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Entry
map04530                    Pathway                                

Name
Tight junction
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; Cellular commiunity
BRITE hierarchy
Pathway map
Tight junction
map04530

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
Other DBs
GO: 
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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