Coordinated control of connexin 26 and connexin 30 at the regulatory and functional level in the inner ear

Proceedings of the National Academy of Sciences of the United States of America

Volumn 105, Issue 48, 2008, Pages 18776-18781

  Ortolano, Saida ^a,b   Di Pasquale, Giovanni ^a   Crispino, Giulia ^a   Anselmi, Fabio ^a   Mammano, Fabio ^a,b   Chiorini, John A ^c  

^a VENETIAN INSTITUTE OF MOLECULAR MEDICINE   (Italy)

^b UNIVERSITY OF PADOVA   (Italy)

^c NATIONAL INSTITUTE OF DENTAL AND CRANIOFACIAL RESEARCH   (United States)

Author keywords

Adeno associated virus; Cochlea; Gap junctions; Gene transfer; Hearing loss

Indexed keywords

CALCIUM ION; CONNEXIN 26; CONNEXIN 30; CRE RECOMBINASE; I KAPPA B; IMMUNOGLOBULIN ENHANCER BINDING PROTEIN;

ARTICLE; BOVINE ADENOVIRUS; CALCIUM SIGNALING; CELL COMMUNICATION; CONTROLLED STUDY; DOWN REGULATION; EVOKED RESPONSE; GAP JUNCTION; GENE EXPRESSION; GENE EXPRESSION REGULATION; GENETIC TRANSDUCTION; INNER EAR; MOUSE; NONHUMAN; PRIORITY JOURNAL; REGULATORY MECHANISM; STIMULATION;

ANIMALS; CALCIUM; CALCIUM SIGNALING; CATTLE; CONNEXINS; DEAFNESS; EAR, INNER; HUMANS; MICE; MICE, KNOCKOUT; NF-KAPPA B; PROTEIN ISOFORMS; RECOMBINANT FUSION PROTEINS; TISSUE CULTURE TECHNIQUES;

ADENO-ASSOCIATED VIRUS; BOVINE ADENO-ASSOCIATED VIRUS;

EID: 57749121610     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.0800831105     Document Type: Article

References (58)

1. Forge A, et al. (2003) Gap junctions in the inner ear: Comparison of distribution patterns in different vertebrates and assessment of connexin composition in mammals. J Comp Neurol 467:207-231.
2. Cohen-Salmon M, et al. (2004) Expression of the connexin43- and connexin45-encoding genes in the developing and mature mouse inner ear. Cell Tissue Res 316:15-22.
3. Suzuki T, Takamatsu T, Oyamada M (2003) Expression of gap junction protein connexin43 in the adult rat cochlea: Comparison with connexin26. J Histochem Cytochem 51:903-912.
4. Kikuchi T, Adams JC, Paul DL, Kimura RS (1994) Gap junction systems in the rat vestibular labyrinth: Immunohistochemical and ultrastructural analysis. Acta Otolaryngol 114:520-528.
5. Kikuchi T, Kimura RS, Paul DL, Adams JC (1995) Gap junctions in the rat cochlea: Immunohistochemical and ultrastructural analysis. Anat Embryol 191:101-118.
6. Lautermann J, et al. (1998) Expression of the gap-junction connexins 26 and 30 in the rat cochlea. Cell Tissue Res 294:415-420.
7. Lautermann J, Frank HG, Jahnke K, Traub O, Winterhager E (1999) Developmental expression patterns of connexin 26 and 30 in the rat cochlea. Dev Genet (Amsterdam) 25:306-311.
8. Krstic RV (1997) Human Microscopic Anatomy (Springer, Berlin).
9. Hibino H, Kurachi Y (2006) Molecular and physiological bases of the K circulation in the mammalian inner ear. Physiology (Bethesda) 21:336-345.
10. Wangemann P (2006) Supporting sensory transduction: Cochlear fluid homeostasis and the endocochlear potential. J Physiol 576:11-21.
11. Zhao HB, Kikuchi T, Ngezahayo A, White TW (2006) Gap junctions and cochlear homeostasis. J Membr Biol 209:177-186.
12. 2+ signaling in the inner ear. Physiology (Bethesda) 22:131-144.
13. Zhao HB, Santos-Sacchi J (2000) Voltage gating of gap junctions in cochlear supporting cells: Evidence for nonhomotypic channels. J Membr Biol 175:17-24.
14. Forge A, Marziano NK, Casalotti SO, Becker DL, Jagger D (2003) The inner ear contains heteromeric channels composed of cx26 and cx30 and deafness-related mutations in cx26 have a dominant negative effect on cx30. Cell Commun Adhes 10:341-346.
15. 2+ signaling than homomeric counterparts. Am J Physiol 288:C613-C623.
16. Harris AL (2001) Emerging issues of connexin channels: Biophysics fills the gap. Q Rev Biophys 34:325-472.
17. Hernandez VH, et al. (2007) Unitary permeability of gap junction channels to second messengers measured by FRET microscopy. Nat Methods 4:353-358.
18. Harris AL (2007) Connexin channel permeability to cytoplasmic molecules. Prog Biophys Mol Biol 94:120-143.
19. Zelante L, et al. (1997) Connexin26 mutations associated with the most common form of non-syndromic neurosensory autosomal recessive deafness (DFNB1) in Mediterraneans. Hum Mol Genet 6:1605-1609.
20. Snoeckx RL, et al. (2005) GJB2 mutations and degree of hearing loss: A multicenter study. Am J Hum Genet 77:945-957.
21. Cohen-Salmon M, et al. (2002) Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell death. Curr Biol 12:1106-1111.
22. Teubner B, et al. (2003) Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential. Hum Mol Genet 12:13-21.
23. Cohen-Salmon M, del Castillo FJ, Petit C (2005) Connexins responsible for hereditary deafness - The tale unfolds. Gap Junctions in Development and Disease, ed Winterhager E (Springer, Berlin), pp 111-134.
24. del Castillo I, et al. (2002) A deletion involving the connexin 30 gene in nonsyndromic hearing impairment. N Engl J Med 346:243-249.
25. del Castillo FJ, et al. (2005) A novel deletion involving the connexin-30 gene, del(GJB6-d13s1854), found in trans with mutations in the GJB2 gene (connexin-26) in subjects with DFNB1 non-syndromic hearing impairment. J Med Genet 42:588-594.
26. del Castillo I, et al. (2003) Prevalence and evolutionary origins of the del(GJB6-D13S1830) mutation in the DFNB1 locus in hearing-impaired subjects: A multicenter study. Am J Hum Genet 73:1452-1458.
27. Wilch E, et al. (2006) Expression of GJB2 and GJB6 is reduced in a novel DFNB1 allele. Am J Hum Genet 79:174-179.
28. Ahmad S, et al. (2007) Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness. Proc Natl Acad Sci USA 104:1337-1341.
29. White TW, Bruzzone R (2000) Gap junctions: Fates worse than death? Curr Biol 10:R685-R688.
30. Gerido DA, White TW (2004) Connexin disorders of the ear, skin, and lens. Biochim Biophys Acta 1662:159-170.
31. Oyamada M, Oyamada Y, Takamatsu T (2005) Regulation of connexin expression. Biochim Biophys Acta 1719:6-23.
32. Schmidt M, Katano H, Bossis I, Chiorini JA (2004) Cloning and characterization of a bovine adeno-associated virus. J Virol 78:6509-6516.
33. Di Pasquale G, et al. (2005) A novel bovine virus efficiently transduces inner ear neuroepithelial cells. Mol Ther 11:849-855.
34. Stancovski I, Baltimore D (1997) NF-kappaB activation: The I kappaB kinase revealed? Cell 91:299-302.
35. Verma IM, Stevenson JK, Schwarz EM, Van Antwerp D, Miyamoto S (1995) Rel/NF-kappa B/I kappa B family: Intimate tales of association and dissociation. Genes Dev 9:2723-2735.
36. Dolmetsch RE, Xu K, Lewis RS (1998) Calcium oscillations increase the efficiency and specificity of gene expression. Nature 392:933-936.
37. Jagger DJ, Forge A (2006) Compartmentalized and signal-selective gap junctional coupling in the hearing cochlea. J Neurosci 26:1260-1268.
38. Tritsch NX, Yi E, Gale JE, Glowatzki E, Bergles DE (2007) The origin of spontaneous activity in the developing auditory system. Nature 450:50-55.
39. Wade MH, Trosko JE, Schindler M (1986) A fluorescence photobleaching assay of gap junction-mediated communication between human cells. Science 232:525-528.
40. Rabut G, Ellenberg J (2005) Photobleaching techniques to study mobility and molecular dynamics of proteins in live cells: FRAP, iFRAP, and FLIP. Live Cell Imaging, eds. Goldman RD, Spector DL (Cold Spring Harbor Lab Press, Cold Spring Harbor, NY) pp. 101-126.
41. Gale JE, Piazza V, Ciubotaru CD, Mammano F (2004) A mechanism for sensing noise damage in the inner ear. Curr Biol 14:526-529.
42. Beltramello M, Piazza V, Bukauskas FF, Pozzan T, Mammano F (2005) Impaired permeability to Ins(1,4,5)P3 in amutant connexin underlies recessive hereditary deafness. Nat Cell Biol 7:63-69.
43. 2+ wave propagation in the organ of Corti. Cell Calcium 41:77-86.
44. 2+ spike frequency can optimize gene expression. Nature 392:936-941.
45. Hu Q, Deshpande S, Irani K, Ziegelstein RC (1999) [Ca2]i oscillation frequency regulates agonist-stimulated NF-kappaB transcriptional activity. J Biol Chem 274:33995-33998.
46. Nelson DE, et al. (2004) Oscillations in NF-kappaB signaling control the dynamics of gene expression. Science 306:704-708.
47. Hennemann H, Kozjek G, Dahl E, Nicholson B, Willecke K (1992) Molecular cloning of mouse connexins 26 and 32: Similar genomic organization but distinct promoter sequences of two gap junction genes. Eur J Cell Biol 58:81-89.
48. Tu ZJ, Kiang DT (1998) Mapping and characterization of the basal promoter of the human connexin26 gene. Biochim Biophys Acta 1443:169-181.
49. Zhao Y, Rivieccio MA, Lutz S, Scemes E, Brosnan CF (2006) The TLR3 ligand polyI: C downregulates connexin 43 expression and function in astrocytes by a mechanism involving the NF-kappaB and PI3 kinase pathways. Glia 54:775-785.
50. Zhao HB, Yu N (2006) Distinct and gradient distributions of connexin26 and connexin30 in the cochlear sensory epithelium of guinea pigs. J Comp Neurol 499:506-518.
51. Nelles E, et al. (1996) Defective propagation of signals generated by sympathetic nerve stimulation in the liver of connexin32-deficient mice. Proc Natl Acad Sci USA 93:9565-9570.
52. Valiunas V, Niessen H, Willecke K, Weingart R (1999) Electrophysiological properties of gap junction channels in hepatocytes isolated from connexin32-deficient and wild-type mice. Pflügers Arch 437:846-856.
53. Dahl E, et al. (1996) Molecular cloning and functional expression of mouse connexin-30, a gap junction gene highly expressed in adult brain and skin. J Biol Chem 271:17903-17910.
54. Qu Y, et al. (2007) Analysis of connexin subunits required for the survival of vestibular hair cells. J Comp Neurol 504:499-507.
55. Cohen-Salmon M, et al. (2007) Connexin30 deficiency causes intrastrial fluid-blood barrier disruption within the cochlear stria vascularis. Proc Natl Acad Sci USA 104:6229-6234.
56. Di Pasquale G, Chiorini JA (2006) AAV transcytosis through barrier epithelia and endothelium. Mol Ther 13:506-516.
57. Kaludov N, Handelman B, Chiorini JA (2002) Scalable purification of adeno-associated virus type 2, 4, or 5 using ion-exchange chromatography. Hum Gene Ther 13:1235-1243.
58. Pfaffl MW (2001) A new mathematical model for relative quantification in real-time RT-PCR. Nucleic Acids Res 29:e45.