| Entry |
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| Name |
Tight junction
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| 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.
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| Class |
Cellular Processes; Cell communication
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| Pathway map |
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| Disease |
| MYH9-related kidney diseases | | Distal muscular dystrophies | | Early infantile epileptic encephalopathy | | Nephrotic syndrome and focal segmental glomerulosclerosis | | Scapuloperoneal myopathy (SPM) | | Myosin storage myopathy (MSM) | | Neonatal ichthyosis-sclerosing cholangitis (NISCH) syndrome | | Familial thoracic aortic aneurysm and dissection (TAAD) | | Distal arthrogryposis (DA) | | Band-like calcification with simplified gyration and polymicrogyria (BLC-PMG) | | Trismus-pseudocamptodactyly syndrome | | FG syndrome (FGS) | | Pilomatricoma | | VACTERL/VATER association | | Hypomagnesemia | | Inclusion body myopathy 3 (IBM3) | | Juvenile-onset dystonia | | Hemorrhagic destruction of the brain, subependymal calcification, and cataracts |
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| Other DBs |
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| Reference |
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| Authors |
Schneeberger EE, Lynch RD. |
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| Title |
The tight junction: a multifunctional complex. |
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| Journal |
Am J Physiol Cell Physiol 286:C1213-28 (2004) |
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| Reference |
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| Authors |
Bazzoni G, Dejana E. |
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| Title |
Endothelial cell-to-cell junctions: molecular organization and role in vascular homeostasis. |
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| Journal |
Physiol Rev 84:869-901 (2004) |
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| Reference |
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| Authors |
Matter K, Balda MS. |
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| Title |
Signalling to and from tight junctions. |
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| Journal |
Nat Rev Mol Cell Biol 4:225-36 (2003) |
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| Reference |
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| Authors |
Tsukita S, Furuse M, Itoh M. |
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| Title |
Multifunctional strands in tight junctions. |
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| Journal |
Nat Rev Mol Cell Biol 2:285-93 (2001) |
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| Reference |
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| Authors |
Gonzalez-Mariscal L, Betanzos A, Nava P, Jaramillo BE. |
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| Title |
Tight junction proteins. |
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| Journal |
Prog Biophys Mol Biol 81:1-44 (2003) |
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| Reference |
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| Authors |
Cheng CY, Mruk DD. |
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| Title |
Cell junction dynamics in the testis: Sertoli-germ cell interactions and male contraceptive development. |
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| Journal |
Physiol Rev 82:825-74 (2002) |
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| Reference |
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| Authors |
Nakanishi H, Takai Y. |
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| Title |
Roles of nectins in cell adhesion, migration and polarization. |
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| Journal |
Biol Chem 385:885-92 (2004) |
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| Reference |
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| Authors |
Balda MS, Matter K. |
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| Title |
Epithelial cell adhesion and the regulation of gene expression. |
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| Journal |
Trends Cell Biol 13:310-8 (2003) |
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| KO pathway |
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