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Heterotypic Docking Compatibility Of Human Connexin 37 With Other Vascular Connexins, Nicholas Kim
Heterotypic Docking Compatibility Of Human Connexin 37 With Other Vascular Connexins, Nicholas Kim
Electronic Thesis and Dissertation Repository
Gap junction (GJ) channels provide direct intercellular communication. A GJ channel consists of two docked hemichannels and each hemichannel is a hexamer of six connexins. Human vascular connexins (Cx37, Cx40, Cx43, and Cx45) can form various types of gap junction channels to synchronize vasodilation/constriction to control local circulation. Most of our knowledge on heterotypic GJs of these vascular connexins comes from studies on rodent connexins. However it is not clear if these human connexins can also form heterotypic GJs. The present study used an in vitro expression system to investigate the coupling status and GJ properties of human heterotypic Cx37/Cx40, …
Atrial Fibrillation-Linked Germline Gja5/Connexin40 Mutants Showed An Increased Hemichannel Function, Matthew D. Hills
Atrial Fibrillation-Linked Germline Gja5/Connexin40 Mutants Showed An Increased Hemichannel Function, Matthew D. Hills
Electronic Thesis and Dissertation Repository
Mutations in GJA5 encoding the gap junction protein connexin40 (Cx40) have been linked to lone atrial fibrillation (AF). Some of these mutants result in impaired gap junction function due to either abnormal connexin localization or impaired gap junction channels, which may play a role in promoting AF. However, the effects of the AF-linked Cx40 mutants on hemichannel function has not been studied. Here we investigated two AF-linked germline Cx40 mutants, V85I and L221I. These two mutants formed putative gap junction plaques at cell-cell interfaces, with similar gap junction coupling conductance as that of wild-type Cx40. Connexin deficient HeLa cells expressing …
The Distribution And Functional Properties Of Pmld-Linked Cx47 Mutations On Cx47/Cx47 Homotypic And Cx47/Cx43 Heterotypic Gap Junctions, Mi Seong Kim
Electronic Thesis and Dissertation Repository
Gap junctions (GJ) allow direct intercellular communication, and consist of connexins (Cx). In the mammalian central nervous system, oligodendrocytes express Cx29, Cx32 and Cx47, whereas astrocytes express Cx26, Cx30 and Cx43. Cx47/Cx47 GJs couple oligodendrocytes, and Cx47/Cx43 channels are the primary heterotypic GJs at oligodendrocyte/astrocyte junctions. Interestingly, mutations in Cx47 have been linked to central hypomyelinating Pelizaeus Merzbacher-like Disease (PMLD). My aim is to determine the cellular distribution and functional properties of PMLD-associated Cx47 mutants (I46M, G149S, G236R, G236S, M286T, T398I). All mutants were detected at the cell-cell interface. These mutants, except G149S and T398I, showed no electrical coupling in …