KEGG   PATHWAY: hsa04530Help
Entry
hsa04530                    Pathway                                

Name
Tight junction - Homo sapiens (human)
Description
Epithelial tight junctions (TJs) are composed of at least three types of transmembrane protein -occludin, claudin and junctional adhesion molecules (JAMs)- and a cytoplasmic 'plaque' consisting of many different proteins that form large complexes. The transmembrane proteins mediate cell adhesion and are thought to constitute the intramembrane and paracellular diffusion barriers. The cytoplasmic 'plaque' contains three major multi-protein complexes consisting largely of scaffolding proteins, the ZO protein complex, the CRB3-Pals1-PATJ complex and the PAR-3-aPKC-PAR-6 complex. The ZO protein complex appears to organize the transmembrane proteins and couple them to other cytoplasmic proteins and to actin microfilaments. Two evolutionarily conserved protein complexes, the CRB3 and PAR complexes are involved in the establishment and maintenance of epithelial cell polarity. Besides these three protein complexes which seem to be constitutively associated at TJs, a number of proteins with different functions has been identified at TJs. These include additional scaffolding proteins like MUPP1 and MAGI-1, adaptor proteins, transcription regulators and RNA processing factors, regulatory proteins like small GTPases and G-proteins, kinases and phosphatases, and heat shock proteins. These are proposed to be involved in junction assembly, barrier regulation, gene transcription, and perhaps other, presently undefined pathways.
Class
Cellular Processes; Cell communication
BRITE hierarchy
Pathway map
Tight junction
hsa04530

All organismsOrtholog table
Disease
H00578  
MYH9-related kidney diseases
H00594  
Distal muscular dystrophies
H00606  
Early infantile epileptic encephalopathy
H00626  
Nephrotic syndrome and focal segmental glomerulosclerosis
H00656  
Scapuloperoneal myopathy (SPM)
H00703  
Myosin storage myopathy (MSM)
H00742  
Neonatal ichthyosis-sclerosing cholangitis (NISCH) syndrome
H00801  
Familial thoracic aortic aneurysm and dissection (TAAD)
H00811  
Distal arthrogryposis (DA)
H00840  
Band-like calcification with simplified gyration and polymicrogyria (BLC-PMG)
H00872  
Trismus-pseudocamptodactyly syndrome
H00894  
FG syndrome (FGS)
H00947  
Pilomatricoma
H01195  
VACTERL/VATER association
H01210  
Hypomagnesemia
H01229  
Inclusion body myopathy 3 (IBM3)
H01255  
Juvenile-onset dystonia
H01301  
Hemorrhagic destruction of the brain, subependymal calcification, and cataracts
Drug
D04510  
Imciromab pentetate (USAN)
D09671  
Sotrastaurin (USAN/INN)
D09718  
Sotrastaurin acetate (USAN)
Other DBs
BSID: 
GO: 
Organism
Homo sapiens (human) [GN:hsa]
Gene
1364  
CLDN4; claudin 4 [KO:K06087]
1365  
CLDN3; claudin 3 [KO:K06087]
1366  
CLDN7; claudin 7 [KO:K06087]
149461  
CLDN19; claudin 19 [KO:K06087]
10686  
CLDN16; claudin 16 [KO:K06087]
23562  
CLDN14; claudin 14 [KO:K06087]
24146  
CLDN15; claudin 15 [KO:K06087]
26285  
CLDN17; claudin 17 [KO:K06087]
49861  
CLDN20; claudin 20 [KO:K06087]
5010  
CLDN11; claudin 11 [KO:K06087]
51208  
CLDN18; claudin 18 [KO:K06087]
53842  
CLDN22; claudin 22 [KO:K06087]
7122  
CLDN5; claudin 5 [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]
137075  
CLDN23; claudin 23 [KO:K06087]
644672  
CLDN25; claudin 25 [KO:K06087]
CLDN24; claudin 24 [KO:K06087]
OCLN; occludin [KO:K06088]
50848  
F11R; F11 receptor [KO:K06089]
58494  
JAM2; junctional adhesion molecule 2 [KO:K06735]
83700  
JAM3; junctional adhesion molecule 3 [KO:K06785]
150084  
IGSF5; immunoglobulin superfamily, member 5 [KO:K06786]
92359  
CRB3; crumbs family member 3 [KO:K06090]
64398  
MPP5; membrane protein, palmitoylated 5 (MAGUK p55 subfamily member 5) [KO:K06091]
10207  
INADL; InaD-like (Drosophila) [KO:K06092]
50855  
PARD6A; par-6 family cell polarity regulator alpha [KO:K06093]
84552  
PARD6G; par-6 family cell polarity regulator gamma [KO:K06093]
84612  
PARD6B; par-6 family cell polarity regulator beta [KO:K06093]
5584  
PRKCI; protein kinase C, iota [KO:K06069] [EC:2.7.11.13]
5590  
PRKCZ; protein kinase C, zeta [KO:K06069] [EC:2.7.11.13]
56288  
PARD3; par-3 family cell polarity regulator [KO:K04237]
3993  
LLGL2; lethal giant larvae homolog 2 (Drosophila) [KO:K06094]
3996  
LLGL1; lethal giant larvae homolog 1 (Drosophila) [KO:K06094]
998  
CDC42; cell division cycle 42 [KO:K04393]
5515  
PPP2CA; protein phosphatase 2, catalytic subunit, alpha isozyme [KO:K04382] [EC:3.1.3.16]
5516  
PPP2CB; protein phosphatase 2, catalytic subunit, beta isozyme [KO:K04382] [EC:3.1.3.16]
5519  
PPP2R1B; protein phosphatase 2, regulatory subunit A, beta [KO:K03456]
5518  
PPP2R1A; protein phosphatase 2, regulatory subunit A, alpha [KO:K03456]
5520  
PPP2R2A; protein phosphatase 2, regulatory subunit B, alpha [KO:K04354]
5521  
PPP2R2B; protein phosphatase 2, regulatory subunit B, beta [KO:K04354]
5522  
PPP2R2C; protein phosphatase 2, regulatory subunit B, gamma [KO:K04354]
55844  
PPP2R2D; protein phosphatase 2, regulatory subunit B, delta [KO:K04354]
5578  
PRKCA; protein kinase C, alpha [KO:K02677] [EC:2.7.11.13]
5579  
PRKCB; protein kinase C, beta [KO:K02677] [EC:2.7.11.13]
5582  
PRKCG; protein kinase C, gamma [KO:K02677] [EC:2.7.11.13]
5580  
PRKCD; protein kinase C, delta [KO:K06068] [EC:2.7.11.13]
5581  
PRKCE; protein kinase C, epsilon [KO:K18050] [EC:2.7.11.13]
5583  
PRKCH; protein kinase C, eta [KO:K18051] [EC:2.7.11.13]
5588  
PRKCQ; protein kinase C, theta [KO:K18052] [EC:2.7.11.13]
1457  
CSNK2A1; casein kinase 2, alpha 1 polypeptide [KO:K03097] [EC:2.7.11.1]
1459  
CSNK2A2; casein kinase 2, alpha prime polypeptide [KO:K03097] [EC:2.7.11.1]
1460  
CSNK2B; casein kinase 2, beta polypeptide [KO:K03115]
2770  
GNAI1; guanine nucleotide binding protein (G protein), alpha inhibiting activity polypeptide 1 [KO:K04630]
2773  
GNAI3; guanine nucleotide binding protein (G protein), alpha inhibiting activity polypeptide 3 [KO:K04630]
2771  
GNAI2; guanine nucleotide binding protein (G protein), alpha inhibiting activity polypeptide 2 [KO:K04630]
8777  
MPDZ; multiple PDZ domain protein [KO:K06095]
9218  
VAPA; VAMP (vesicle-associated membrane protein)-associated protein A, 33kDa [KO:K06096]
27134  
TJP3; tight junction protein 3 [KO:K06097]
7082  
TJP1; tight junction protein 1 [KO:K05701]
9414  
TJP2; tight junction protein 2 [KO:K06098]
51776  
ZAK; sterile alpha motif and leucine zipper containing kinase AZK [KO:K04424] [EC:2.7.11.25]
1499  
CTNNB1; catenin (cadherin-associated protein), beta 1, 88kDa [KO:K02105]
8531  
YBX3; Y box binding protein 3 [KO:K06099]
8189  
SYMPK; symplekin [KO:K06100]
55870  
ASH1L; ash1 (absent, small, or homeotic)-like (Drosophila) [KO:K06101] [EC:2.1.1.43]
1019  
CDK4; cyclin-dependent kinase 4 [KO:K02089] [EC:2.7.11.22]
57530  
CGN; cingulin [KO:K06102]
60  
ACTB; actin, beta [KO:K05692]
71  
ACTG1; actin, gamma 1 [KO:K05692]
4633  
MYL2; myosin, light chain 2, regulatory, cardiac, slow [KO:K10351]
4636  
MYL5; myosin, light chain 5, regulatory [KO:K12753]
58498  
MYL7; myosin, light chain 7, regulatory [KO:K12754]
10398  
MYL9; myosin, light chain 9, regulatory [KO:K12755]
93408  
MYL10; myosin, light chain 10, regulatory [KO:K12756]
103910  
MYL12B; myosin, light chain 12B, regulatory [KO:K12757]
10627  
MYL12A; myosin, light chain 12A, regulatory, non-sarcomeric [KO:K12757]
29895  
MYLPF; myosin light chain, phosphorylatable, fast skeletal muscle [KO:K12758]
22989  
MYH15; myosin, heavy chain 15 [KO:K10352]
4619  
MYH1; myosin, heavy chain 1, skeletal muscle, adult [KO:K10352]
4620  
MYH2; myosin, heavy chain 2, skeletal muscle, adult [KO:K10352]
4621  
MYH3; myosin, heavy chain 3, skeletal muscle, embryonic [KO:K10352]
4622  
MYH4; myosin, heavy chain 4, skeletal muscle [KO:K10352]
4626  
MYH8; myosin, heavy chain 8, skeletal muscle, perinatal [KO:K10352]
4627  
MYH9; myosin, heavy chain 9, non-muscle [KO:K10352]
4628  
MYH10; myosin, heavy chain 10, non-muscle [KO:K10352]
4629  
MYH11; myosin, heavy chain 11, smooth muscle [KO:K10352]
57644  
MYH7B; myosin, heavy chain 7B, cardiac muscle, beta [KO:K10352]
79784  
MYH14; myosin, heavy chain 14, non-muscle [KO:K10352]
8735  
MYH13; myosin, heavy chain 13, skeletal muscle [KO:K10352]
4625  
MYH7; myosin, heavy chain 7, cardiac muscle, beta [KO:K17751]
4624  
MYH6; myosin, heavy chain 6, cardiac muscle, alpha [KO:K17751]
387  
RHOA; ras homolog family member A [KO:K04513]
4301  
MLLT4; myeloid/lymphoid or mixed-lineage leukemia (trithorax homolog, Drosophila) [KO:K05702]
3265  
HRAS; Harvey rat sarcoma viral oncogene homolog [KO:K02833]
3845  
KRAS; Kirsten rat sarcoma viral oncogene homolog [KO:K07827]
4893  
NRAS; neuroblastoma RAS viral (v-ras) oncogene homolog [KO:K07828]
6237  
RRAS; related RAS viral (r-ras) oncogene homolog [KO:K07829]
22800  
RRAS2; related RAS viral (r-ras) oncogene homolog 2 [KO:K07830]
22808  
MRAS; muscle RAS oncogene homolog [KO:K07831]
8573  
CASK; calcium/calmodulin-dependent serine protein kinase (MAGUK family) [KO:K06103] [EC:2.7.11.1]
154810  
AMOTL1; angiomotin like 1 [KO:K06104]
93643  
TJAP1; tight junction associated protein 1 (peripheral) [KO:K06105]
2017  
CTTN; cortactin [KO:K06106]
3059  
HCLS1; hematopoietic cell-specific Lyn substrate 1 [KO:K06106]
29119  
CTNNA3; catenin (cadherin-associated protein), alpha 3 [KO:K05691]
1495  
CTNNA1; catenin (cadherin-associated protein), alpha 1, 102kDa [KO:K05691]
1496  
CTNNA2; catenin (cadherin-associated protein), alpha 2 [KO:K05691]
2035  
EPB41; erythrocyte membrane protein band 4.1 [KO:K06107]
2036  
EPB41L1; erythrocyte membrane protein band 4.1-like 1 [KO:K06107]
2037  
EPB41L2; erythrocyte membrane protein band 4.1-like 2 [KO:K06107]
23136  
EPB41L3; erythrocyte membrane protein band 4.1-like 3 [KO:K06107]
87  
ACTN1; actinin, alpha 1 [KO:K05699]
88  
ACTN2; actinin, alpha 2 [KO:K05699]
89  
ACTN3; actinin, alpha 3 [KO:K05699]
81  
ACTN4; actinin, alpha 4 [KO:K05699]
6714  
SRC; v-src avian sarcoma (Schmidt-Ruppin A-2) viral oncogene homolog [KO:K05704] [EC:2.7.10.2]
7525  
YES1; v-yes-1 Yamaguchi sarcoma viral oncogene homolog 1 [KO:K05705] [EC:2.7.10.2]
5865  
RAB3B; RAB3B, member RAS oncogene family [KO:K06108]
5872  
RAB13; RAB13, member RAS oncogene family [KO:K06109]
11336  
EXOC3; exocyst complex component 3 [KO:K06110]
60412  
EXOC4; exocyst complex component 4 [KO:K06111]
9223  
MAGI1; membrane associated guanylate kinase, WW and PDZ domain containing 1 [KO:K05631]
9863  
MAGI2; membrane associated guanylate kinase, WW and PDZ domain containing 2 [KO:K05629]
260425  
MAGI3; membrane associated guanylate kinase, WW and PDZ domain containing 3 [KO:K06112]
5728  
PTEN; phosphatase and tensin homolog [KO:K01110] [EC:3.1.3.67 3.1.3.48 3.1.3.16]
207  
AKT1; v-akt murine thymoma viral oncogene homolog 1 [KO:K04456] [EC:2.7.11.1]
208  
AKT2; v-akt murine thymoma viral oncogene homolog 2 [KO:K04456] [EC:2.7.11.1]
10000  
AKT3; v-akt murine thymoma viral oncogene homolog 3 [KO:K04456] [EC:2.7.11.1]
6709  
SPTAN1; spectrin, alpha, non-erythrocytic 1 [KO:K06114]
Compound
C05981  
Phosphatidylinositol-3,4,5-trisphosphate
Reference
  Authors
Schneeberger EE, Lynch RD.
  Title
The tight junction: a multifunctional complex.
  Journal
Am J Physiol Cell Physiol 286:C1213-28 (2004)
Reference
  Authors
Bazzoni G, Dejana E.
  Title
Endothelial cell-to-cell junctions: molecular organization and role in vascular homeostasis.
  Journal
Physiol Rev 84:869-901 (2004)
Reference
  Authors
Matter K, Balda MS.
  Title
Signalling to and from tight junctions.
  Journal
Nat Rev Mol Cell Biol 4:225-36 (2003)
Reference
  Authors
Tsukita S, Furuse M, Itoh M.
  Title
Multifunctional strands in tight junctions.
  Journal
Nat Rev Mol Cell Biol 2:285-93 (2001)
Reference
  Authors
Gonzalez-Mariscal L, Betanzos A, Nava P, Jaramillo BE.
  Title
Tight junction proteins.
  Journal
Prog Biophys Mol Biol 81:1-44 (2003)
Reference
  Authors
Cheng CY, Mruk DD.
  Title
Cell junction dynamics in the testis: Sertoli-germ cell interactions and male contraceptive development.
  Journal
Physiol Rev 82:825-74 (2002)
Reference
  Authors
Nakanishi H, Takai Y.
  Title
Roles of nectins in cell adhesion, migration and polarization.
  Journal
Biol Chem 385:885-92 (2004)
Reference
  Authors
Balda MS, Matter K.
  Title
Epithelial cell adhesion and the regulation of gene expression.
  Journal
Trends Cell Biol 13:310-8 (2003)
KO pathway
 

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