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Full-Text Articles in Cell and Developmental Biology
Ablation Of Both Cx40 And Panx1 Results In Similar Cardiovascular Phenotypes Exhibited In Cx40 Knockout Mice, Nicole M. Novielli-Kuntz, Meghan L. Jelen, Kevin Barr, Leon J. Delalio, Qingping Feng, Brant E. Isakson, Robert Gros, Dale Laird
Ablation Of Both Cx40 And Panx1 Results In Similar Cardiovascular Phenotypes Exhibited In Cx40 Knockout Mice, Nicole M. Novielli-Kuntz, Meghan L. Jelen, Kevin Barr, Leon J. Delalio, Qingping Feng, Brant E. Isakson, Robert Gros, Dale Laird
Anatomy and Cell Biology Publications
Connexins (Cxs) and pannexins (Panxs) are highly regulated large-pore channel-forming proteins that participate in cellular communication via small molecular exchange with the extracellular microenvironment, or in the case of connexins, directly between cells. Given the putative functional overlap between single membrane-spanning connexin hemichannels and Panx channels, and cardiovascular system prevalence, we generated the first Cx40(-/-)Panx1(-/-) mouse with the anticipation that this genetic modification would lead to a severe cardiovascular phenotype. Mice null for both Cx40 and Panx1 produced litter sizes and adult growth progression similar to wild-type (WT), Cx40(-/-) and Panx1(-/-) mice. Akin to Cx40(-/-) mice, Cx40(-/-) Panx1(-/-) mice exhibited …
Skin Disease And Non-Syndromic Hearing Loss-Linked Cx30 Mutations Exhibit Several Distinct Cellular Pathologies, Amy Berger, John Kelly, Patrick Lajoie, Qing Shao, Dale Laird
Skin Disease And Non-Syndromic Hearing Loss-Linked Cx30 Mutations Exhibit Several Distinct Cellular Pathologies, Amy Berger, John Kelly, Patrick Lajoie, Qing Shao, Dale Laird
Anatomy and Cell Biology Publications
Connexin 30 (Cx30), a member of the large gap junction protein family, plays a role in the homeostasis of the epidermis and inner ear through gap junctional intercellular communication (GJIC). Here, we investigated the underlying mechanisms of four autosomal dominant Cx30 gene mutations linked to hearing loss and/or various skin diseases. First, the T5M mutant linked to non-syndromic hearing loss formed functional gap junction channels and hemichannels, similar to wild type Cx30. The loss-of-function V37E mutant associated with Clouston syndrome or keratitis-ichthyosis-deafness syndrome was retained in the endoplasmic reticulum and significantly induced apoptosis. The G59R mutant linked to Vohwinkel and …