The cytoplasmic accumulations of the cataract-associated mutant, Connexin50P88S, are long-lived and form in the endoplasmic reticulum

Experimental Eye Research

Volumn 88, Issue 3, 2009, Pages 600-609

  Lichtenstein, Alexandra ^a   Gaietta, Guido M ^b   Deerinck, Thomas J ^b   Crum, John ^b   Sosinsky, Gina E ^b,c   Beyer, Eric C ^a   Berthoud, Viviana M ^a  

^a UNIVERSITY OF CHICAGO   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

cataract; connexin; correlative microscopy; gap junction; tetracysteine

Indexed keywords

ARSENIC DERIVATIVE; ARSENIC DERIVATIVE FIASH; ARSENIC DERIVATIVE REASH; DIAMINOBENZIDINE; GAP JUNCTION PROTEIN; GAP JUNCTION PROTEIN CX50P88S; UNCLASSIFIED DRUG;

ARTICLE; CARBOXY TERMINAL SEQUENCE; CATARACT; CONTROLLED STUDY; CYTOPLASM; ENDOPLASMIC RETICULUM; FLUORESCENCE MICROSCOPY; GENETIC TRANSFECTION; HELA CELL; HUMAN; HUMAN CELL; IMMUNOFLUORESCENCE; PRIORITY JOURNAL; PROTEIN MOTIF; PROTEIN STABILITY; PROTEIN STRUCTURE; PROTEIN SYNTHESIS; ROUGH ENDOPLASMIC RETICULUM; TOMOGRAPHY; TRANSMISSION ELECTRON MICROSCOPY; ULTRASTRUCTURE;

APOPTOSIS REGULATORY PROTEINS; CALCIUM-BINDING PROTEINS; CATARACT; CONNEXINS; CYTOPLASM; ELECTRON MICROSCOPE TOMOGRAPHY; ENDOPLASMIC RETICULUM; EYE PROTEINS; HELA CELLS; HUMANS; MICROSCOPY, ELECTRON; MUTATION;

EID: 61549139397     PISSN: 00144835     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.exer.2008.11.024     Document Type: Article

References (25)

1. Appenzeller-Herzog, and Hauri H.-P. The ER-Golgi intermediate compartment (ERGIC): in search of its identity and function. J. Cell Sci. 119 (2006) 2173-2183
2. Arora A., Minogue P.J., Liu X., Reddy M.A., Ainsworth J.R., Bhattacharya S.S., Webster A.R., Hunt D.M., Ebihara L., Moore A.T., Beyer E.C., and Berthoud V.M. A novel GJA8 mutation is associated with autosomal dominant lamellar pulverulent cataract: further evidence for gap junction dysfunction in human cataract. J. Med. Genet. 43 (2006) e2
3. Berthoud V.M., Bassnett S., and Beyer E.C. Cultured chicken embryo lens cells resemble differentiating fiber cells in vivo and contain two kinetic pools of connexin56. Exp. Eye Res. 68 (1999) 475-484
4. Berthoud V.M., Minogue P.J., Guo J., Williamson E.K., Xu X., Ebihara L., and Beyer E.C. Loss of function and impaired degradation of a cataract-associated mutant connexin50. Eur. J. Cell Biol. 82 (2003) 209-221
5. Berthoud V.M., Minogue P.J., Laing J.G., and Beyer E.C. Pathways for degradation of connexins and gap junctions. Cardiovasc. Res. 62 (2004) 256-267
6. Gaietta G., Deerinck T.J., Adams S.R., Bouwer J., Tour O., Laird D.W., Sosinsky G.E., Tsien R.Y., and Ellisman M.H. Multicolor and electron microscopic imaging of connexin trafficking. Science 296 (2002) 503-507
7. Gilliland K.O., Freel C.D., Lane C.W., Fowler W.C., and Costello M.J. Multilamellar bodies as potential scattering particles in human age-related nuclear cataracts. Mol. Vis. 7 (2001) 120-130
8. Goodenough D.A. Lens gap junctions: a structural hypothesis for nonregulated low-resistance intercellular pathways. Invest. Ophthalmol. Vis. Sci. 18 (1979) 1104-1122
9. Jamora C., Yamanouye N., Van Lint J., Laudenslager J., Vandenheede J., Faulkner D., and Malhotra V. Gβγ-mediated regulation of Golgi organization is through the direct activation of protein kinase D. Cell 98 (1999) 59-68
10. Jiang J.X., and Goodenough D.A. Phosphorylation of lens-fiber connexins in lens organ cultures. Eur. J. Biochem. 255 (1998) 37-44
11. Kistler J., Kirkland B., and Bullivant S. Identification of a 70,000-D protein in lens membrane junctional domains. J. Cell Biol. 101 (1985) 28-35
12. Kremer J.R., Mastronarde D.N., and McIntosh J.R. Computer visualization of three-dimensional image data using IMOD. J. Struct. Biol. 116 (1996) 71-76
13. Lawrence A., Bouwer J.C., Perkins G., and Ellisman M.H. Transform-based backprojection for volume reconstruction of large format electron microscope tilt series. J. Struct. Biol. 154 (2006) 144-167
14. Lee T.H., and Lindstedt A.D. Potential role for protein kinases in regulation of bidirectional endoplasmic reticulum-to-Golgi transport revealed by protein kinase inhibitor H89. Mol. Biol. Cell 11 (2000) 2577-2590
15. Martone M.E., Tran J., Wong W.W., Sargis J., Fong L., Larson S., Lamont S.P., Gupta A., and Ellisman M.H. The cell centered database project: an update on building community resources for managing and sharing 3D imaging data. J. Struct. Biol. 161 (2008) 220-231
16. Paul D.L., Ebihara L., Takemoto L.J., Swenson K.I., and Goodenough D.A. Connexin46, a novel lens gap junction protein, induces voltage-gated currents in nonjunctional plasma membrane of Xenopus oocytes. J. Cell Biol. 115 (1991) 1077-1089
17. Perkins G.A., Sosinsky G.E., Ghassemzadeh S., Perez A., Jones Y., and Ellisman M.H. Electron tomographic analysis of cytoskeletal cross-bridges in the paranodal region of the node of Ranvier in peripheral nerves. J. Struct. Biol. 161 (2008) 469-480
18. Reddy M.A., Francis P.J., Berry V., Bhattacharya S.S., and Moore A.T. Molecular genetic basis of inherited cataract and associated phenotypes. Surv. Ophthalmol 49 (2004) 300-315
19. Ri Y., Ballesteros J.A., Abrams C.K., Oh S., Verselis V., Weinstein H., and Bargiello T.A. The role of a conserved proline residue in mediating conformational changes with voltage gating of Cx32 gap junctions. Biophys. J. 76 (1999) 2887-2898
20. Rup D.M., Veenstra R.D., Wang H.-Z., Brink P.R., and Beyer E.C. Chick connexin-56, a novel lens gap junction protein. Molecular cloning and functional expression. J. Biol. Chem. 268 (1993) 706-712
21. Sáez J.C., Berthoud V.M., Brañes M.C., Martínez A.D., and Beyer E.C. Plasma membrane channels formed by connexins: their regulation and functions. Physiol. Rev. 83 (2003) 1359-1400
22. Saraste J., and Kuismanen E. Pre- and post-Golgi vacuoles operate in the transport of Semliki Forest virus membrane glycoproteins to the cell surface. Cell 38 (1984) 535-549
23. Shiels A., Mackay D., Ionides A., Berry V., Moore A., and Bhattacharya S. A missense mutation in the human connexin50 gene (GJA8) underlies autosomal dominant " zonular pulverulent" cataract, on chromosome 1q. Am. J. Hum. Genet. 62 (1998) 526-532
24. Suchyna T.M., Xu L.X., Gao F., Fourtner C.R., and Nicholson B.J. Identification of a proline residue as a transduction element involved in voltage gating of gap junctions. Nature 365 (1993) 847-849
25. White T.W., Bruzzone R., Goodenough D.A., and Paul D.L. Mouse Cx50, a functional member of the connexin family of gap junction proteins, is the lens fiber protein MP70. Mol. Biol. Cell 3 (1992) 711-720