Connections between cells which allow passage of small molecules and electric current. Gap junctions were first described anatomically as regions of close apposition between cells with a narrow (1-2 nm) gap between cell membranes. The variety in the properties of gap junctions is reflected in the number of CONNEXINS, the family of proteins which form the junctions.
Direct contact of a cell with a neighboring cell. Most such junctions are too small to be resolved by light microscopy, but they can be visualized by conventional or freeze-fracture electron microscopy, both of which show that the interacting CELL MEMBRANE and often the underlying CYTOPLASM and the intervening EXTRACELLULAR SPACE are highly specialized in these regions. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p792)
Preparation for electron microscopy of minute replicas of exposed surfaces of the cell which have been ruptured in the frozen state. The specimen is frozen, then cleaved under high vacuum at the same temperature. The exposed surface is shadowed with carbon and platinum and coated with carbon to obtain a carbon replica.
Ion Channel Gating
The opening and closing of ion channels due to a stimulus. The stimulus can be a change in membrane potential (voltage-gated), drugs or chemical transmitters (ligand-gated), or a mechanical deformation. Gating is thought to involve conformational changes of the ion channel which alters selective permeability.
Zonula Occludens-1 Protein
A class of large neuroglial (macroglial) cells in the central nervous system - the largest and most numerous neuroglial cells in the brain and spinal cord. Astrocytes (from "star" cells) are irregularly shaped with many long processes, including those with "end feet" which form the glial (limiting) membrane and directly and indirectly contribute to the BLOOD-BRAIN BARRIER. They regulate the extracellular ionic and chemical environment, and "reactive astrocytes" (along with MICROGLIA) respond to injury.
Molecular Sequence Data
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Gap junction signalling mediated through connexin-43 is required for chick limb development. (1/2276)During chick limb development the gap junction protein Connexin-43 (Cx43) is expressed in discrete spatially restricted domains in the apical ectodermal ridge (AER) and mesenchyme of the zone of polarising activity. Antisense oligonucleotides (ODNs) were used to investigate the role of Connexin-43 (Cx43) in the development of the chick limb bud. We have used unmodified ODNs in Pluronic F-127 gel, which is liquid at low temperature but sets at room temperature and so remains situated at the point of application. As a mild surfactant, the gel increases antisense ODN penetration and supplies ODNs to the embryo continually for 12-18 h. We have shown a strong decrease in Cx43 protein expression after application of specific antisense oligonucleotides but the abundance of a closely related protein, Connexin-32 (Cx32), was not affected. Application of antisense Cx43 ODNs at stages 8-15 HH before limb outgrowth resulted in dramatic limb phenotypes. About 40% of treated embryos exhibited defects such as truncation of the limb bud, fragmentation into two or more domains, or complete splitting of the limb bud into two or three branches. Molecular analysis of antisense treated embryos failed to detect Shh or Bmp-2 in anterior structures and suggested that extra lobes seen in nicked and split limbs were not a result of establishment of new signalling centres as found after the application of FGF to the flank. However, examination of markers for the AER showed a number of abnormalities. In severely truncated specimens we were unable to detect the expression of either Fgf-4 or Fgf-8. In both nicked and split limbs the expression of these genes was discontinuous. Down-regulation of Cx43 after the antisense application could be comparable to AER removal and results in distal truncation of the limb bud. Taken together these data suggest the existence of a feedback loop between the FGFs and signalling mediated by Cx43. (+info)
Chemical signaling from colonic smooth muscle cells to DRG neurons in culture. (2/2276)Transduction mechanisms between target cells within the intestinal wall and peripheral terminals of extrinsic primary afferent neurons are poorly understood. The purpose of this study was to characterize the interactions between smooth muscle cells from the rat distal colon and lumbar dorsal root ganglion (DRG) neurons in coculture. DRG neurons visually appeared to make contact with several myocytes. We show that brief mechanical stimulation of these myocytes resulted in intracellular Ca2+ concentration ([Ca2+]i) transients that propagated into 57% of the contacting neurites. Direct mechanical stimulation of DRG neurites cultured without smooth muscle had no effect. We also show that colonic smooth muscle cells express multiple connexin mRNAs and that these connexins formed functional gap junctions, as evidenced by the intercellular transfer of Lucifer yellow. Furthermore, thapsigargin pretreatment and neuronal heparin injection abolished the increase in neurite [Ca2+]i, indicating that the neuronal Ca2+ signal was triggered by inositol 1,4, 5-trisphosphate-mediated Ca2+ release from intracellular stores. Our results provide evidence for intercellular chemical communication between DRG neurites and intestinal smooth muscle cells that mediates the exchange of second messenger molecules between different cell types. (+info)
Central visual, acoustic, and motor pathway involvement in a Charcot-Marie-Tooth family with an Asn205Ser mutation in the connexin 32 gene. (3/2276)BACKGROUND: X linked dominant Charcot-Marie-Tooth disease (CMT1X) is an inherited motor and sensory neuropathy that mainly affects the peripheral nervous system. CMT1X is associated with mutations in the gap junction protein connexin 32 (Cx32). Cx32 is expressed in Schwann cells and oligodendrocytes in the peripheral (PNS) and in the (CNS) respectively. METHODS: A CMT1X family with a Cx32 mutation was examined clinically and electrophysiologically to determine whether PNS, or CNS, or both pathways were affected. RESULTS: In a CMT1X family a novel mutation (Asn205Ser) was found in the fourth transmembrane domain of Cx32. The patients showed typical clinical and electrophysiological abnormalities in the PNS, but in addition visual, acoustic, and motor pathways of the CNS were affected subclinically. This was indicated by pathological changes in visually evoked potentials (VEPs), brainstem auditory evoked potentials (BAEPs), and central motor evoked potentials (CMEPs). CONCLUSIONS: These findings underscore the necessity of a careful analysis of CNS pathways in patients with CMT and Cx32 mutations. Abnormal electrophysiological findings in CNS pathway examinations should raise the suspicion of CMTX and a search for gene mutations towards Cx32 should be considered. (+info)
Upregulation of connexin 26 is a feature of keratinocyte differentiation in hyperproliferative epidermis, vaginal epithelium, and buccal epithelium. (4/2276)In epidermis, it has been suggested, intercellular communication through gap junctions is important in coordinating cell behavior. The connexins, may facilitate selective assembly or permeability of gap junctions, influencing the distribution of metabolites between cells. Using immunohistochemistry, we have compared the distribution of connexins 26 and 43 with that of proliferating cells (Ki67 labeling) in normal epidermis, hyperplastic epidermis (tape-stripped epidermis, psoriatic lesions, and viral warts), and vaginal and buccal epithelia. Connexin 43 was abundant in spinous layers of all epidermal specimens and in vaginal and buccal epithelia. Connexin 26 was absent from the interfollicular and interductal epidermis of normal hair-bearing skin, and nonlesional psoriatic epidermis but present at very low levels in plantar epidermis. Connexin 26 was prominent in lesional psoriatic epidermis and viral warts and in vaginal and buccal epithelia. In three independent experiments connexin 26 appeared in a patchy intercellular distribution in the basal epidermis within 24 h of tape stripping, proceeding to more extensive distribution in basal and suprabasal layers by 48 h. The increase in connexin 26 preceded that in cell proliferation. In vaginal epithelium, buccal epithelium, and viral warts connexin 26 was restricted mainly to suprabasal, nonproliferating cells. In psoriatic lesional epidermis connexin 26 was also located mainly in suprabasal, nonproliferating cells. Connexin 26 was present in a patchy distribution in the basal layer of psoriatic lesional epidermis, but double labeling for connexin 26 and Ki67 showed that many connexin 26 positive basal cells were nonproliferative, suggesting that connexin 26 may be related to differentiation rather than to proliferation. These observations would be consistent with a role for connexin 26 containing gap junctions during both early and later stages of keratinocyte differentiation in hyperplastic epidermis and in vaginal and buccal epithelia. (+info)
Intracellular trafficking pathways in the assembly of connexins into gap junctions. (5/2276)Trafficking pathways underlying the assembly of connexins into gap junctions were examined using living COS-7 cells expressing a range of connexin-aequorin (Cx-Aeq) chimeras. By measuring the chemiluminescence of the aequorin fusion partner, the translocation of oligomerized connexins from intracellular stores to the plasma membrane was shown to occur at different rates that depended on the connexin isoform. Treatment of COS-7 cells expressing Cx32-Aeq and Cx43-Aeq with brefeldin A inhibited the movement of these chimera to the plasma membrane by 84 +/- 4 and 88 +/- 4%, respectively. Nocodazole treatment of the cells expressing Cx32-Aeq and Cx43-Aeq produced 29 +/- 16 and 4 +/- 7% inhibition, respectively. In contrast, the transport of Cx26 to the plasma membrane, studied using a construct (Cx26/43T-Aeq) in which the short cytoplasmic carboxyl-terminal tail of Cx26 was replaced with the extended carboxyl terminus of Cx43, was inhibited 89 +/- 5% by nocodazole and was minimally affected by exposure of cells to brefeldin A (17 +/-11%). The transfer of Lucifer yellow across gap junctions between cells expressing wild-type Cx32, Cx43, and the corresponding Cx32-Aeq and Cx43-Aeq chimeras was reduced by nocodazole treatment and abolished by brefeldin A treatment. However, the extent of dye coupling between cells expressing wild-type Cx26 or the Cx26/43T-Aeq chimeras was not significantly affected by brefeldin A treatment, but after nocodazole treatment, transfer of dye to neighboring cells was greatly reduced. These contrasting effects of brefeldin A and nocodazole on the trafficking properties and intercellular dye transfer are interpreted to suggest that two pathways contribute to the routing of connexins to the gap junction. (+info)
Conduction disturbances and increased atrial vulnerability in Connexin40-deficient mice analyzed by transesophageal stimulation. (6/2276)BACKGROUND: Recently, it has been reported that connexin40 (Cx40) deficiency in targeted mouse mutants is associated with a prolongation of P-wave and QRS complex duration on surface electrograms. The specific effects of Cx40 deficiency on sinus node function, sinoatrial, and atrioventricular conduction properties as well as on atrial vulnerability have not yet been investigated systematically by electrophysiological analysis. METHODS AND RESULTS: Fifty-two mice (18 Cx40(+/+), 15 Cx40(+/-), and 19 Cx40(-/-) mice) were subjected to rapid atrial transesophageal stimulation after anesthesia with avertin. A significant prolongation of sinus node recovery time was noticed in Cx40(-/-) mice compared with Cx40(+/-) and Cx40(+/+) mice (287.8+/-109.0 vs 211.1+/-61.8 vs 204.4+/-60.9 ms; P<0.05). In addition, Wenckebach periodicity occurred at significantly longer atrial pacing cycle lengths in Cx40(-/-) mice than in Cx40(+/-) or Cx40(+/+) mice (93. 3+/-11.8 vs 83.9+/-9.7 vs 82.8+/-8.0 ms, P<0.05). Analysis of 27 Cx40(-/-) mice showed a significant increase in intra-atrial conduction time and atrioventricular conduction time compared with 52 Cx40(+/-) and 31 wild-type (Cx40(+/+)) mice. Furthermore, in Cx40(-/-) mice, atrial tachyarrhythmias could be induced frequently by atrial burst pacing, whereas no atrial arrhythmias were inducible in heterozygous or wild-type mice. CONCLUSIONS: This study demonstrates that Cx40 deficiency is associated with sinoatrial, intra-atrial, and atrioventricular conduction disturbances. In atrial myocardium of the mouse, Cx40 deficiency results in increased atrial vulnerability and might contribute to arrhythmogenesis. (+info)
Are human placental bed giant cells merely aggregates of small mononuclear trophoblast cells? An ultrastructural and immunocytochemical study. (7/2276)The ultrastructure of placental bed giant cells in early human pregnancies of 7-12 weeks gestational age is described. Their nature and function was further characterized by confocal immunofluorescence microscopy of paraffin sections labelled for cytokeratin, gap junction connexins (CX) 32 or 43, and placental hormones, alpha-human chorionic gonadotrophin (alpha-HCG) and human placental lactogen (HPL). Placental bed giant cells were observed with two phenotypes; as single large trophoblast cells containing one or more nuclear profiles in a voluminous cytoplasm, and as cell aggregates comprising mononuclear trophoblast cells in close apposition separated by narrow intercellular spaces. Cells within the aggregates are attached to one another by desmosomes, and also possess gap junctions as shown by immunolabelling for CX32 and CX43. By contrast, gap junctions were absent in the true multinucleated giant cells. Organelles present within the cytoplasm of the giant cells and their immunoreactivity for HPL and alpha-HCG suggest protein synthesis. (+info)
Functional and morphological correlates of connexin50 expressed in Xenopus laevis oocytes. (8/2276)Electrophysiological and morphological methods were used to study connexin50 (Cx50) expressed in Xenopus laevis oocytes. Oocytes expressing Cx50 exhibited a new population of intramembrane particles (9.0 nm in diameter) in the plasma membrane. The particles represented hemichannels (connexin hexamers) because (a) their cross-sectional area could accommodate 24 +/- 3 helices, (b) when their density reached 300-400/microm2, they formed complete channels (dodecamers) in single oocytes, and assembled into plaques, and (c) their appearance in the plasma membrane was associated with a whole-cell current, which was activated at low external Ca2+ concentration ([Ca2+]o), and was blocked by octanol and by intracellular acidification. The Cx50 hemichannel density was directly proportional to the magnitude of the Cx50 Ca2+-sensitive current. Measurements of hemichannel density and the Ca2+-sensitive current in the same oocytes suggested that at physiological [Ca2+]o (1-2 mM), hemichannels rarely open. In the cytoplasm, hemichannels were present in approximately 0.1-microm diameter "coated" and in larger 0.2-0.5-microm diameter vesicles. The smaller coated vesicles contained endogenous plasma membrane proteins of the oocyte intermingled with 5-40 Cx50 hemichannels, and were observed to fuse with the plasma membrane. The larger vesicles, which contained Cx50 hemichannels, gap junction channels, and endogenous membrane proteins, originated from invaginations of the plasma membrane, as their lumen was labeled with the extracellular marker peroxidase. The insertion rate of hemichannels into the plasma membrane (80, 000/s), suggested that an average of 4,000 small coated vesicles were inserted every second. However, insertion of hemichannels occurred at a constant plasma membrane area, indicating that insertion by vesicle exocytosis (60-500 microm2 membranes/s) was balanced by plasma membrane endocytosis. These exocytotic and endocytotic rates suggest that the entire plasma membrane of the oocyte is replaced in approximately 24 h. (+info)
Connexins are commonly named according to their molecular weights, e.g. Cx26 is the connexin protein of 26 kDa. A competing ... Media related to connexins at Wikimedia Commons Connexins at the US National Library of Medicine Medical Subject Headings (MeSH ... Kamasawa N, Sik A, Morita M, Yasumura T, Davidson KG, Nagy JI, Rash JE (2005). "Connexin-47 and connexin-32 in gap junctions of ... Aronica E, Gorter JA, Jansen GH, Leenstra S, Yankaya B, Troost D (May 2001). "Expression of connexin 43 and connexin 32 gap- ...
Charcot-Marie-Tooth disease classifications
Abrams, Charles K.; Rash, John E. (2009). "Connexins in the Nervous System". In Harris, Andrew; Locke, Darren (eds.). Connexins ...
How the connexins may be transported to the plaques using tubulin is becoming clearer. The formation plaque and non-connexin ... By study of connexins still in membranes lipids associated with the connexins have been studied. It was found that specific ... Connexin proteins expressed in neuronal gap junctions include: mCX36 mCX57 mCX45 with mRNAs for at least five other connexins ( ... Before innexins and connexins were well characterized, the genes coding for connexin gap junction channels were classified in ...
This gene is a member of the connexin gene family. The encoded protein is a component of gap junctions, which are composed of ... 2003). "Comparison of connexin 43, 40 and 45 expression patterns in the developing human and mouse hearts". Cell Commun. Adhes ... Gap junction gamma-1 protein (GJC1), also known as gap junction alpha-7 protein (GJA7) and connexin 45 (Cx45) - is a protein ... "Entrez Gene: GJA7 gap junction protein, alpha 7, 45kDa". Andrew L Harris; Darren Locke (2009). Connexins, A Guide. New York: ...
2004). "Molecular interaction of connexin 30.3 and connexin 31 suggests a dominant-negative mechanism associated with ... Gap junction beta-4 protein (GJB4), also known as connexin 30.3 (Cx30.3) - is a protein that in humans is encoded by the GJB4 ... "Entrez Gene: GJB4 gap junction protein, beta 4". Andrew L Harris; Darren Locke (2009). Connexins, A Guide. New York: Springer. ... 2000). "Mutation in the gene for connexin 30.3 in a family with erythrokeratodermia variabilis". Am. J. Hum. Genet. 67 (5): ...
2004). "Molecular interaction of connexin 30.3 and connexin 31 suggests a dominant-negative mechanism associated with ... This gene is a member of the connexin gene family. The encoded protein is a component of gap junctions, which are composed of ... Gap junction beta-3 protein (GJB3), also known as connexin 31 (Cx31) - is a protein that in humans is encoded by the GJB3 gene ... 2001). "Connexin 31 (GJB3) is expressed in the peripheral and auditory nerves and causes neuropathy and hearing impairment". ...
Connexins are membrane-spanning proteins that assemble to form gap junction channels that facilitate the transfer of ions and ... Most of the mutations of the GJB1 gene switch or change a single amino acid in the gap junction (connexin-32) protein, although ... Gap junction beta-1 protein (GJB1), also known as connexin 32 (Cx32) is a transmembrane protein that in humans is encoded by ... Gap junction beta-1 protein is a member of the gap junction connexin family of proteins that regulates and controls the ...
Connexin 30 has been found to be co-localized with connexin 26. Cx30 and Cx26 have also been found to form heteromeric and ... Connexin 26 and connexin 30 are commonly accepted to be the predominant gap junction proteins in the cochlea. Genetic knockout ... Connexins span the plasma membrane 4 times, with amino- and carboxy-terminal regions facing the cytoplasm. Connexin genes are ... Erbe CB, Harris KC, Runge-Samuelson CL, Flanary VA, Wackym PA (April 2004). "Connexin 26 and connexin 30 mutations in children ...
Connexons are formed by six 7.5 nm long, four-pass membrane-spanning protein subunits called connexins, which may be identical ... Andrew L. Harris; Darren Locke (2009). Connexins, a guide. New York: Springer. p. 574. ISBN 978-1-934115-46-6. Haas, Julie S.; ...
The connexin-43 internal ribosome entry site is an RNA element present in the 5' UTR of the mRNA of GJA1. This internal ... Gap junction alpha-1 protein (GJA1), also known as connexin 43 (Cx43), is a protein that in humans is encoded by the GJA1 gene ... Page for Connexin-43 internal ribosome entry site (IRES) at Rfam (Articles with short description, Short description matches ... GJA1 is the most ubiquitously expressed connexin and is detected in most cell types. It is the major protein in heart gap ...
1998). "Assembly of connexins and MP26 in lens fiber plasma membranes studied by SDS-fracture immunolabeling". J. Cell Sci. 111 ... 2002). "Multimeric connexin interactions prior to the trans-Golgi network". J. Cell Sci. 114 (Pt 22): 4013-24. doi:10.1242/jcs. ... 2004). "Lens connexins alpha3Cx46 and alpha8Cx50 interact with zonula occludens protein-1 (ZO-1)". Mol. Biol. Cell. 14 (6): ... Andrew L Harris; Darren Locke (2009). Connexins, A Guide. New York: Springer. p. 574. ISBN 978-1-934115-46-6. Hsieh CL, Kumar ...
This is possible due to six connexin proteins interacting to form a cylinder with a pore in the centre called a connexon. The ... Andrew L Harris; Darren Locke (2009). Connexins, A Guide. New York: Springer. p. 574. ISBN 978-1-934115-46-6. Yan HH, Mruk DD, ... Wei CJ, Xu X, Lo CW (2004). "Connexins and cell signaling in development and disease". Annual Review of Cell and Developmental ... Connexon pores vary in size, polarity and therefore can be specific depending on the connexin proteins that constitute each ...
... , also called connexin-36 (CX36), is a member of the connexin gene family that is expressed predominantly in mammalian ... Connexins associate in groups of 6 and are organized radially around a central pore to form connexons. Each gap junction ... Gap junction delta-2 protein (GJD2) also known as connexin-36 (Cx36) or gap junction alpha-9 protein (GJA9) is a protein that ... "Entrez Gene: GJA9 gap junction protein, alpha 9, 36kDa". Andrew L Harris; Darren Locke (2009). Connexins, A Guide. New York: ...
Structurally, pannexins and connexins are very similar, consisting of 4 transmembrane domains, 2 extracellular and 1 ... Probenecid does not affect channels formed by connexins, but it inhibits pannexin-1 channels. Panchin Y, Kelmanson I, Matz M, ... Andrew L Harris & Darren Locke (2009). Connexins, A Guide. New York: Springer. p. 574. ISBN 978-1-934115-46-6. (Protein pages ... Probenecid, a well-established drug for the treatment of gout, allows for discrimination between channels formed by connexins ...
Connexin types can be further differentiated by using their predicted molecular weight (ex: Connexin 43 is Cx 43 due to its ... In biology, a connexon, also known as a connexin hemichannel, is an assembly of six proteins called connexins that form the ... Connexons made of the same type of connexins are considered homomeric, while connexons made of differing types of connexins are ... forming a hemi-channel that is composed of connexins. Connexins are the smaller protein molecules that make up connexons and ...
Proteins, called connexins, purified from fractions of enriched gap junctions from different tissues differ. The connexins are ... Connexin 26 also plays a role in tumor suppression through mediation of the cell cycle. The abnormal expression of Cx26, ... Gap junction beta-2 protein (GJB2), also known as connexin 26 (Cx26) - is a protein that in humans is encoded by the GJB2 gene ... Kelsell DP, Dunlop J, Stevens HP, Lench NJ, Liang JN, Parry G, Mueller RF, Leigh IM (May 1997). "Connexin 26 mutations in ...
1998). "Assembly of connexins and MP26 in lens fiber plasma membranes studied by SDS-fracture immunolabeling". J. Cell Sci. 111 ... Rong P, Wang X, Niesman I, Wu Y, Benedetti LE, Dunia I, Levy E, Gong X (January 2002). "Disruption of Gja8 (alpha8 connexin) in ... 2007). "Connexin 50 gene on human chromosome 1q21 is associated with schizophrenia in matched case control and family-based ... 2004). "Lens connexins alpha3Cx46 and alpha8Cx50 interact with zonula occludens protein-1 (ZO-1)". Mol. Biol. Cell. 14 (6): ...
Harris AL, Locke D (2009). Connexins, a guide. New York: Springer. p. 574. ISBN 978-1-934115-46-6. Haas JS, Zavala B, Landisman ...
Pannexins or Connexins? Chapter 12. In: A. Harris, D. Locke (eds.), Connexins: A Guide doi:10.1007/978-1-59745-489-6_12 Bao L, ... While the connexin family of gap junction proteins was well-characterized in vertebrates, no homologues were found in non- ... Structurally, innexins and connexins are very similar, consisting of 4 transmembrane domains, 2 extracellular and 1 ... Gap junction proteins with no sequence homology to connexins were initially identified in fruit flies. It was suggested that ...
This gene is a member of the connexin gene family. The encoded protein is a component of gap junctions, which are composed of ... Gap junction alpha-5 protein (GJA5), also known as connexin 40 (Cx40) - is a protein that in humans is encoded by the GJA5 gene ... 2003). "Comparison of connexin 43, 40 and 45 expression patterns in the developing human and mouse hearts". Cell Commun. Adhes ... 2002). "Connexin expression in Huntington's diseased human brain". Cell Biol. Int. 22 (11-12): 837-47. doi:10.1006/cbir. ...
This protein, like other Connexin proteins, forms connections between cells known as gap junctions. Connexin 37 can be found in ... Gap junction alpha-4 protein, also known as Connexin-37 or Cx37, is a protein that in humans is encoded by the GJA4 gene. ... Willecke K, Jungbluth S, Dahl E, Hennemann H, Heynkes R, Grzeschik KH (December 1990). "Six genes of the human connexin gene ... Andrew L Harris; Darren Locke (2009). Connexins, A Guide. New York: Springer. p. 574. ISBN 978-1-934115-46-6. Beyer EC, Paul DL ...
Retamal MA (2014). "Connexin and Pannexin hemichannels are regulated by redox potential". Frontiers in Physiology. 5: 80. doi: ... Hemichannels consist of connexins. Pannexins are involved in the process of purinergic signalling. They release adenosine ... Spray DC, Ye ZC, Ransom BR (November 2006). "Functional connexin "hemichannels": a critical appraisal". Glia. 54 (7): 758-73. ... and opens both connexin and pannexin channels, therefore contributing to the propagation of calcium waves across astrocytes and ...
The abnormal distribution of gap junction proteins such as GJA1 (also known as Connexin 43), and GJA5 (Connexin 40) causes non- ... Saffitz JE (2006). "Connexins, conduction, and atrial fibrillation". N. Engl. J. Med. 354 (25): 2712-14. doi:10.1056/ ... Gollob MH, Jones DL, Krahn AD (2006). "Somatic mutations in the connexin 40 gene (GJA5) in atrial fibrillation". New England ...
Integrins and connexins are markedly up-regulated. A key role in this concerted action, which leads to a 2-to-20 fold increase ...
Gap junction modulation
Nitrosylation can either be induced on the connexin proteins or proteins that further regulate connexin such as kinases. The ... The gap junction protein connexin generally possesses a number of phosphorylation sites (connexin Cx43 has 21). The binding of ... positively charged connexin close with hyperpolarization and negatively charged connexins close with depolarization. Other than ... The pH sensitivity depends on the type of connexin composing the gap junction, but the channels generally close at a pH of 6.4- ...
Hereditary mucoepithelial dysplasia
Lampe, P.; Lau, A. F. (Dec 2000). "Regulation of gap junctions by phosphorylation of connexins". Archives of Biochemistry and ... Lampe, P. D.; Lau, A. F. (Jul 2004). "The effects of connexin phosphorylation on gap junctional communication". The ...
Kandouz, Mustapha; Batist, Gerald (2010). "Gap junctions and connexins as therapeutic targets in cancer". Expert Opinion on ... White, T. W (2002). "Unique and redundant connexin contributions to lens development". Science. 295 (5553): 319-20. Bibcode: ... "Roles for alpha 1 connexin in morphogenesis of chick embryos revealed using a novel antisense approach". Developmental Genetics ... is accompanied by down-regulation of gap junctional intercellular communication and translocation of connexin 43 in NIH3T3 ...
... acts at connexins, preferentially to connexin 43 (Cx43). Treatment with rotigaptide has been shown to activate ... Each connexon is made up of 6 functional units (connexins) that associate together to form a channel between adjacent cells. ... increases gap junction intercellular communication in cardiac myocytes and HeLa cells expressing connexin 43. British Journal ...
These gap junctions are made of proteins called connexins. There are fewer gap junctions within the SA node and they are ...
... innexin connexin pannexin Beyer, EC; Berthoud, VM (27 May 2017). "Gap junction gene and protein families: Connexins, innexins, ... like the vertebrate connexin gap junction protein, vinnexin subunits assemble together to form a channel in the plasma membrane ...
Connexins in Prostate Cancer Initiation and Progression
A retina réskapcsolatait alkotó connexin fehérjék expresszióját befolyásoló tényezők gerinces állatokban
Mice lacking connexin40 have cardiac conduction abnormalities characteristic of atrioventricular block and bundle branch block
Deafness - Genes and Disease - NCBI Bookshelf
... but the most common cause of hearing loss in American and European populations is a mutation in the connexin 26 (Cx26) gene. ... Connexin 26. Connexin 26 (GJB2) is one of the main proteins involved in potassium (K+) homeostasis in the cochlea of the inner ... Cx26 is located on chromosome 13q11-12 and codes for a gap junction protein called connexin 26. Gap junctions are plasma ... but the most common cause of hearing loss in American and European populations is a mutation in the connexin 26 (Cx26) gene. ...
GJA1 gene: MedlinePlus Genetics
... which is one of 21 connexin proteins. Learn about this gene and related health conditions. ... The GJA1 gene provides instructions for making a protein called connexin 43, ... The GJA1 gene provides instructions for making a protein called connexin 43, which is one of 21 connexin proteins. Connexins ... Connexin 43 is found in many tissues such as the eyes, skin, bone, ears, heart, and brain, and it plays a role in their normal ...
About Genetics and Hearing Loss | A Parent's Guide to Genetics and Hearing Loss
Human immunodeficiency virus-1/simian immunodeficiency virus infection induces opening of pannexin-1 channels resulting in...
Frontiers | Brugada Syndrome: Warning of a Systemic Condition?
MESH TREE NUMBER CHANGES - 2008 MeSH
3. Connexins and their channels in cell growth and cell death.. Vinken M; Vanhaecke T; Papeleu P; Snykers S; Henkens T; Rogiers ... 9. [The connexin family, gap junctional intercellular communication and tumor suppression].. Zhang ZQ; Lin ZX. Sheng Li Ke Xue ... Connexins in leukocytes: shuttling messages?. Wong CW; Christen T; Kwak BR. Cardiovasc Res; 2004 May; 62(2):357-67. PubMed ID: ... 8. Gap junctions and connexins as therapeutic targets in cancer.. Kandouz M; Batist G. Expert Opin Ther Targets; 2010 Jul; 14(7 ...
Vohwinkel Syndrome: Practice Essentials, Pathophysiology, Etiology
One is a missense mutation of the GJB2 gene coding connexin-26, a gap junction protein. [12, 13, 14, 15, 16] This mutation on ... Connexin mutations associated with palmoplantar keratoderma and profound deafness in a single family. Eur J Hum Genet. 2000 Jun ... Connexins are building blocks of gap junctions that are plasma membrane complexes facilitating and regulating the passage of ... Connexins 26, 30, 30.3, 31, and 43 are related to cutaneous diseases associated with multiple organ involvement. Mutations in ...
NIH Guide: ISCHEMIC HEART DISEASE IN BLACKS
Dandy-walker Syndrome: Disease Bioinformatics: Novus Biologicals
Connexins Preferred Term Term UI T052822. Date01/01/1999. LexicalTag NON. ThesaurusID NLM (1994). ... Connexins Preferred Concept UI. M0026696. Registry Number. 0. Scope Note. A group of homologous proteins which form the ... Connexin Term UI T000999743. Date11/29/2019. LexicalTag NON. ThesaurusID NLM (2021). ... Connexin Gap Junction Channel Proteins Gap Junction Protein Gap Junction Proteins Registry Number. 0. Previous Indexing. ...
Combettes - Google Scholar
Real Estate Investment Strategy | PATRIZIA SE
Alumni | National Institutes of Health Oxford-Cambridge Scholars Program
NIDCD Working Group Considerations for Developing and Implementing Genetic Diagnostic Tests for Hereditary Hearing Impairment...
Mechanisms of gap junction traffic in health and disease<...
Pesquisa | Biblioteca Virtual em Saúde - BRASIL
The average optical density of claudin-11, zonal occludin-1 (ZO-1), and connexin 43 (Cx43) decreased significantly in the Cd ... connexin 43; BTB: blood-testis barrier; MAPKs: mitogen-activated protein kinases; OSP: oligodendrocyte-specific protein; Cxs: ... connexins; GJIC: gap junctional intercellular communication; ROS: reactive oxygen species; MDA: malondialdehyde; TGF: tumor ...
A gap junction beta subunit that forms heteromeric hemichannels when paired with alpha subunits such as connexin-40 or CONNEXIN ... A gap junction beta subunit that forms heteromeric hemichannels when paired with alpha subunits such as connexin-40 or CONNEXIN ... Mutations in the connexin 30 gene (GJ6B) are associated with CLOUSTONS SYNDROME and some hereditary forms of deafness.. ... Mutations in the connexin 30 gene (GJ6B) are associated with CLOUSTONS SYNDROME and some hereditary forms of deafness. ...
Cardiovascular Medicine Research | Internal Medicine | IU School of Medicine
A gap junction beta subunit that forms heteromeric hemichannels when paired with alpha subunits such as connexin-40 or CONNEXIN ... A gap junction beta subunit that forms heteromeric hemichannels when paired with alpha subunits such as connexin-40 or CONNEXIN ... Connexin 30 Preferred Term Term UI T000921776. Date04/17/2017. LexicalTag NON. ThesaurusID NLM (2018). ... Connexins (1996-2017). Public MeSH Note. 2018. History Note. 2018. Date Established. 2018/01/01. Date of Entry. 2017/07/11. ...
- Gap junctions GJ are conglomerations of cell-cell channels that are formed by a family of 21 distinct proteins, called connexin Cxs. (dtic.mil)
- Studies of the expression of connexins - the family of proteins from which gap junctions are formed - reveal that connexin40 (Cx40) is prominent in the conduction system . (nih.gov)
- Connexins play a role in cell-to-cell communication by forming channels, or gap junctions, between cells. (medlineplus.gov)
- It is unclear how a shortage of connexin 43 at the cell surface affects the structure of gap junctions in the outermost layer of skin (epidermis), or how these changes result in the skin abnormalities characteristic of EKVP. (medlineplus.gov)
- Gap junctions formed with abnormal connexin 43 proteins are often permanently closed, preventing the transport of any molecules. (medlineplus.gov)
- Some mutations prevent connexin 43 proteins from traveling to the cell surface where they are needed to form gap junctions. (medlineplus.gov)
- 8. Gap junctions and connexins as therapeutic targets in cancer. (nih.gov)
- So the shape of the cellular processes is preserved, and we can see important features like vesicles and post-synaptic densities and connexins (although they didn't find any gap junctions in this sample), but physiological and pharmacological information is only inferred. (scienceblogs.com)
- Connexins are a group of membrane proteins that oligomerize to construct gap junctions protein. (genominfo.org)
- Connexin 26 (GJB2) is one of the main proteins involved in potassium (K+) homeostasis in the cochlea of the inner ear. (nih.gov)
- The GJA1 gene provides instructions for making a protein called connexin 43, which is one of 21 connexin proteins. (medlineplus.gov)
- The connexins are a group of membrane proteins that form connexon in the cell membrane [ 6 ]. (genominfo.org)
- Two connexons, each containing six connexin proteins, link across the extracellular space to form a gap junction channel. (genominfo.org)
- Cx26 is located on chromosome 13q11-12 and codes for a gap junction protein called connexin 26. (nih.gov)
- It changes a single protein building block (amino acid) in the connexin 43 protein: at position 239, the amino acid arginine is replaced with the amino acid glutamine (written as Arg239Gln or R239Q). (medlineplus.gov)
- It is unknown how this change affects the function of the connexin 43 protein or leads to the bone abnormalities characteristic of craniometaphyseal dysplasia. (medlineplus.gov)
- Each of the known mutations changes a single amino acid in the connexin 43 protein. (medlineplus.gov)
- 8. Leukemogenic AML1-ETO fusion protein upregulates expression of connexin 43: the role in AML 1-ETO-induced growth arrest in leukemic cells. (nih.gov)
- The GJC2 sequence encodes connexin 47 protein (Cx47). (genominfo.org)
- Connexin 47 (Cx47, GenBank NP 065168.2) is encoded by the GJC2 gene and is a member of a highly conserved protein family of connexins [ 4 , 5 ]. (genominfo.org)
- Many deafness genes exist, but the most common cause of hearing loss in American and European populations is a mutation in the connexin 26 ( Cx26 ) gene. (nih.gov)
- The degree of hearing loss in persons with Connexin 26-related deafness varies from moderate to profound, and it appears that there may be a variable time of initial expression as well as a variable rate of progression. (nih.gov)
- Mutations in the connexin 30 gene (GJ6B) are associated with CLOUSTON'S SYNDROME and some hereditary forms of deafness. (bvsalud.org)
- 9. [The connexin family, gap junctional intercellular communication and tumor suppression]. (nih.gov)
- I aim to determine the role of NSC-EC gap junctional interactions via connexin 43 in the regulation of NSC behavior in vivo and in vitro under normal brain homeostasis conditions and in an injury-induced neuro-regeneration model. (virginia.edu)
- A gap junction beta subunit that forms heteromeric hemichannels when paired with alpha subunits such as connexin-40 or CONNEXIN 43. (bvsalud.org)
- Most of these mutations change an amino acid in connexin 43. (medlineplus.gov)
- 3. Connexins and their channels in cell growth and cell death. (nih.gov)
- In addition, connexin 43 attaches (binds) several signaling molecules that can relay communication signals inside the cell. (medlineplus.gov)
- Connexin 43 is found in many tissues such as the eyes, skin, bone, ears, heart, and brain, and it plays a role in their normal development and function. (medlineplus.gov)
- Mutation analysis of the connexin 26 gene revealed that she carried a paternally inherited mutation, p.Asp46Glu and a maternally inherited M34T variant. (nih.gov)
- The GJB4 gene provides instructions for making a protein called gap junction beta 4, more commonly known as connexin 30.3. (medlineplus.gov)
- Sbidian E, Bousseloua N, Jonard L, Leclerc-Mercier S, Bodemer C, Hadj-Rabia S. Novel mutation in GJB4 gene (connexin 30.3) in a family with erythrokeratodermia variabilis. (medlineplus.gov)
- 14. Enhancing effect of connexin 32 gene on vinorelbine-induced cytotoxicity in A549 lung adenocarcinoma cells. (nih.gov)
- The connexins are the products of an identified gene family which has both highly conserved and highly divergent regions. (bvsalud.org)
- Each of these mutations changes a single protein building block (amino acid) in connexin 30.3. (medlineplus.gov)
- It changes a single protein building block (amino acid) in the connexin 43 protein: at position 239, the amino acid arginine is replaced with the amino acid glutamine (written as Arg239Gln or R239Q). (nih.gov)
- Each of the known mutations changes a single amino acid in the connexin 43 protein. (nih.gov)
- Most of these mutations change an amino acid in connexin 43. (nih.gov)
- Here we used single-particle cryo-electron microscopy to investigate the structural basis of connexin co-assembly in native lens gap junction channels composed of connexin 46 and connexin 50 (Cx46/50). (nih.gov)
- Yoshikata-Isokawa Y, Itoh M, Nakagawa H. Japanese sporadic case of erythrokeratodermia variabilis caused by the connexin-30.3 (GJB4) mutation: Is Glycine 12 a mutational hotspot in the connexin family? (medlineplus.gov)