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 isoform a [KO:K06088]
100653049  [KO:K07604]
1041  CDSN; corneodesmosin precursor [KO:K23457]
10558  SPTLC1; serine palmitoyltransferase 1 isoform a [KO:K00654] [EC:2.3.1.50]
10686  CLDN16; claudin-16 [KO:K06087]
10999  SLC27A4; long-chain fatty acid transport protein 4 [KO:K08745] [EC:6.2.1.-]
110806278  SPRR5; small proline-rich protein 5 [KO:K28331]
11187  PKP3; plakophilin-3 isoform PKP3a [KO:K28841]
11240  PADI2; protein-arginine deiminase type-2 [KO:K01481] [EC:3.5.3.15]
112802  KRT71; keratin, type II cytoskeletal 71 [KO:K07605]
121214  SDR9C7; short-chain dehydrogenase/reductase family 9C member 7 [KO:K24425]
121391  KRT74; keratin, type II cytoskeletal 74 [KO:K07605]
122526777  LCE7A; late cornified envelope protein 7A [KO:K28332]
125115  KRT40; keratin, type I cytoskeletal 40 [KO:K07604]
126410  CYP4F22; ultra-long-chain fatty acid omega-hydroxylase [KO:K17731] [EC:1.14.14.177]
126637  TCHHL1; trichohyalin-like protein 1 [KO:K23769]
1282  COL4A1; collagen alpha-1(IV) chain isoform 1 preproprotein [KO:K06237]
1284  COL4A2; collagen alpha-2(IV) chain preproprotein [KO:K06237]
1285  COL4A3; collagen alpha-3(IV) chain precursor [KO:K06237]
1286  COL4A4; collagen alpha-4(IV) chain precursor [KO:K06237]
1287  COL4A5; collagen alpha-5(IV) chain isoform 1 precursor [KO:K06237]
1288  COL4A6; collagen alpha-6(IV) chain isoform A precursor [KO:K06237]
1294  COL7A1; collagen alpha-1(VII) chain precursor [KO:K16628]
1308  COL17A1; collagen alpha-1(XVII) chain [KO:K07603]
1364  CLDN4; claudin-4 [KO:K06087]
1365  CLDN3; claudin-3 [KO:K06087]
1366  CLDN7; claudin-7 isoform 1 [KO:K06087]
137075  CLDN23; claudin-23 [KO:K06087]
140576  S100A16; protein S100-A16 [KO:K23768]
140807  KRT72; keratin, type II cytoskeletal 72 isoform 1 [KO:K07605]
144501  KRT80; keratin, type II cytoskeletal 80 isoform K80 [KO:K07605]
147183  KRT25; keratin, type I cytoskeletal 25 [KO:K07604]
147409  DSG4; desmoglein-4 isoform 2 preproprotein [KO:K07599]
149461  CLDN19; claudin-19 isoform a [KO:K06087]
151516  ASPRV1; retroviral-like aspartic protease 1 [KO:K24552]
162605  KRT28; keratin, type I cytoskeletal 28 [KO:K07604]
163778  SPRR4; small proline-rich protein 4 [KO:K28331]
1823  DSC1; desmocollin-1 isoform Dsc1a preproprotein [KO:K07600]
1828  DSG1; desmoglein-1 preproprotein [KO:K07596]
1829  DSG2; desmoglein-2 preproprotein [KO:K07597]
1830  DSG3; desmoglein-3 preproprotein [KO:K07598]
1832  DSP; desmoplakin isoform I [KO:K10381]
192666  KRT24; keratin, type I cytoskeletal 24 [KO:K07604]
196374  KRT78; keratin, type II cytoskeletal 78 isoform 1 [KO:K07605]
199834  LCE4A; late cornified envelope protein 4A [KO:K28332]
204219  CERS3; ceramide synthase 3 isoform 2 [KO:K24622]
2053  EPHX2; bifunctional epoxide hydrolase 2 isoform 1 [KO:K08726] [EC:3.3.2.10 3.1.3.76]
2125  EVPL; envoplakin isoform 2 [KO:K10383]
2312  FLG; filaggrin [KO:K10384]
23254  KAZN; kazrin isoform E [KO:K28344]
23562  CLDN14; claudin-14 [KO:K06087]
23581  CASP14; caspase-14 precursor [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 protein 5A [KO:K28332]
25984  KRT23; keratin, type I cytoskeletal 23 isoform 1 [KO:K07604]
26154  ABCA12; glucosylceramide transporter ABCA12 isoform a [KO:K05646]
26239  LCE2B; late cornified envelope protein 2B [KO:K28332]
26285  CLDN17; claudin-17 [KO:K06087]
2629  GBA1; lysosomal acid glucosylceramidase isoform 1 precursor [KO:K01201] [EC:3.2.1.45]
28227  PPP2R3B; serine/threonine-protein phosphatase 2A regulatory subunit B'' subunit beta [KO:K11583]
285848  PNPLA1; omega-hydroxyceramide transacylase isoform 3 [KO:K16813] [EC:2.3.1.296]
286887  KRT6C; keratin, type II cytoskeletal 6C [KO:K07605]
29943  PADI1; protein-arginine deiminase type-1 [KO:K01481] [EC:3.5.3.15]
3034  HAL; histidine ammonia-lyase isoform 1 [KO:K01745] [EC:4.3.1.3]
319101  KRT73; keratin, type II cytoskeletal 73 [KO:K07605]
338324  S100A7A; protein S100-A7A [KO:K21126]
338785  KRT79; keratin, type II cytoskeletal 79 [KO:K07605]
342574  KRT27; keratin, type I cytoskeletal 27 [KO:K07604]
353131  LCE1A; late cornified envelope protein 1A [KO:K28332]
353132  LCE1B; late cornified envelope protein 1B [KO:K28332]
353133  LCE1C; late cornified envelope protein 1C isoform 1 [KO:K28332]
353134  LCE1D; late cornified envelope protein 1D [KO:K28332]
353135  LCE1E; late cornified envelope protein 1E [KO:K28332]
353137  LCE1F; late cornified envelope protein 1F [KO:K28332]
353139  LCE2A; late cornified envelope protein 2A [KO:K28332]
353140  LCE2C; late cornified envelope protein 2C [KO:K28332]
353141  LCE2D; late cornified envelope protein 2D [KO:K28332]
353142  LCE3A; late cornified envelope protein 3A [KO:K28332]
353143  LCE3B; late cornified envelope protein 3B [KO:K28332]
353144  LCE3C; late cornified envelope protein 3C [KO:K28332]
353145  LCE3E; late cornified envelope protein 3E [KO:K28332]
353238  PADI6; inactive protein-arginine deiminase type-6 [KO:K01481] [EC:3.5.3.15]
353288  KRT26; keratin, type I cytoskeletal 26 [KO:K07604]
3655  ITGA6; integrin alpha-6 isoform 6 precursor [KO:K06485]
3691  ITGB4; integrin beta-4 isoform 1 precursor [KO:K06525]
3713  IVL; involucrin [KO:K28330]
3728  JUP; junction plakoglobin [KO:K10056]
374454  KRT77; keratin, type II cytoskeletal 1b [KO:K07605]
376497  SLC27A1; long-chain fatty acid transport protein 1 [KO:K08745] [EC:6.2.1.-]
3848  KRT1; keratin, type II cytoskeletal 1 [KO:K07605]
3849  KRT2; keratin, type II cytoskeletal 2 epidermal [KO:K07605]
3850  KRT3; keratin, type II cytoskeletal 3 [KO:K07605]
3851  KRT4; keratin, type II cytoskeletal 4 [KO:K07605]
3852  KRT5; keratin, type II cytoskeletal 5 [KO:K07605]
3853  KRT6A; keratin, type II cytoskeletal 6A [KO:K07605]
3854  KRT6B; keratin, type II cytoskeletal 6B [KO:K07605]
3855  KRT7; keratin, type II cytoskeletal 7 [KO:K07605]
3856  KRT8; keratin, type II cytoskeletal 8 isoform 2 [KO:K07605]
3857  KRT9; keratin, type I cytoskeletal 9 [KO:K07604]
3858  KRT10; keratin, type I cytoskeletal 10 isoform 1 [KO:K07604]
3859  KRT12; keratin, type I cytoskeletal 12 [KO:K07604]
3860  KRT13; keratin, type I cytoskeletal 13 isoform a [KO:K07604]
3861  KRT14; keratin, type I cytoskeletal 14 [KO:K07604]
3866  KRT15; keratin, type I cytoskeletal 15 [KO:K07604]
3868  KRT16; keratin, type I cytoskeletal 16 [KO:K07604]
3872  KRT17; keratin, type I cytoskeletal 17 [KO:K07604]
3875  KRT18; keratin, type I cytoskeletal 18 [KO:K07604]
3880  KRT19; keratin, type I cytoskeletal 19 [KO:K07604]
3881  KRT31; keratin, type I cuticular Ha1 [KO:K07604]
3882  KRT32; keratin, type I cuticular Ha2 [KO:K07604]
3883  KRT33A; keratin, type I cuticular Ha3-I [KO:K07604]
3884  KRT33B; keratin, type I cuticular Ha3-II [KO:K07604]
3885  KRT34; keratin, type I cuticular Ha4 [KO:K07604]
3886  KRT35; keratin, type I cuticular Ha5 [KO:K07604]
388697  HRNR; hornerin [KO:K23757]
388698  FLG2; filaggrin-2 [KO:K10384]
3887  KRT81; keratin, type II cuticular Hb1 [KO:K07605]
3888  KRT82; keratin, type II cuticular Hb2 [KO:K07605]
3889  KRT83; keratin, type II cuticular Hb3 [KO:K07605]
3890  KRT84; keratin, type II cuticular Hb4 [KO:K07605]
3891  KRT85; keratin, type II cuticular Hb5 isoform 1 [KO:K07605]
3892  KRT86; keratin, type II cuticular Hb6 [KO:K07605]
390792  KRT39; keratin, type I cytoskeletal 39 [KO:K07604]
3909  LAMA3; laminin subunit alpha-3 isoform 1 precursor [KO:K06240]
3911  LAMA5; laminin subunit alpha-5 precursor [KO:K06240]
3914  LAMB3; laminin subunit beta-3 precursor [KO:K06244]
3918  LAMC2; laminin subunit gamma-2 isoform a precursor [KO:K06246]
4014  LORICRIN; loricrin [KO:K10385]
49861  CLDN20; claudin-20 precursor [KO:K06087]
5010  CLDN11; claudin-11 isoform 1 [KO:K06087]
5045  FURIN; furin isoform 1 preproprotein [KO:K01349] [EC:3.4.21.75]
5046  PCSK6; proprotein convertase subtilisin/kexin type 6 isoform PACE4-AI preproprotein [KO:K08672] [EC:3.4.21.-]
51099  ABHD5; 1-acylglycerol-3-phosphate O-acyltransferase ABHD5 isoform a [KO:K13699] [EC:2.3.1.51]
51208  CLDN18; claudin-18 isoform 1 [KO:K06087]
51350  KRT76; keratin, type II cytoskeletal 2 oral [KO:K07605]
51702  PADI3; protein-arginine deiminase type-3 [KO:K01481] [EC:3.5.3.15]
5317  PKP1; plakophilin-1 isoform 1b [KO:K10387]
5318  PKP2; plakophilin-2 isoform 2b [KO:K12642]
5339  PLEC; plectin isoform 1 [KO:K10388]
53842  CLDN22; claudin-22 [KO:K06087]
54474  KRT20; keratin, type I cytoskeletal 20 precursor [KO:K07604]
5493  PPL; periplakin [KO:K10386]
55012  PPP2R3C; serine/threonine-protein phosphatase 2A regulatory subunit B'' subunit gamma isoform 1 [KO:K11583]
5515  PPP2CA; serine/threonine-protein phosphatase 2A catalytic subunit alpha isoform isoform 1 [KO:K04382] [EC:3.1.3.16]
5516  PPP2CB; serine/threonine-protein phosphatase 2A catalytic subunit beta isoform [KO:K04382] [EC:3.1.3.16]
5518  PPP2R1A; serine/threonine-protein phosphatase 2A 65 kDa regulatory subunit A alpha isoform isoform 1 [KO:K03456]
5519  PPP2R1B; serine/threonine-protein phosphatase 2A 65 kDa regulatory subunit A beta isoform isoform a [KO:K03456]
5520  PPP2R2A; serine/threonine-protein phosphatase 2A 55 kDa regulatory subunit B alpha isoform isoform 1 [KO:K04354]
5521  PPP2R2B; serine/threonine-protein phosphatase 2A 55 kDa regulatory subunit B beta isoform isoform e [KO:K04354]
5522  PPP2R2C; protein phosphatase 2, regulatory subunit B, gamma isoform a [KO:K04354]
5523  PPP2R3A; serine/threonine-protein phosphatase 2A regulatory subunit B'' subunit alpha isoform 1 [KO:K11583]
5525  PPP2R5A; serine/threonine-protein phosphatase 2A 56 kDa regulatory subunit alpha isoform isoform 1 [KO:K11584]
5526  PPP2R5B; serine/threonine-protein phosphatase 2A 56 kDa regulatory subunit beta isoform [KO:K11584]
5527  PPP2R5C; serine/threonine-protein phosphatase 2A 56 kDa regulatory subunit gamma isoform isoform a [KO:K11584]
5528  PPP2R5D; serine/threonine-protein phosphatase 2A 56 kDa regulatory subunit delta isoform isoform 1 [KO:K11584]
5529  PPP2R5E; serine/threonine-protein phosphatase 2A 56 kDa regulatory subunit epsilon isoform isoform a [KO:K11584]
55304  SPTLC3; serine palmitoyltransferase 3 [KO:K00654] [EC:2.3.1.50]
55844  PPP2R2D; serine/threonine-protein phosphatase 2A 55 kDa regulatory subunit B delta isoform isoform a [KO:K04354]
5652  PRSS8; prostasin preproprotein [KO:K08664] [EC:3.4.21.-]
57402  S100A14; protein S100-A14 [KO:K23767]
57704  GBA2; non-lysosomal glucosylceramidase isoform 1 [KO:K17108] [EC:3.2.1.45]
59344  ALOXE3; hydroperoxide isomerase ALOXE3 isoform 2 [KO:K18684] [EC:4.2.1.152 5.4.4.7]
6271  S100A1; protein S100-A1 [KO:K23758]
6273  S100A2; protein S100-A2 isoform 1 [KO:K23759]
6274  S100A3; protein S100-A3 [KO:K23760]
6275  S100A4; protein S100-A4 [KO:K23761]
6276  S100A5; protein S100-A5 [KO:K23762]
6277  S100A6; protein S100-A6 [KO:K23764]
6278  S100A7; protein S100-A7 [KO:K21126]
6279  S100A8; protein S100-A8 isoform d [KO:K21127]
6280  S100A9; protein S100-A9 [KO:K21128]
6281  S100A10; protein S100-A10 [KO:K17274]
6282  S100A11; protein S100-A11 [KO:K23765]
6283  S100A12; protein S100-A12 [KO:K23766]
6284  S100A13; protein S100-A13 [KO:K23765]
642  BLMH; bleomycin hydrolase [KO:K01372] [EC:3.4.22.40]
644672  CLDN25; claudin-25 [KO:K06087]
64834  ELOVL1; very long chain fatty acid elongase 1 isoform 1 [KO:K10247] [EC:2.3.1.199]
667  DST; dystonin isoform 1e precursor [KO:K10382]
6698  SPRR1A; cornifin-A [KO:K28331]
6699  SPRR1B; cornifin-B [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; suppressor of tumorigenicity 14 protein [KO:K08670] [EC:3.4.21.109]
6785  ELOVL4; very long chain fatty acid elongase 4 [KO:K10249] [EC:2.3.1.199]
7051  TGM1; protein-glutamine gamma-glutamyltransferase K [KO:K05619] [EC:2.3.2.13]
7053  TGM3; protein-glutamine gamma-glutamyltransferase E [KO:K05620] [EC:2.3.2.13]
7062  TCHH; trichohyalin [KO:K18626]
7082  TJP1; tight junction protein 1 isoform a [KO:K05701]
7122  CLDN5; claudin-5 isoform 2 [KO:K06087]
7357  UGCG; 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 catalytic subunit [KO:K01367] [EC:3.4.22.52]
84283  TMEM79; transmembrane protein 79 [KO:K28333]
84648  LCE3D; late cornified envelope protein 3D [KO:K28332]
8687  KRT38; keratin, type I cuticular Ha8 [KO:K07604]
8688  KRT37; keratin, type I cuticular Ha7 [KO:K07604]
8689  KRT36; keratin, type I cuticular Ha6 [KO:K07604]
9069  CLDN12; claudin-12 [KO:K06087]
9071  CLDN10; claudin-10 isoform b [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, type II cytoskeletal 75 [KO:K07605]
9333  TGM5; protein-glutamine gamma-glutamyltransferase 5 isoform 1 [KO:K05622] [EC:2.3.2.13]
9517  SPTLC2; serine palmitoyltransferase 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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