Permeation Pathway of Homomeric Connexin 26 and Connexin 30 Channels Investigated by Molecular Dynamics

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Permeation of calcium through purified connexin 26 hemichannels.

BACKGROUND Indirect evidence suggests that connexin hemichannels are permeable to Ca(2+), but direct demonstration is lacking. RESULTS Calcium moves into liposomes containing purified Cx26 in response to a concentration gradient. CONCLUSION Cx26 hemichannels are permeable to Ca(2+). SIGNIFICANCE Cx26 hemichannels may play a role in Ca(2+) influx into cells under conditions that lead to he...

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

Connexin 26 (Cx26) and connexin 30 (Cx30) are encoded by two genes (GJB2 and GJB6, respectively) that are found within 50 kb in the same complex deafness locus, DFNB1. Immunocytochemistry and quantitative PCR analysis of Cx30 KO mouse cultures revealed that Cx26 is downregulated at the protein level and at the mRNA level in nonsensory cells located between outer hair cells and the stria vascula...

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trans-dominant inhibition of connexin-43 by mutant connexin-26: implications for dominant connexin disorders affecting epidermal differentiation.

Dominant mutations of GJB2-encoding connexin-26 (Cx26) have pleiotropic effects, causing either hearing impairment (HI) alone or in association with palmoplantar keratoderma (PPK/HI). We examined a British family with the latter phenotype and identified a new dominant GJB2 mutation predicted to eliminate the amino acid residue E42 (DeltaE42) in Cx26. To dissect the pathomechanisms that result i...

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Mutations of the GJB2 gene encoding the connexin 26 (Cx26) gap junction protein, which is widely expressed in the inner ear, are the primary cause of hereditary non-syndromic hearing loss in several populations. The deafness-associated single amino acid substitution of methionine 34 (M34) in the first transmembrane helix (TM1) with a threonine (T) ensues in the production of mutant Cx26M34T cha...

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ژورنال

عنوان ژورنال: Journal of Biomolecular Structure and Dynamics

سال: 2012

ISSN: 0739-1102,1538-0254

DOI: 10.1080/073911012010525027