keyword
https://read.qxmd.com/read/38365697/clinical-genetic-profile-and-therapy-evaluation-of-11-chinese-pediatric-patients-with-fanconi-bickel-syndrome
#21
JOURNAL ARTICLE
Taozi Du, Yu Xia, Chengkai Sun, Zhuwen Gong, Lili Liang, Zizhen Gong, Ruifang Wang, Deyun Lu, Kaichuang Zhang, Yi Yang, Yuning Sun, Manqing Sun, Yu Sun, Bing Xiao, Wenjuan Qiu
BACKGROUND: Fanconi-Bickel syndrome (FBS) is a rare autosomal recessive disorder characterized by impaired glucose and galactose utilization as well as proximal renal tubular dysfunction. METHODS: Clinical, biochemical, genetic, treatment, and follow-up data for 11 pediatric patients with FBS were retrospectively analysed. RESULTS: Hepatomegaly (10/11), short stature (10/11) and hypophosphataemic rickets (7/11) were the most common initial symptoms...
February 16, 2024: Orphanet Journal of Rare Diseases
https://read.qxmd.com/read/38356600/analysis-of-adaptive-molecular-mechanisms-in-response-to-low-salinity-in-antennal-gland-of-mud-crab-scylla-paramamosain
#22
JOURNAL ARTICLE
Nan Mo, Tianyi Feng, Dandan Zhu, Jiaxin Liu, Shucheng Shao, Rui Han, Wentao Lu, Pingping Zhan, Zhaoxia Cui
As an important marine aquaculture species, the mud crab ( Scylla paramamosain ) is a good candidate for studying the osmoregulatory mechanism of crustaceans. While previous studies have focused on the osmoregulatory function of the gills, this study aims to explore the osmoregulatory function of the antennal glands. By the comparative transcriptomic analysis, we found the pathways of ion regulation including "proximal tubule bicarbonate reclamation" and "mineral absorption" were activated in the antennal glands of the crabs long-term dwelling in low salinity...
February 15, 2024: Heliyon
https://read.qxmd.com/read/38332178/acid-base-transporters-in-the-context-of-tumor-heterogeneity
#23
REVIEW
Stine Helene Falsig Pedersen
The copious metabolic acid production and -extrusion by cancer cells render poorly vascularized regions of solid tumors highly acidic. A growing list of proton - and bicarbonate transporters has been suggested to contribute to net acid extrusion from cancer cells, and/or been shown to be dysregulated and favor malignant development in various cancers. The great majority of these roles have been studied at the level of the cancer cells. However, recent advances in understanding of the cellular and physicochemical heterogeneity of solid tumors both enable and necessitate a reexamination of the regulation and roles of acid-base transporters in such malignancies...
February 9, 2024: Pflügers Archiv: European Journal of Physiology
https://read.qxmd.com/read/38321620/a-carboxysome-based-co-2-concentrating-mechanism-for-c-3-crop-chloroplasts-advances-and-the-road-ahead
#24
JOURNAL ARTICLE
Nghiem D Nguyen, Sacha B Pulsford, Britta Förster, Sarah Rottet, Loraine Rourke, Benedict M Long, G Dean Price
The introduction of the carboxysome-based CO2 concentrating mechanism (CCM) into crop plants has been modelled to significantly increase crop yields. This projection serves as motivation for pursuing this strategy to contribute to global food security. The successful implementation of this engineering challenge is reliant upon the transfer of a microcompartment that encapsulates cyanobacterial Rubisco, known as the carboxysome, alongside active bicarbonate transporters. To date, significant progress has been achieved with respect to understanding various aspects of the cyanobacterial CCM, and more recently, different components of the carboxysome have been successfully introduced into plant chloroplasts...
February 6, 2024: Plant Journal
https://read.qxmd.com/read/38293640/mechanistic-characterization-of-waterborne-selenite-uptake-in-the-water-flea-daphnia-magna-indicates-water-chemistry-affects-toxicity-in-coal-mine-impacted-waters
#25
JOURNAL ARTICLE
Chantelle E Klaczek, Greg G Goss, Chris N Glover
Concentrations of selenium that exceed regulatory guidelines have been associated with coal mining activities and have been linked to detrimental effects on aquatic ecosystems and the organisms therein. Although the major route of selenium uptake in macroinvertebrates is via the diet, the uptake of waterborne selenite (HSeO3 - ), the prominent form at circumneutral pH, can be an important contributor to selenium body burden and thus selenium toxicity. In the current study, radiolabelled selenite (Se75 ) was used to characterize the mechanism of selenite uptake in the water flea, Daphnia magna ...
2024: Conservation Physiology
https://read.qxmd.com/read/38284496/functional-promiscuity-of-small-multidrug-resistance-transporters-from-staphylococcus-aureus-pseudomonas-aeruginosa-and-francisella-tularensis
#26
JOURNAL ARTICLE
Peyton J Spreacker, Andrea K Wegrzynowicz, Colin J Porter, Will F Beeninga, Sydnye Demas, Emma N Powers, Katherine A Henzler-Wildman
Small multidrug resistance transporters efflux toxic compounds from bacteria and are a minimal system to understand multidrug transport. Most previous studies have focused on EmrE, the model SMR from Escherichia coli, finding that EmrE has a broader substrate profile than previously thought and that EmrE may perform multiple types of transport, resulting in substrate-dependent resistance or susceptibility. Here, we performed a broad screen to identify potential substrates of three other SMRs: PAsmr from Pseudomonas aeruginosa; FTsmr from Francisella tularensis; and SAsmr from Staphylococcus aureus...
January 29, 2024: Molecular Microbiology
https://read.qxmd.com/read/38280610/impact-of-road-transport-system-on-groundwater-quality-inferred-from-explainable-artificial-intelligence-xai
#27
JOURNAL ARTICLE
Kyungwon Kwak, Eun Hak Lee
Road networks constitute a vital component of modern society, facilitating rapid transportation and driving economic activities by enabling the smooth movement of goods and people. However, the expansion of road systems carries significant environmental considerations, particularly regarding its impact on groundwater quality. Thus, it is crucial to understand the complex relationship between groundwater quality and the road traffic system. This paper aims to identify the impact of road transport systems on groundwater quality using a data-driven approach...
January 25, 2024: Science of the Total Environment
https://read.qxmd.com/read/38264228/life-threatening-coronary-vasospasm-in-patients-with-type-2-diabetes-with-sglt2-inhibitor-induced-euglycemic-ketoacidosis-a-report-of-two-consecutive-cases
#28
Junko Kawahara, Bunji Kaku, Kunimasa Yagi, Naotaka Kitagawa, Maki Yokoyama, Yusuke Wakabayashi, Satoko Senda, Hiroyuki Takata, Yoshio Hiraiwa
Euglycemic diabetic ketoacidosis (eDKA) has emerged as an adverse event associated with sodium-glucose transporter-2 inhibitors (SGLT2i). We present two consecutive cases of SGLT2i-induced eDKA, both manifested as life-threatening coronary vasospastic angina (VSA). Case 1: A 64-year-old male overweight patient with type 2 diabetes (BMI 28.2 kg/m2 ), treated with dapagliflozin 5 mg daily for 6 months and a restricted diet for 2 months, experienced loss of consciousness following severe chest pain while driving, resulting in a traffic accident: plasma glucose, 163 mg/dL; urine ketones, (+++); bicarbonate (HCO3 - ), 13...
January 2024: Diabetology International
https://read.qxmd.com/read/38262945/pro-inflammatory-cytokines-stimulate-cftr-dependent-anion-secretion-in-pancreatic-ductal-epithelium
#29
JOURNAL ARTICLE
Dora Angyal, Tessa A Groeneweg, Anny Leung, Max Desain, Kalyan Dulla, Hugo R de Jonge, Marcel J C Bijvelds
BACKGROUND: Loss of CFTR-dependent anion and fluid secretion in the ducts of the exocrine pancreas is thought to contribute to the development of pancreatitis, but little is known about the impact of inflammation on ductal CFTR function. Here we used adult stem cell-derived cell cultures (organoids) obtained from porcine pancreas to evaluate the effects of pro-inflammatory cytokines on CFTR function. METHODS: Organoids were cultured from porcine pancreas and used to prepare ductal epithelial monolayers...
January 23, 2024: Cellular & Molecular Biology Letters
https://read.qxmd.com/read/38258089/multiple-regulatory-signals-and-components-in-the-modulation-of-bicarbonate-transporters
#30
REVIEW
Hyeong Jae Kim, Jeong Hee Hong
Bicarbonate transporters are responsible for the appropriate flux of bicarbonate across the plasma membrane to perform various fundamental cellular functions. The functions of bicarbonate transporters, including pH regulation, cell migration, and inflammation, are highlighted in various cellular systems, encompassing their participation in both physiological and pathological processes. In this review, we focused on recently identified modulatory signaling components that regulate the expression and activity of bicarbonate transporters...
January 5, 2024: Pharmaceutics
https://read.qxmd.com/read/38184688/mechanism-of-anion-exchange-and-small-molecule-inhibition-of-pendrin
#31
JOURNAL ARTICLE
Lie Wang, Anthony Hoang, Eva Gil-Iturbe, Arthur Laganowsky, Matthias Quick, Ming Zhou
Pendrin (SLC26A4) is an anion exchanger that mediates bicarbonate (HCO3 - ) exchange for chloride (Cl- ) and is crucial for maintaining pH and salt homeostasis in the kidney, lung, and cochlea. Pendrin also exports iodide (I- ) in the thyroid gland. Pendrin mutations in humans lead to Pendred syndrome, causing hearing loss and goiter. Inhibition of pendrin is a validated approach for attenuating airway hyperresponsiveness in asthma and for treating hypertension. However, the mechanism of anion exchange and its inhibition by drugs remains poorly understood...
January 6, 2024: Nature Communications
https://read.qxmd.com/read/38179545/metal-selectivity-and-translocation-mechanism-characterization-in-proteoliposomes-of-the-transmembrane-nicot-transporter-nixa-from-helicobacter-pylori
#32
JOURNAL ARTICLE
Jayoh A Hernandez, Paul S Micus, Sean Alec Lois Sunga, Luca Mazzei, Stefano Ciurli, Gabriele Meloni
Essential trace metals play key roles in the survival, replication, and virulence of bacterial pathogens. Helicobacter pylori ( H. pylori ), the main bacterial cause of gastric ulcers, requires Ni(ii) to colonize and persist in the acidic environment inside the stomach, exploiting the nickel-containing enzyme urease to catalyze the hydrolysis of urea to ammonia and bicarbonate and create a pH-buffered microenvironment. Urease utilizes Ni(ii) as a catalytic cofactor for its activity. In ureolytic bacteria, unique transmembrane (TM) transporters evolved to guarantee the selective uptake and efflux of Ni(ii) across cellular membranes to meet the cellular requirements...
January 3, 2024: Chemical Science
https://read.qxmd.com/read/38165186/euglycemic-diabetic-ketoacidosis-experience-with-44-patients-and-comparison-to-hyperglycemic-diabetic-ketoacidosis
#33
JOURNAL ARTICLE
Jordan Sell, Nathan L Haas, Frederick K Korley, James A Cranford, Benjamin S Bassin
INTRODUCTION: Euglycemic diabetic ketoacidosis (DKA) (glucose <250 milligrams per deciliter (mg/dL) has increased in recognition since introduction of sodium-glucose co-transporter 2 (SGLT2) inhibitors but remains challenging to diagnose and manage without the hyperglycemia that is otherwise central to diagnosing DKA, and with increased risk for hypoglycemia with insulin use. Our objective was to compare key resource utilization and safety outcomes between patients with euglycemic and hyperglycemic DKA from the same period...
November 2023: Western Journal of Emergency Medicine
https://read.qxmd.com/read/38149052/putting-bicarbonate-on-the-spot-pharmacological-insights-for-cftr-correction-in-the-airway-epithelium
#34
JOURNAL ARTICLE
Miroslaw Zajac, Agathe Lepissier, Elise Dréano, Benoit Chevalier, Aurélie Hatton, Mairead Kelly-Aubert, Daniela Guidone, Gabrielle Planelles, Aleksander Edelman, Emmanuelle Girodon, Alexandre Hinzpeter, Gilles Crambert, Iwona Pranke, Luis J V Galietta, Isabelle Sermet-Gaudelus
Introduction: Cystic fibrosis (CF) is caused by defective Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) proteins. CFTR controls chloride (Cl- ) and bicarbonate (HCO3 - ) transport into the Airway Surface Liquid (ASL). We investigated the impact of F508del-CFTR correction on HCO3 - secretion by studying transepithelial HCO3 - fluxes. Methods: HCO3 - secretion was measured by pH-stat technique in primary human respiratory epithelial cells from healthy subjects (WT) and people with CF (pwCF) carrying at least one F508del variant...
2023: Frontiers in Pharmacology
https://read.qxmd.com/read/38132452/a-comparative-kidney-transcriptome-analysis-of-bicarbonate-loaded-insrr-null-mice
#35
JOURNAL ARTICLE
E A Gantsova, O V Serova, D Eladari, D M Bobrovskiy, A G Petrenko, A V Elchaninov, I E Deyev
The maintenance of plasma pH is critical for life in all organisms. The kidney plays a critical role in acid-base regulation in vertebrates by controlling the plasma concentration of bicarbonate. The receptor tyrosine kinase IRR (insulin receptor-related receptor) is expressed in renal β-intercalated cells and is involved in alkali sensing due to its ability to autophosphorylate under alkalization of extracellular medium (pH > 7.9). In mice with a knockout of the insrr gene, which encodes for IRR, urinary bicarbonate secretion in response to alkali loading is impaired...
December 4, 2023: Current Issues in Molecular Biology
https://read.qxmd.com/read/38113802/effects-of-electron-acceptors-and-donors-on-anaerobic-biodegradation-of-pahs-in-marine-sediments
#36
JOURNAL ARTICLE
Qingguo Chen, Zhenzhen Li, Yu Chen, Mei Liu, Qiao Yang, Baikang Zhu, Jun Mu, Lijuan Feng, Zhi Chen
Polycyclic aromatic hydrocarbons (PAHs) are typical organic pollutants accumulated in the environment. PAHs' bioremediation in sediments can be promoted by adding electron acceptor (EA) and electron donor (ED). Bicarbonate and sulfate were chosen as two EAs, and acetate and lactate were selected as two EDs. Six groups of amendments were added into the sediments to access their role in the anaerobic biodegradation of five PAHs, containing phenanthrene, anthracene, fluoranthene, pyrene, and benzo[a]pyrene. The concentrations of PAHs, EAs and EDs, electron transport system activity, and microbial diversity were analyzed during 126-day biodegradation in serum bottles...
December 18, 2023: Marine Pollution Bulletin
https://read.qxmd.com/read/38092167/insight-into-the-potential-mechanism-of-bicarbonate-assimilation-promoted-by-mixotrophic-in-co-2-absorption-and-microalgae-conversion-system
#37
JOURNAL ARTICLE
Pengcheng Li, Dantong Wang, Zhan Hu, Danqing Chen, Yi Wang, Min Wang, Shuzhen Wei, Chunfeng Song
CO2 absorption-microalgae conversion (CAMC) system is a promising carbon capture and utilization technology. However, the use of HCO3 - as a carbon source often led to a slower growth rate of microalgae, which also limited the application of CAMC system. In this study, the assimilation efficiency of HCO3 - in CAMC system was improved through mixotrophic, and the potential mechanism was investigated. The HCO3 - assimilation efficiency and biomass under mixotrophic were 34.79% and 31.76% higher than that of control...
December 11, 2023: Chemosphere
https://read.qxmd.com/read/38078375/acetazolamide-and-human-carbonic-anhydrases-retrospect-review-and-discussion-of-an-intimate-relationship
#38
REVIEW
Dimitrios Tsikas
Acetazolamide (AZM) is a strong pharmacological sulphonamide-type (R-SO2 -NH2 , p K a 7.2) inhibitor of the activity of several carbonic anhydrase (CA) isoforms, notably of renal CA II ( K i , 12 nM) and CA IV ( K i , 74 nM). AZM is clinically used for about eighty years in various diseases including epilepsy and glaucoma. Pharmacological AZM increases temporarily the urinary excretion of bicarbonate (HCO3 - ) and sodium ions (Na+ ) and sustainably the urinary pH. AZM is excreted almost unchanged over several hours at high rates in the urine...
December 2024: Journal of Enzyme Inhibition and Medicinal Chemistry
https://read.qxmd.com/read/38069156/co-expression-network-analysis-of-the-transcriptome-identified-hub-genes-and-pathways-responding-to-saline-alkaline-stress-in-sorghum-bicolor-l
#39
JOURNAL ARTICLE
Hongcheng Wang, Lvlan Ye, Lizhou Zhou, Junxing Yu, Biao Pang, Dan Zuo, Lei Gu, Bin Zhu, Xuye Du, Huinan Wang
Soil salinization, an intractable problem, is becoming increasingly serious and threatening fragile natural ecosystems and even the security of human food supplies. Sorghum ( Sorghum bicolor L.) is one of the main crops growing in salinized soil. However, the tolerance mechanisms of sorghum to saline-alkaline soil are still ambiguous. In this study, RNA sequencing was carried out to explore the gene expression profiles of sorghum treated with sodium bicarbonate (150 mM, pH = 8.0, treated for 0, 6, 12 and 24 h)...
November 27, 2023: International Journal of Molecular Sciences
https://read.qxmd.com/read/38054405/biallelic-variants-in-slc4a10-encoding-the-sodium-dependent-chloride-bicarbonate-exchanger-ncbe-lead-to-a-neurodevelopmental-disorder
#40
JOURNAL ARTICLE
Reza Maroofian, Mina Zamani, Rauan Kaiyrzhanov, Lutz Liebmann, Ehsan Ghayoor Karimiani, Barbara Vona, Antje K Huebner, Daniel G Calame, Vinod K Misra, Saeid Sadeghian, Reza Azizimalamiri, Mohammad Hasan Mohammadi, Jawaher Zeighami, Sogand Heydaran, Mehran Beiraghi Toosi, Javad Akhondian, Meisam Babaei, Narges Hashemi, Rhonda E Schnur, Mohnish Suri, Jonas Setzke, Matias Wagner, Theresa Brunet, Christopher M Grochowski, Lisa Emrick, Wendy K Chung, Ute A Hellmich, Miriam Schmidts, James R Lupski, Hamid Galehdari, Mariasavina Severino, Henry Houlden, Christian A Hübner
PURPOSE: SLC4A10 encodes a plasma membrane-bound transporter, which mediates Na+ -dependent HCO3 - import thus mediating net acid extrusion. Slc4a10 knockout (KO) mice show collapsed brain ventricles, an increased seizure threshold, mild behavioral abnormalities, impaired vision, and deafness. METHODS: Utilizing exome/genome sequencing in families with undiagnosed neurodevelopmental disorders (NDD) and international data sharing, 11 patients from 6 independent families with biallelic variants in SLC4A10 were identified...
December 2, 2023: Genetics in Medicine: Official Journal of the American College of Medical Genetics
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