X-linked Charcot-Marie-Tooth disease (CMTX1) results from numerous mutations in the GJB1 gene encoding the gap junction protein connexin32 (Cx32) and is one of the commonest forms of inherited neuropathy. (korea.ac.kr)
While most GJB1 mutations appear to cause peripheral neuropathy through loss of Cx32 function, the cellular mechanisms underlying the CNS manifestations remain controversial. (korea.ac.kr)
X-linked Charcot-Marie-Tooth disease (CMT1X), one of the commonest forms of inherited demyelinating neuropathy, results from GJB1 genemutations causing loss of function of the gap junction protein connexin32 (Cx32). (quanterix.com)
The aim of this study was to examine whether delayed gene replacement therapy after the onset of peripheral neuropathy can provide a therapeutic benefit in the Gjb1-null/Cx32 knockout (KO) model of CMT1X. (quanterix.com)
After delivery of the LV-Mpz.GJB1 lentiviral vector by a single lumbar intrathecal injection into 6-month old Gjb1-null mice we confirmed expression of Cx32 in lumbar roots and sciatic nerves correctly localized at the paranodal myelin areas. (quanterix.com)
X-linked Charcot-Marie-Tooth disease type 1 (CMT1X) is a demyelinating neuropathy resulting from loss-of-function mutations affecting the GJB1/connexin 32 (Cx32) gene. (bvsalud.org)
We previously showed functional and morphological improvement in Gjb1-null mice following AAV9-mediated delivery of human Cx32 driven by the myelin protein zero (Mpz) promoter in Schwann cells. (bvsalud.org)
Cx263
U ovom radu istražen je poremećaj u staničnoj komunikaciji u yotari miševima analizom izražaja koneksina (Cxs) Cx26, Cx32, Cx37, Cx40, Cx43 i Cx45 tijekom razvoja jetre embrionalnih dana E13.5 i E15.5. (unist.hr)
We discovered that a small subset of beta connexins (Cx26, Cx30 and Cx32) are directly sensitive to CO 2 -the plasma membrane channels of these connexins open to increases in CO 2 . (warwick.ac.uk)
Studies using microsomes have revealed that oligomerization of connexins Cx26, Cx43, and Cx32 can occur after insertion of connexins in the ER membrane (Falk et al. (reactome.org)
Cx433
Cx32 in both cells localized in cytoplasm did not form intercellular plaques, and decreased Cx43 expression. (iyte.edu.tr)
Cx32 varlığı Cx43 ifadesini azaltırken, bu azalma Hs578T hücresinde anlamlıdır. (iyte.edu.tr)
Here, we describe a portion of the pore structure of unapposed hemichannels formed by a Cx32 chimera, Cx32*Cx43E1, in which the first extracellular loop (E1) of Cx32 is replaced with the E1 of Cx43. (elsevierpure.com)
Protein2
Finally, lysosomal and proteasomal inhibition in cells expressing the start codon mutant constructs failed to recover any detection of Cx32 as a result of impaired protein degradation. (korea.ac.kr)
Our results indicate that the Cx32 start codon mutation is equivalent to a complete loss of the protein with failure of translation, although transcription is not impaired. (korea.ac.kr)
Schwann1
Owing to the expression of Cx32 not only in Schwann cells but also in oligodendrocytes, a subset of CMT1X patients develops central nervous system (CNS) clinical manifestations in addition to peripheral neuropathy. (korea.ac.kr)
Sciatic1
Re-establishment of WT Cx32 function resulted in improved muscle strength and increased sciatic nerve motor conduction velocities in all treated groups from both mutant lines when treated before as well as after the onset of the neuropathy. (bvsalud.org)
Cellular1
Cx32 overexpression increased cellular proliferation with significant increase in S phase in Hs578T cells with no significant change on MCF7 cells. (iyte.edu.tr)
Expression3
None of the cloned constructs resulted in detectable expression of Cx32 by immunocytochemistry or immunoblot, although mRNA was produced at normal levels. (korea.ac.kr)
Widespread expression of virally delivered Cx32 was demonstrated in both genotypes. (bvsalud.org)
In contrast, Cx32 expression decreased in cells treated with NDMA and ESP. (yonsei.ac.kr)
Function1
Thus, complete loss of Cx32 function is sufficient to produce CNS dysfunction with clinical manifestations. (korea.ac.kr)
Cells6
Through overexpressing Cx32, its functions in breast cancer cells were investigated in Hs578T and MCF7 breast cancer cells. (iyte.edu.tr)
Cx32 overexpression reduced the migration and invasion capacity in both cells and in Hs578T cells showed reduction of mesenchymal and increase of epithelial marker expressions. (iyte.edu.tr)
In conclusion, Cx32 increases proliferation and decreases communication in Hs578T cells while not affecting MCF7 cells. (iyte.edu.tr)
Due to changes in gap junctional functions, Cx32 might be acting in relation to GJIC in Hs578T cells and outside of it in MCF7 cells. (iyte.edu.tr)
All in all, presence of Cx32 made Hs578T cells act similar to endogenously Cx32 expressing MCF7 cells. (iyte.edu.tr)
Together, these data show that Cx29 and Cx32 are expressed by myelinating glial cells with unique distributions. (acp2018.org)
Complete1
However, the complete picture for the role of Cx32 in breast cancer remains to be elusive. (iyte.edu.tr)
Connexin 324
Using quantitative stereologic analyses we demonstrated that decreased expression of the major hepatocyte gap junction protein, connexin 32 (Cx32), in rat AHF is a consistent observation in several protocols of multistage hepatocarcinogenesis. (nih.gov)
CMT1X, the X-linked form of Charcot-Marie-Tooth disease, is associated with mutations in connexin 32 (Cx32), a gap junction protein expressed in Schwann cells (SCs) and in oligodendrocytes in the CNS. (uic.edu)
On evaluating cell surface protein that are recognized to control stem cell proliferation, we noticed a reduced appearance of cell surface area connexin 32 (Cx32) plaques in GRP94-null LSK cells. (monossabios.com)
Connexin 43 (Cx43) in the endosteal specific niche market is an essential regulator of HSC homing and migration within an irradiated microenvironment [25], while connexin 32 (Cx32) can be necessary for preserving hematopoietic progenitors in the BM. (monossabios.com)
Cx475
In addition to the extensive gap junction coupling between astrocytes themselves, oligodendrocytes are thought to be exclusively coupled to astrocytes (O:A coupling) via heterotypic gap junctions composed of Cx47:Cx43 and Cx32:Cx30. (nih.gov)
We used fluorescent dyes to examine functional coupling in acute slices from the cerebra of mice lacking Cx32 and/or Cx47. (nih.gov)
O:O coupling was more affected in mice lacking Cx32 than in mice lacking Cx47. (nih.gov)
Cx47, but not Cx32, was required for O:A coupling. (nih.gov)
A pro-inflammatory environment connected with elevated cytokine levels at baseline has been not too long ago documented in Cx32/Cx47 dKO mice suggesting that connexin deficiency in oligodendrocytes drives CNS inflammation independently of external immune triggers [74]. (dna-alkylating.com)
Mice6
We have previously shown that form the standpoint of RNA expression, the peripheral nerves of mice lacking Cx32 show a remarkable resemblance to injured nerves from wild-type mice. (uic.edu)
Cx32 KO mice with or with out the presence of your T55I mutant showed a worse phenotype than WT mice. (dna-alkylating.com)
Therefore, axons of Cx32 KO mice might have enhanced vulnerability to inflammatory tension. (dna-alkylating.com)
Despite the fact that our previous research in Cx32 KO T55I mice failed to show a dominant effect beyond the basic KO phenotype beneath regular situations [57], LPS-induced neuroinflammation affected more severely the KO T55I than the "simple" KO and WT mice. (dna-alkylating.com)
LPS induced a considerable elevation of BiP expression specially in KO T55I mice, as opposed to WT and Cx32 KO mice, even though Cx32 KO mice also showed a considerably larger ER-stress response when compared with WT mice. (dna-alkylating.com)
Nevertheless, Cx43 knock-in Cx32 (Cx43KI32) and Cx43 knock-in Cx40 (Cx43KI40) mice showed functional and morphological differences, compared to wild-type mice. (biomedcentral.com)
Deficient1
AHF generated by Wy-14,643, ciprofibrate, and a choline/methionine-deficient dietary regimen also showed decreased Cx32 expression. (nih.gov)
Gene1
The promoter region of the mouse Cx32 gene contains two putative binding sites for the transcription factor HNF-1 and consensus motifs for NF-1 as well as NFkappaB within 680bp upstream of the main transcription start site. (uthscsa.edu)
Show2
His morning radio show "En Perspectiva" has been on the air continuously since 1985. (wikipedia.org)
In June 1985 Emiliano Cotelo joined the journalists Enrique Alonso Fernández, Carlos Núñez and Claudio Paolillo, who had recently started the morning show "En Perspectiva", in Del Palacio FM station. (wikipedia.org)
Specifically2
Chronic PB treatment also resulted in a reversible decrease in Cx32 specifically in mid- and centro-lobular hepatocytes. (nih.gov)
Acta Neuropathologica Communications (2016) four:Page 14 ofretention of misfolded Cx32 in the ER top to ER-stress response specifically below inflammatory conditions, which in turn exacerbates further oligodendrocyte homeostasis. (dna-alkylating.com)