Consequences and therapy of the metabolic acidosis of chronic kidney disease, Pediatr. Nephrol, vol.26, pp.19-28, 2011. ,
Urinary ammonia and long-term outcomes in chronic kidney disease, Kidney Int, vol.88, pp.137-145, 2015. ,
Proximal renal tubular acidosis: a not so rare disorder of multiple etiologies, Nephrol. Dial. Transplant. Off. Publ. Eur. Dial. Transpl. Assoc. -Eur. Ren. Assoc, vol.27, pp.4273-4287, 2012. ,
NBCe1 expression is required for normal renal ammonia metabolism, Am. J. Physiol. Renal Physiol, vol.309, pp.658-666, 2015. ,
NBCe1-A Regulates Proximal Tubule Ammonia Metabolism under Basal Conditions and in Response to Metabolic Acidosis, J. Am. Soc. Nephrol. JASN, vol.29, pp.1182-1197, 2018. ,
Colonic anion secretory defects and metabolic acidosis in mice lacking the NBC1 Na+/HCO3-cotransporter, J. Biol. Chem, vol.282, pp.9042-9052, 2007. ,
Familial pure proximal renal tubular acidosis--a clinical and genetic study, Nephrol. Dial. Transplant. Off. Publ. Eur. Dial. Transpl. Assoc. -Eur. Ren. Assoc, vol.23, pp.1211-1215, 2008. ,
Effect of bath and luminal potassium concentration on ammonia production and secretion by mouse proximal tubules perfused in vitro, J. Clin. Invest, vol.86, pp.32-39, 1990. ,
Electrophysiology of ammonia transport in renal straight proximal tubules, Kidney Int, vol.40, pp.1082-1089, 1991. ,
Molecular diversity and regulation of renal potassium channels, Physiol. Rev, vol.85, pp.319-371, 2005. ,
Acid secretion-associated translocation of KCNJ15 in gastric parietal cells, Am. J. Physiol. Gastrointest. Liver Physiol, vol.301, pp.591-600, 2011. ,
Cloning, expression, and localization of a rat hepatocyte inwardly rectifying potassium channel, Am. J. Physiol. Gastrointest. Liver Physiol, vol.282, pp.233-240, 2002. ,
Differential assembly of inwardly rectifying K+ channel subunits, Kir4.1 and Kir5.1, in brain astrocytes, J. Biol. Chem, vol.279, pp.44065-44073, 2004. ,
A panoramic view of gene expression in the human kidney, Proc. Natl. Acad. Sci. U. S. A, vol.100, pp.13710-13715, 2003. ,
Ammonia transport in the kidney by Rhesus glycoproteins, Am. J. Physiol. Renal Physiol, vol.306, pp.1107-1120, 2014. ,
Role of NH3 and NH4+ transporters in renal acid-base transport, Am. J. Physiol. -Ren. Physiol, vol.300, pp.11-23, 2011. ,
Regulation of renal amino acid transporters during metabolic acidosis, Am. J. Physiol. Renal Physiol, vol.292, pp.555-566, 2007. ,
Changes in mRNAs for enzymes of glutamine metabolism in kidney and liver during ammonium chloride acidosis, Am. J. Physiol, vol.267, pp.400-406, 1994. ,
What is the underlying defect in patients with isolated, proximal renal tubular acidosis?, Am. J. Nephrol, vol.9, pp.265-268, 1989. ,
Regulatory mechanism of cell pH in the renal proximal tubule of bullfrog nephron, Jpn. J. Physiol, vol.35, pp.741-763, 1985. ,
Proximal renal tubular acidosis in TASK2 K+ channeldeficient mice reveals a mechanism for stabilizing bicarbonate transport, Proc. Natl. Acad. Sci. U ,
URL : https://hal.archives-ouvertes.fr/hal-00094493
, , vol.101, pp.8215-8220, 2004.
Fused cells of frog proximal tubule: II. Voltagedependent intracellular pH, J. Membr. Biol, vol.101, pp.259-265, 1988. ,
Mechanism of basolateral membrane H+/OH-/HCO-3 transport in the rat proximal convoluted tubule. A sodium-coupled electrogenic process, J. Gen. Physiol, vol.86, pp.613-636, 1985. ,
Potassium conductance in straight proximal tubule cells of the mouse. Effect of barium, verapamil and quinidine, Biochim. Biophys. Acta, vol.900, pp.275-281, 1987. ,
Role of KCNE1-Dependent K+ Fluxes in Mouse Proximal Tubule, J. Am. Soc. Nephrol, vol.12, pp.2003-2011, 2001. ,
Intracellular pH regulation in the renal proximal tubule of the salamander. Basolateral HCO3-transport, J. Gen. Physiol, vol.81, pp.53-94, 1983. ,
Rheogenic sodium-bicarbonate cotransport in the peritubular cell membrane of rat renal proximal tubule, Pflugers Arch, vol.405, pp.360-366, 1985. ,
Effects of ouabain and temperature on cell membrane potentials in isolated perfused straight proximal tubules of the mouse kidney, Pflugers Arch, vol.407, pp.252-257, 1986. ,
Kir4.1 K+ channels are regulated by external cations, Channels, vol.5, pp.269-279, 2011. ,
Ammonia transport in a mathematical model of rat proximal tubule, Am. J. Physiol.-Ren. Physiol, vol.267, pp.237-248, 1994. ,
Ammonium as a substrate for Na+-K+-ATPase in rabbit proximal tubules, Am. J. Physiol, vol.250, pp.497-502, 1986. ,
Ammonium replaces potassium in supporting sodium transport by the Na-K-ATPase of renal proximal straight tubules, Am. J. Physiol, vol.249, pp.785-788, 1985. ,
Renal ammonia metabolism and transport, Renal Ammonia Metabolism and Transport. Compr. Physiol. Compr. Physiol, vol.3, pp.201-220, 2013. ,
Molecular analysis of system N suggests novel physiological roles in nitrogen metabolism and synaptic transmission, Cell, vol.99, pp.769-780, 1999. ,
Chronic metabolic acidosis increases NHE3 protein abundance in rat kidney, Am. J. Physiol, vol.271, pp.917-925, 1996. ,
Chronic metabolic acidosis enhances NHE-3 protein abundance and transport activity in the rat thick ascending limb by increasing NHE-3 mRNA, J. Clin. Invest, vol.99, pp.24-30, 1997. ,
Stoichiometry of Na+-HCO-3 cotransport in basolateral membrane vesicles isolated from rabbit renal cortex, J. Clin. Invest, vol.79, pp.1276-1280, 1987. ,
Mechanism of bicarbonate exit across basolateral membrane of the rabbit proximal convoluted tubule, Am. J. Physiol, vol.246, pp.889-896, 1984. ,
Functional analysis of NBC1 mutants associated with proximal renal tubular acidosis and ocular abnormalities, J. Am. Soc. Nephrol. JASN, vol.16, pp.2270-2278, 2005. ,
Impaired urinary ammonium excretion in patients with isolated proximal renal tubular acidosis, J. Am. Soc. Nephrol. JASN, vol.4, pp.1073-1078, 1993. ,
Unraveling the Molecular Pathogenesis of Isolated Proximal Renal Tubular Acidosis, J. Am. Soc. Nephrol, vol.13, pp.2171-2177, 2002. ,
Acid and mineral balances and bone in familial proximal renal tubular acidosis, Kidney Int, vol.58, pp.1267-1277, 2000. ,
Roles and Regulation of Renal K Channels, Annu. Rev. Physiol, vol.78, pp.415-435, 2016. ,
Mechanisms in hyperkalemic renal tubular acidosis, J. Am. Soc. Nephrol. JASN, vol.20, pp.251-254, 2009. ,
,
, K+ channel subunit contrasts with that observed in SeSAME/EAST syndrome, Proc. Natl. Acad. Sci. U. S. A, vol.108, pp.10361-10366, 2011.
1/Kir5.1 channel forms the major K+ channel in the basolateral membrane of mouse renal collecting duct principal cells, Am. J. Physiol. Renal Physiol, vol.294, pp.1398-1407, 2008. ,
URL : https://hal.archives-ouvertes.fr/hal-02453208
A Na+-and Cl--activated K+ channel in the thick ascending limb of mouse kidney, J. Gen. Physiol, vol.127, pp.205-215, 2006. ,
URL : https://hal.archives-ouvertes.fr/inserm-02448150
The interpretation of current-clamp recordings in the cell-attached patch-clamp configuration, Biophys. J, vol.88, pp.739-750, 2005. ,
Cell-attached voltage-clamp and current-clamp recording and stimulation techniques in brain slices, J. Neurosci. Methods, vol.154, pp.1-18, 2006. ,
, Uv (?l/g BW/ day)
Clinical and urine parameters of Kcnj15 +/+ and Kcnj15 -/-mice under basal and acid-loading conditions. Parameters were mesured before (Day 0) and after 2 or 8 days of an oral 0.28 M NH 4 Cl-induced acid load, p.11 ,
Uv : urine volume. BW : body weight. * P < 0.05 vs Day 0 (paired Student's t test), ? P < 0.05 vs Kcnj15 +/+ mice at the given day ,
, Protein extraction. For western blot experiments, mouse kidneys snap-frozen into liquid
, After a centrifugation at 5,000 g for 10 minutes, protein content of supernatants was measured using the Pierce? BCA Protein Assay Kit
, Western blotting. 60 µg proteins were denaturated in a 2X Laemmli buffer containing
,
blocked using a TBS buffer supplemented with 5 % non-fat milk proteins and 0.2 % NP-40, then exposed to primary antibody, washed and finally exposed to the following HRP-conjugated secondary antibody: 170-6515 goat anti-rabbit, 10% polyacrylamide gels, transfered onto nitrocellulose membranes, vol.1 ,
, The primary antibodies used were: APC-058 rabbit anti-Kir4.2, SPC-400D rabbit anti
, :1000 dilution), Cayman Chemical 10004943 rabbit anti-PEPCK (Bertin Bioreagent, dilution), APC-037 rabbit anti-TASK2 (Alomone Labs, 1:1000 dilution), vol.1, p.2500, 2000.
, The rabbit anti-PDG (1:5000 dilution) was kindly provided by Pr Javier Marquez (University of Malaga, Spain) Membrane potential measurement. Tubules were isolated from kidneys after a collagenase treatment as previously described, S5 then transferred into a chamber placed on the stage of an inverted microscope. The bath solution contained (mM) 130 NaCl, 20000.
heat polished and backfilled with the bath solution. (Bio-Logic Science Instruments, Seyssinet-Pariset, France) set in the zero current-clamp (I0) mode was filtered at 50 Hz by a NPI Electronic LPBF-48DG low-pass 8-pole Bessel filter (Tamm, Germany) and digitized at a 100 Hz sampling rate by an Axon Instruments/Molecular Devices Digidata 1440A A/D converter and P-clamp software, HEPES, 10 glucose and was adjusted to pH 7.4 with tris(hydroxymethyl)aminoethane. Patch pipettes were pulled from thin-walled borosilicate glass capillaries, pp.20-25 ,
, Intracellular pH measurements. Dye-loaded tubules were excited alternatively at 440
, BW 20 nm) every 2 seconds with an OptoLED light source (Cairn Research, Faversham, GB) and an iXon EMCCD camera (Andor Technology, vol.500
, 23 mM NaHCO 3 , 2 mM K 2 HPO 4 , 1.5 mM CaCl 2 , 1.2 mM MgSO 4 , 5 mM L-Alanine, 5.5 mM Dglucose and 10 mM HEPES) or a nominally Na + -free solution in which NMDG-Cl and NMDG-HCO 3 were substituted for NaCl and NaHCO 3, respectively. The tubular lumen was perfused with the Na + -free solution all, Proximal tubules were superfused with either a Na + -containing solution (119 mM NaCl
H + exchanger to the intracellular pH responses. All solutions were continuously gassed with 95% O2/5% CO 2 (pH ~7.40) and kept at 37°C ,
Expression of a functional Kir4 family inward rectifier K+ channel from a gene cloned from mouse liver, J. Physiol, vol.514, pp.639-653, 1999. ,
In vivo formation of a proton-sensitive K+ channel by heteromeric subunit assembly of Kir5.1 with Kir4.1, J. Physiol, vol.525, pp.587-592, 2000. ,
Identification of native atrial G-protein-regulated inwardly rectifying K+ (GIRK4) channel homomultimers, J. Biol. Chem, vol.273, pp.27499-27504, 1998. ,
Cloning, expression, and localization of a rat hepatocyte inwardly rectifying potassium channel, Am. J. Physiol. Gastrointest. Liver Physiol, vol.282, pp.233-240, 2002. ,
A Na+-and Cl--activated K+ channel in the thick ascending limb of mouse kidney, J. Gen. Physiol, vol.127, pp.205-215, 2006. ,