Matches in SemOpenAlex for { <https://semopenalex.org/work/W2061220576> ?p ?o ?g. }
- W2061220576 endingPage "69" @default.
- W2061220576 startingPage "52" @default.
- W2061220576 abstract "Mutations in connexins (Cxs), the constitutive protein subunits of gap junction (GJ) intercellular channels, are one of the most common human genetic defects that cause severe prelingual non-syndromic hearing impairments. Many subtypes of Cxs (e.g., Cxs 26, 29, 30, 31, 43) and pannexins (Panxs) are expressed in the cochlea where they contribute to the formation of a GJ-based intercellular communication network. Cx26 and Cx30 are the predominant cochlear Cxs and they co-assemble in most GJ plaques to form hybrid GJs. The cellular localization of specific Cx subtypes provides a basis for understanding the molecular structure of GJs and hemichannels in the cochlea. Information about the interactions among the various co-assembled Cx partners is critical to appreciate the functional consequences of various types of genetic mutations. In vitro studies of reconstituted GJs in cell lines have yielded surprisingly heterogeneous mechanisms of dysfunction caused by various Cx mutations. Availability of multiple lines of Cx-mutant mouse models has provided some insight into the pathogenesis processes in the cochlea of deaf mice. Here we summarize recent advances in understanding the structure and function of cochlear GJs and give a critical review of current findings obtained from both in vitro studies and mouse models on the mechanisms of Cx mutations that lead to cell death in the cochlea and hearing loss." @default.
- W2061220576 created "2016-06-24" @default.
- W2061220576 creator A5011934928 @default.
- W2061220576 creator A5016194081 @default.
- W2061220576 creator A5043327323 @default.
- W2061220576 creator A5054375172 @default.
- W2061220576 creator A5068622836 @default.
- W2061220576 creator A5090872806 @default.
- W2061220576 date "2009-06-01" @default.
- W2061220576 modified "2023-10-17" @default.
- W2061220576 title "Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models" @default.
- W2061220576 cites W136179538 @default.
- W2061220576 cites W1526839233 @default.
- W2061220576 cites W1558835853 @default.
- W2061220576 cites W1566269937 @default.
- W2061220576 cites W1573567421 @default.
- W2061220576 cites W1604501714 @default.
- W2061220576 cites W1623067417 @default.
- W2061220576 cites W1785765296 @default.
- W2061220576 cites W1922067896 @default.
- W2061220576 cites W1950685957 @default.
- W2061220576 cites W1960493235 @default.
- W2061220576 cites W1963768450 @default.
- W2061220576 cites W1964479042 @default.
- W2061220576 cites W1966708427 @default.
- W2061220576 cites W1968140144 @default.
- W2061220576 cites W1969140718 @default.
- W2061220576 cites W1970118869 @default.
- W2061220576 cites W1971116368 @default.
- W2061220576 cites W1972299038 @default.
- W2061220576 cites W1972563179 @default.
- W2061220576 cites W1972687274 @default.
- W2061220576 cites W1973038834 @default.
- W2061220576 cites W1974420316 @default.
- W2061220576 cites W1979492365 @default.
- W2061220576 cites W1980450550 @default.
- W2061220576 cites W1982145211 @default.
- W2061220576 cites W1982322107 @default.
- W2061220576 cites W1985811665 @default.
- W2061220576 cites W1987594610 @default.
- W2061220576 cites W1987762534 @default.
- W2061220576 cites W1988291240 @default.
- W2061220576 cites W1990617520 @default.
- W2061220576 cites W1991259792 @default.
- W2061220576 cites W1992740972 @default.
- W2061220576 cites W1993967715 @default.
- W2061220576 cites W1994416315 @default.
- W2061220576 cites W1996849285 @default.
- W2061220576 cites W1997092809 @default.
- W2061220576 cites W1997151269 @default.
- W2061220576 cites W2002112119 @default.
- W2061220576 cites W2002581254 @default.
- W2061220576 cites W2002945032 @default.
- W2061220576 cites W2003837366 @default.
- W2061220576 cites W2004043082 @default.
- W2061220576 cites W2004859427 @default.
- W2061220576 cites W2005504872 @default.
- W2061220576 cites W2007522135 @default.
- W2061220576 cites W2007985643 @default.
- W2061220576 cites W2007989815 @default.
- W2061220576 cites W2008937128 @default.
- W2061220576 cites W2009257155 @default.
- W2061220576 cites W2011346918 @default.
- W2061220576 cites W2011355532 @default.
- W2061220576 cites W2013475033 @default.
- W2061220576 cites W2013736577 @default.
- W2061220576 cites W2017203200 @default.
- W2061220576 cites W2019857777 @default.
- W2061220576 cites W2020849158 @default.
- W2061220576 cites W2022023926 @default.
- W2061220576 cites W2023163614 @default.
- W2061220576 cites W2023782716 @default.
- W2061220576 cites W2024324016 @default.
- W2061220576 cites W2025147658 @default.
- W2061220576 cites W2025174728 @default.
- W2061220576 cites W2026004874 @default.
- W2061220576 cites W2026411032 @default.
- W2061220576 cites W2028230155 @default.
- W2061220576 cites W2030167054 @default.
- W2061220576 cites W2030313410 @default.
- W2061220576 cites W2034076601 @default.
- W2061220576 cites W2036430171 @default.
- W2061220576 cites W2036777729 @default.
- W2061220576 cites W2036930489 @default.
- W2061220576 cites W2037076862 @default.
- W2061220576 cites W2041913689 @default.
- W2061220576 cites W2042259903 @default.
- W2061220576 cites W2042585863 @default.
- W2061220576 cites W2043690660 @default.
- W2061220576 cites W2043982229 @default.
- W2061220576 cites W2045021920 @default.
- W2061220576 cites W2047669413 @default.
- W2061220576 cites W2048297880 @default.
- W2061220576 cites W2050385103 @default.
- W2061220576 cites W2050630969 @default.
- W2061220576 cites W2051465829 @default.
- W2061220576 cites W2051715869 @default.
- W2061220576 cites W2052971367 @default.