title: A "Tric" to tighten cell-cell junctions in the cochlea for hearing.
abstract: Tricellulin is a tricellular tight junction-associated membrane protein that controls movement of solutes at these specialized cell intersections. Mutations in the gene encoding tricellulin, TRIC, lead to nonsyndromic deafness. In this issue of the JCI, Nayak et al. created a gene-targeted knockin mouse in order to mimic the pathology of a human TRIC mutation. Deafness appears to be caused either by an increase in the K+ ion concentration around the basolateral surfaces of the outer hair cells or, alternatively, by an increase in small molecules such as ATP around the hair bundle, leading to cellular dysfunction and degeneration. Furthermore, the mice have features suggestive of syndromic hearing loss, which may have implications for care and treatment of patients harboring TRIC mutations.
authors: Division of Cell Biology, Department of Physiology and Cell Biology, Kobe University Graduate School of Medicine, Kobe, Japan.
authors: Tomohito
authors: T
authors: Higashi
authors: Danielle R
authors: DR
authors: Lenz
authors: Mikio
authors: M
authors: Furuse
authors: Karen B
authors: KB
authors: Avraham
mesh_terms: D000818:Animals; D005260:Female; D018072:Hair Cells, Auditory, Outer / Q000378:metabolism*; D034381:Hearing Loss / Q000378:metabolism*; D062794:MARVEL Domain Containing 2 Protein / Q000172:deficiency*; D008297:Male; D019108:Tight Junctions / Q000378:metabolism*
chemical_list: D062794:MARVEL Domain Containing 2 Protein; C508309:Marveld2 protein, mouse
references: Nat Rev Mol Cell Biol. 2001 Apr;2(4):285-93
references: 11283726
references: Arch Intern Med. 2011 Nov 14;171(20):1851-2
references: 22083573
references: Am J Hum Genet. 2010 Jul 9;87(1):101-9
references: 20602916
references: Am J Hum Genet. 2006 Dec;79(6):1040-51
references: 17186462
references: J Cell Sci. 2013 Feb 15;126(Pt 4):966-77
references: 23239027
references: J Cell Sci. 2006 Dec 1;119(Pt 23):4819-27
references: 17130295
references: J Clin Invest. 2013 Sep;123(9):4036-49
references: 23979167
references: Annu Rev Physiol. 2011;73:311-34
references: 21073336
references: JAMA. 2002 Mar 13;287(10):1308-20
references: 11886323
references: J Cell Biol. 2005 Dec 19;171(6):939-45
references: 16365161
references: EMBO J. 2003 Oct 15;22(20):5422-34
references: 14532115
references: Am J Hum Genet. 2011 Feb 11;88(2):127-37
references: 21255762
references: PLoS Genet. 2009 Aug;5(8):e1000610
references: 19696885
references: Nature. 2002 Apr 25;416(6883):874-8
references: 11976689
references: J Cell Sci. 1973 Nov;13(3):763-86
references: 4203962
references: Hum Mol Genet. 2003 Aug 15;12(16):2049-61
references: 12913076
references: J Cell Sci. 2011 Feb 15;124(Pt 4):548-55
references: 21245199
references: J Neurosci. 2004 Aug 11;24(32):7051-62
references: 15306639
references: Proc R Soc Lond B Biol Sci. 1968 Nov 5;171(1023):227-47
references: 4386844
references: J Cell Sci. 2004 Oct 1;117(Pt 21):5087-96
references: 15456848
references: Cell. 2001 Jan 12;104(1):165-72
references: 11163249