding protein A1 [KO:K23758]
6273  S100A2; S100 calcium binding protein A2 [KO:K23759]
6274  S100A3; S100 calcium binding protein A3 [KO:K23760]
6275  S100A4; S100 calcium binding protein A4 [KO:K23761]
6276  S100A5; S100 calcium binding protein A5 [KO:K23762]
6277  S100A6; S100 calcium binding protein A6 [KO:K23764]
6278  S100A7; S100 calcium binding protein A7 [KO:K21126]
6279  S100A8; S100 calcium binding protein A8 [KO:K21127]
6280  S100A9; S100 calcium binding protein A9 [KO:K21128]
6281  S100A10; S100 calcium binding protein A10 [KO:K17274]
6282  S100A11; S100 calcium binding protein A11 [KO:K23765]
6283  S100A12; S100 calcium binding protein A12 [KO:K23766]
6284  S100A13; S100 calcium binding protein A13 [KO:K23765]
642  BLMH; bleomycin hydrolase [KO:K01372] [EC:3.4.22.40]
644672  CLDN25; claudin 25 [KO:K06087]
64834  ELOVL1; ELOVL fatty acid elongase 1 [KO:K10247] [EC:2.3.1.199]
667  DST; dystonin [KO:K10382]
6698  SPRR1A; small proline rich protein 1A [KO:K28331]
6699  SPRR1B; small proline rich protein 1B [KO:K28331]
6700  SPRR2A; small proline rich protein 2A [KO:K28331]
6701  SPRR2B; small proline rich protein 2B [KO:K28331]
6703  SPRR2D; small proline rich protein 2D [KO:K28331]
6704  SPRR2E; small proline rich protein 2E [KO:K28331]
6705  SPRR2F; small proline rich protein 2F [KO:K28331]
6706  SPRR2G; small proline rich protein 2G [KO:K28331]
6707  SPRR3; small proline rich protein 3 [KO:K28331]
6768  ST14; ST14 transmembrane serine protease matriptase [KO:K08670] [EC:3.4.21.109]
6785  ELOVL4; ELOVL fatty acid elongase 4 [KO:K10249] [EC:2.3.1.199]
7051  TGM1; transglutaminase 1 [KO:K05619] [EC:2.3.2.13]
7053  TGM3; transglutaminase 3 [KO:K05620] [EC:2.3.2.13]
7062  TCHH; trichohyalin [KO:K18626]
7082  TJP1; tight junction protein 1 [KO:K05701]
7122  CLDN5; claudin 5 [KO:K06087]
7357  UGCG; UDP-glucose ceramide glucosyltransferase [KO:K00720] [EC:2.4.1.80]
79852  EPHX3; epoxide hydrolase 3 [KO:K22368] [EC:3.3.2.10]
823  CAPN1; calpain 1 [KO:K01367] [EC:3.4.22.52]
84283  TMEM79; transmembrane protein 79 [KO:K28333]
84648  LCE3D; late cornified envelope 3D [KO:K28332]
8687  KRT38; keratin 38 [KO:K07604]
8688  KRT37; keratin 37 [KO:K07604]
8689  KRT36; keratin 36 [KO:K07604]
9069  CLDN12; claudin 12 [KO:K06087]
9071  CLDN10; claudin 10 [KO:K06087]
9073  CLDN8; claudin 8 [KO:K06087]
9074  CLDN6; claudin 6 [KO:K06087]
9075  CLDN2; claudin 2 [KO:K06087]
9076  CLDN1; claudin 1 [KO:K06087]
9080  CLDN9; claudin 9 [KO:K06087]
9119  KRT75; keratin 75 [KO:K07605]
9333  TGM5; transglutaminase 5 [KO:K05622] [EC:2.3.2.13]
9517  SPTLC2; serine palmitoyltransferase long chain base subunit 2 [KO:K00654] [EC:2.3.1.50]
Compound
C00064  L-Glutamine
C00076  Calcium cation
C00135  L-Histidine
C00154  Palmitoyl-CoA
C00162  Fatty acid
C00187  Cholesterol
C00785  Urocanate
C00836  Sphinganine
C01879  5-Oxoproline
C02934  3-Dehydrosphinganine
C22352  Ultra-long-chain fatty acid
C22353  Ultra-long-chain omega-hydroxy fatty acid
C23027  Ultra-long-chain omega-hydroxy fatty acyl-CoA
C23028  Ultra-long-chain omega-hydroxyceramide
C23029  linoleate-esterified acylceramide
C23030  Acylglucosylceramide
Reference
PMID:15803139
  Authors
Candi E, Schmidt R, Melino G
  Title
The cornified envelope: a model of cell death in the skin.
  Journal
Nat Rev Mol Cell Biol 6:328-40 (2005)
DOI:10.1038/nrm1619
Reference
PMID:29153780
  Authors
Egawa G, Kabashima K
  Title
Barrier dysfunction in the skin allergy.
  Journal
Allergol Int 67:3-11 (2018)
DOI:10.1016/j.alit.2017.10.002
Reference
PMID:32218335
  Authors
Ishitsuka Y, Roop DR
  Title
Loricrin: Past, Present, and Future.
  Journal
Int J Mol Sci 21:ijms21072271 (2020)
DOI:10.3390/ijms21072271
Reference
PMID:34639001
  Authors
Gallegos-Alcala P, Jimenez M, Cervantes-Garcia D, Salinas E
  Title
The Keratinocyte as a Crucial Cell in the Predisposition, Onset, Progression, Therapy and Study of the Atopic Dermatitis.
  Journal
Int J Mol Sci 22:ijms221910661 (2021)
DOI:10.3390/ijms221910661
Reference
PMID:30776025
  Authors
Goleva E, Berdyshev E, Leung DY
  Title
Epithelial barrier repair and prevention of allergy.
  Journal
J Clin Invest 129:1463-1474 (2019)
DOI:10.1172/JCI124608
Reference
PMID:23954553
  Authors
Breiden B, Sandhoff K
  Title
The role of sphingolipid metabolism in cutaneous permeability barrier formation.
  Journal
Biochim Biophys Acta 1841:441-52 (2014)
DOI:10.1016/j.bbalip.2013.08.010
Reference
PMID:31974308
  Authors
Yamamoto H, Hattori M, Chamulitrat W, Ohno Y, Kihara A
  Title
Skin permeability barrier formation by the ichthyosis-causative gene FATP4 through formation of the barrier lipid omega-O-acylceramide.
  Journal
Proc Natl Acad Sci U S A 117:2914-2922 (2020)
DOI:10.1073/pnas.1917525117
Reference
PMID:36902463
  Authors
Ge F, Sun K, Hu Z, Dong X
  Title
Role of Omega-Hydroxy Ceramides in Epidermis: Biosynthesis, Barrier Integrity and Analyzing Method.
  Journal
Int J Mol Sci 24:ijms24055035 (2023)
DOI:10.3390/ijms24055035
Related
pathway
hsa00600  Sphingolipid metabolism
KO pathway
ko04382   

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