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Shoichi Suzuki, Masahiro Ogawa, Shoichiro Ohta, Satoshi Nunomura, Yasuhiro Nanri, Hiroshi Shiraishi, Yasutaka Mitamura, Tomohito Yoshihara, James J Lee, Kenji Izuhara
Hypothiocyanite (OSCN(-)) serves as a potent innate defense system against microbes in the lungs. OSCN(-) is generated by the catalysis of peroxidases using thiocyanate transported via several anion transporters, including pendrin/SLC26A4 and hydrogen peroxide (H2O2) generated by Duox1 and Duox2. We previously demonstrated that expression of pendrin, peroxidases, and Duox1/Duox2 is upregulated in bronchial asthma patients and/or asthma model mice and that these molecules are important in accelerating airway inflammation...
November 18, 2016: Journal of Biological Chemistry
Axel Wolf, Alexandra Frohne, Matthew Allen, Thomas Parzefall, Martin Koenighofer, Markus M Schreiner, Christian Schoefer, Klemens Frei, Trevor Lucas
BACKGROUND: Heterozygous mutations in GJB2 (MIM: 121011) encoding the gap junction protein connexin 26 are overrepresented in patient groups suffering from nonsyndromic sensorineural hearing impairment (HI) implying the involvement of additional genetic factors. Mutations in SLC26A4 (MIM: 605646), encoding the protein pendrin can cause both Pendred syndrome and autosomal recessive, nonsyndromic HI locus 4 type sensorineural HI (MIM: 600791). OBJECTIVES: Aim of this study was to investigate the role of SLC26A4 coding mutations in a nonsyndromic hearing impairment (NSHI) patient group bearing heterozygous GJB2 35delG mutations...
November 17, 2016: Otology & Neurotology
Nozomu Mori, Takenori Miyashita, Ryuhei Inamoto, Ai Matsubara, Terushige Mori, Kosuke Akiyama, Hiroshi Hoshikawa
Ion transport and its regulation in the endolymphatic sac (ES) are reviewed on the basis of recent lines of evidence. The morphological and physiological findings demonstrate that epithelial cells in the intermediate portion of the ES are more functional in ion transport than those in the other portions. Several ion channels, ion transporters, ion exchangers, and so on have been reported to be present in epithelial cells of ES intermediate portion. An imaging study has shown that mitochondria-rich cells in the ES intermediate portion have a higher activity of Na(+), K(+)-ATPase and a higher Na(+) permeability than other type of cells, implying that molecules related to Na(+) transport, such as epithelial sodium channel (ENaC), Na(+)-K(+)-2Cl(-) cotransporter 2 (NKCC2) and thiazide-sensitive Na(+)-Cl(-) cotransporter (NCC), may be present in mitochondria-rich cells...
November 1, 2016: European Archives of Oto-rhino-laryngology
Nancy Schanze, Simon Friedrich Jacobi, Eddy Rijntjes, Stefan Mergler, Marta Del Olmo, Carolin S Hoefig, Noushafarin Khajavi, Ina Lehmphul, Heike Biebermann, Jens Mittag, Josef Koehrle
BACKGROUND: 3-Iodothyronamine (3-T1AM) is an endogenous decarboxylated thyroid hormone (TH) metabolite. Pharmacological doses of 3-T1AM decrease heart rate, body temperature and metabolic rate in rodents - effects that are contrary to classic TH excess. Furthermore, a single dose of 3-T1AM was shown to suppress the hypothalamic-pituitary-thyroid (HPT) axis in rats. We hypothesize that 3-T1AM might play a role in the fine tuning of TH action and might have a direct regulatory effect on the thyroid gland...
October 27, 2016: Thyroid: Official Journal of the American Thyroid Association
Claudio Bassot, Giovanni Minervini, Emanuela Leonardi, Silvio C E Tosatto
Human pendrin (SLC26A4) is an anion transporter mostly expressed in the inner ear, thyroid and kidney. SLC26A4 gene mutations are associated with a broad phenotypic spectrum, including Pendred Syndrome and non-syndromic hearing loss with enlarged vestibular aqueduct (ns-EVA). No experimental structure of pendrin is currently available, making phenotype-genotype correlations difficult as predictions of transmembrane (TM) segments vary in number. Here, we propose a novel three-dimensional (3D) pendrin transmembrane domain model based on the SLC26Dg transporter...
October 19, 2016: Biochimie
Zineb Eskalli, Younes Achouri, Stephan Hahn, Marie-Christine Many, Julie Craps, Samuel Refetoff, Xiao-Hui Liao, Jacques E Dumont, Jacqueline Van Sande, Bernard Corvilain, Françoise Miot, Xavier De Deken
BACKGROUND: The dual oxidases (Duox) are involved in hydrogen peroxide generation, which is essential for thyroid hormone synthesis, and therefore they are markers of thyroid function. During inflammation, cytokines upregulate DUOX gene expression in the airway and the intestine, suggesting a role for these proteins in innate immunity. It was previously demonstrated that interleukin-4 (IL-4) upregulates DUOX gene expression in thyrocytes. Although the role of IL-4 in autoimmune thyroid diseases has been studied extensively, the effects of IL-4 on thyroid physiology remain largely unknown...
October 2016: Thyroid: Official Journal of the American Thyroid Association
Carsten A Wagner
No abstract text is available yet for this article.
December 2016: Journal of the American Society of Nephrology: JASN
Jamile Calil-Silveira, Caroline Serrano-Nascimento, Raquel Cardoso Laconca, Letícia Schmiedecke, Rafael Barrera Salgueiro, Ayrton Kimidi Kondo, Maria Tereza Nunes
BACKGROUND: Iodine is essential for thyroid hormone synthesis and is an important regulator of thyroid function. Chronic iodine deficiency leads to hypothyroidism, but iodine excess also impairs thyroid function causing hyperthyroidism, hypothyroidism, and/or thyroiditis. This study aimed to investigate the underlying mechanisms by which exposure to chronic iodine excess impairs pituitary-thyroid axis function. METHODS: Male Wistar rats were treated for two months with NaI (0...
October 2016: Thyroid: Official Journal of the American Thyroid Association
Manoocher Soleimani, Sharon Barone, Jie Xu, Saeed Alshahrani, Marybeth Brooks, Francis X McCormack, Roger D Smith, Kamyar Zahedi
Contribution of salt wasting and volume depletion to the pathogenesis of hypercalciuria and hyperphosphaturia is poorly understood. Pendrin/NCC double KO (pendrin/NCC-dKO) mice display severe salt wasting under basal conditions and develop profound volume depletion, prerenal renal failure, and metabolic alkalosis and are growth retarded. Microscopic examination of the kidneys of pendrin/NCC-dKO mice revealed the presence of calcium phosphate deposits in the medullary collecting ducts, along with increased urinary calcium and phosphate excretion...
2016: PloS One
Marian Y Hu, Katharina Michael, Cornelia M Kreiss, Meike Stumpp, Sam Dupont, Yung-Che Tseng, Magnus Lucassen
CO2-driven seawater acidification has been demonstrated to enhance intestinal bicarbonate secretion rates in teleosts, leading to an increased release of CaCO3 under simulated ocean acidification scenarios. In this study, we investigated if increasing CO2 levels stimulate the intestinal acid-base regulatory machinery of Atlantic cod (Gadus morhua) and whether temperatures at the upper limit of thermal tolerance stimulate or counteract ion regulatory capacities. Juvenile G. morhua were acclimated for 4 weeks to three CO2 levels (550, 1200, and 2200 μatm) covering present and near-future natural variability, at optimum (10°C) and summer maximum temperature (18°C), respectively...
2016: Frontiers in Physiology
Fengguo Zhang, Xiaohui Bai, Yun Xiao, Xue Zhang, Guodong Zhang, Jianfeng Li, Lei Xu, Haibo Wang
OBJECTIVE: To investigate the genetic causes of hearing loss in a two generation Chinese family with enlarged vestibular aqueduct syndrome (EVAS). METHODS: Clinical and genetic evaluations were conducted in a deaf proband and her normal-hearing parents. Sanger sequencing analysis of all the 21 exons, the exon-intron boundaries and the promoter in SLC26A4 gene was performed to detect the pathogenic mutations. PCR-restricted fragment length polymorphism (PCR-RFLP) was used to further identify the mutation...
June 2016: International Journal of Pediatric Otorhinolaryngology
Emanuele Bernardinelli, Roberta Costa, Charity Nofziger, Markus Paulmichl, Silvia Dossena
BACKGROUND/AIMS: Pendrin is a Cl-/I-/HCO3- exchanger playing a fundamental role in controlling blood pressure and airway function, therefore representing an attractive target for the treatment of hypertensive states and respiratory distresses. A review of the literature regarding the ability of some compounds (namely several known inhibitors of ion transport) to block pendrin activity revealed discordant findings. These incongruous findings may be due, in part, to the concentration of compound and/or the nature of the model system used in the study...
2016: Cellular Physiology and Biochemistry
Chun-E Jia, Dingyuan Jiang, Huaping Dai, Fei Xiao, Chen Wang
OBJECTIVE: The aim of this study is to evaluate the role of pendrin in acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) and to explore whether pendrin expression existing on alveolar cells. METHODS: ALI C57BL/6 mice model induced by lipopolysaccharide (LPS) was established. The expression of pendrin in lung was analyzed by RT-PCR and western blotting methods, the changes of lung inflammatory parameters and pathology were observed, the cellular distribution of pendrin in the lung was determined using immunofluorescence...
2016: American Journal of Translational Research
Ayako Nishio, Taku Ito, Hui Cheng, Tracy S Fitzgerald, Philine Wangemann, Andrew J Griffith
SLC26A4 mutations cause fluctuating and progressive hearing loss associated with enlargement of the vestibular aqueduct (EVA). SLC26A4 encodes a transmembrane anion exchanger called pendrin expressed in nonsensory epithelial cells of the lateral wall of cochlea, vestibular organs and endolymphatic sac. We previously described a transgenic mouse model of EVA with doxycycline (dox)-inducible expression of Slc26a4 in which administration of dox from conception to embryonic day 17.5 (DE17.5) resulted in hearing fluctuation between 1 and 3months of age...
August 4, 2016: Neuroscience
Onur Cil, Peter M Haggie, Puay-Wah Phuan, Joseph-Anthony Tan, Alan S Verkman
Pendrin is a Cl(-)/HCO3(-) exchanger expressed in type B and non-A, non-B intercalated cells in the distal nephron, where it facilitates Cl(-) absorption and is involved in Na(+) absorption and acid-base balance. Pendrin-knockout mice show no fluid-electrolyte abnormalities under baseline conditions, although mice with double knockout of pendrin and the Na(+)/Cl(-) cotransporter (NCC) manifest profound salt wasting. Thus, pendrin may attenuate diuretic-induced salt loss, but this function remains unconfirmed...
December 2016: Journal of the American Society of Nephrology: JASN
Anne Sinning, Nikita Radionov, Francesco Trepiccione, Karen I López-Cayuqueo, Maximilien Jayat, Stéphanie Baron, Nicolas Cornière, R Todd Alexander, Juliette Hadchouel, Dominique Eladari, Christian A Hübner, Régine Chambrey
We recently described a novel thiazide-sensitive electroneutral NaCl transport mechanism resulting from the parallel operation of the Cl(-)/HCO3 (-) exchanger pendrin and the Na(+)-driven Cl(-)/2HCO3 (-) exchanger (NDCBE) in β-intercalated cells of the collecting duct. Although a role for pendrin in maintaining Na(+) balance, intravascular volume, and BP is well supported, there is no in vivo evidence for the role of NDCBE in maintaining Na(+) balance. Here, we show that deletion of NDCBE in mice caused only subtle perturbations of Na(+) homeostasis and provide evidence that the Na(+)/Cl(-) cotransporter (NCC) compensated for the inactivation of NDCBE...
May 5, 2016: Journal of the American Society of Nephrology: JASN
Jinsei Jung, Jiyoon Kim, Shin Hye Roh, Ikhyun Jun, Robert D Sampson, Heon Yung Gee, Jae Young Choi, Min Goo Lee
Mutations in SLC26A4, which encodes pendrin, are responsible for hearing loss with an enlarged vestibular aqueduct and Pendred syndrome. The most prevalent mutation in East Asia is p.H723R (His723Arg), which leads to defects in protein folding and cell-surface expression. Here we show that H723R-pendrin can be rescued to the cell surface by an HSP70 co-chaperone DNAJC14-dependent unconventional trafficking pathway. Blockade of ER-to-Golgi transport or activation of ER stress signals induced Golgi-independent cell-surface expression of H723R-pendrin and restored its cell-surface Cl(-)/HCO3(-) exchange activity...
2016: Nature Communications
Bo Gyung Kim, Tae-Hyun Yoo, Ji Eun Yoo, Young Joon Seo, Jinsei Jung, Jae Young Choi
Pendrin is a membrane transporter encoded by solute carrier family26A4 (SLC26A4). Mutations in this gene are known to cause hearing loss, and recent data from animal studies indicate a link between pendrin expression and hypertension, although this association in humans is unclear. To clarify this issue, we investigated the influence of pendrin on blood pressure by analyzing demographic and biochemical data-including blood pressure and urinary electrolyte excretion-in patients with bi-allelic SLC26A4 mutations...
April 19, 2016: Clinical Genetics
Mina Patel-Chamberlin, Mujan Varasteh Kia, Jie Xu, Sharon Barone, Kamyar Zahedi, Manoocher Soleimani
BACKGROUND: The absence of NCC does not cause significant salt wasting in NCC deficient mice under basal conditions. We hypothesized that ENaC and pendrin play important roles in compensatory salt absorption in the setting of NCC inactivation, and their inhibition and/or downregulation can cause significant salt wasting in NCC KO mice. METHODS: WT and NCC KO mice were treated with a daily injection of either amiloride, an inhibitor of ENaC, or acetazolamide (ACTZ), a blocker of salt and bicarbonate reabsorption in the proximal tubule and an inhibitor of carbonic anhydrases in proximal tubule and intercalated cells, or a combination of acetazolamide plus amiloride for defined durations...
2016: PloS One
Peter M Haggie, Puay-Wah Phuan, Joseph-Anthony Tan, Lorna Zlock, Walter E Finkbeiner, A S Verkman
Pendrin (SLC26A4) is a Cl(-)/anion exchanger expressed in the epithelium of inflamed airways where it is thought to facilitate Cl(-) absorption and HCO3 (-) secretion. Studies using pendrin knockout mice and airway epithelial cells from hearing-impaired subjects with pendrin loss of function suggest involvement of pendrin in inflammatory lung diseases, including cystic fibrosis (CF), perhaps by regulation of airway surface liquid (ASL) volume. Here we identified small-molecule pendrin inhibitors and demonstrated their efficacy in increasing ASL volume...
June 2016: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
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