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Brain : a Journal of Neurology Mar 2023We describe five families from different regions in Norway with a late-onset autosomal-dominant hereditary polyneuropathy sharing a heterozygous variant in the SLC12A6...
We describe five families from different regions in Norway with a late-onset autosomal-dominant hereditary polyneuropathy sharing a heterozygous variant in the SLC12A6 gene. Mutations in the same gene have previously been described in infants with autosomal-recessive hereditary motor and sensory neuropathy with corpus callosum agenesis and mental retardation (Andermann syndrome), and in a few case reports describing dominantly acting de novo mutations, most of them with onset in childhood. The phenotypes in our families demonstrated heterogeneity. Some of our patients only had subtle to moderate symptoms and some individuals even no complaints. None had CNS manifestations. Clinical and neurophysiological evaluations revealed a predominant sensory axonal polyneuropathy with slight to moderate motor components. In all 10 patients the identical SLC12A6 missense variant, NM_001365088.1 c.1655G>A p.(Gly552Asp), was identified. For functional characterization, the mutant potassium chloride cotransporter 3 was modelled in Xenopus oocytes. This revealed a significant reduction in potassium influx for the p.(Gly552Asp) substitution. Our findings further expand the spectrum of SLC12A6 disease, from biallelic hereditary motor and sensory neuropathy with corpus callosum agenesis and mental retardation and monoallelic early-onset hereditary motor and sensory neuropathy caused by de novo mutations, to late-onset autosomal-dominant axonal neuropathy with predominant sensory deficits.
Topics: Humans; Agenesis of Corpus Callosum; Hereditary Sensory and Motor Neuropathy; Intellectual Disability; Mutation; Phenotype; Symporters
PubMed: 36542484
DOI: 10.1093/brain/awac488 -
Foods (Basel, Switzerland) Jun 2023One of the salting methods in cheese production implies salting the milk before coagulation used in making Domiati-type cheeses and a variety of autochthonous "Lički...
One of the salting methods in cheese production implies salting the milk before coagulation used in making Domiati-type cheeses and a variety of autochthonous "Lički Škripavac" cheese. The most used sodium replacer is potassium. This study investigated the influence of different added salt concentrations (1%, 1.5%, and 2%) and NaCl to KCl ratios (100%, 50:50%, 25:75%) on the rennet coagulation and curd firmness in bovine milk. The milk coagulation parameters were determined with a computerized renneting meter, Lactodinamograph. The results showed significant interactions between the salt concentrations and NaCl to KCl ratios ( < 0.0001, α = 0.05) by prolonging the beginning of coagulation (10-20 min) and curd firming rate (1-5 min) by an increase in salt concentration for all treatments. The 50:50 treatment values (RCT, k, a, a, a) were closest to the control (without salt) and had the best results among all treatments in the lower (1%) and medium (1.5%) salt concentration ( > 0.0001, α = 0.05) while in the highest salt concentration (2%) the treatment effect was nonsignificant ( > 0.05). These results should help future studies make a lower sodium product appealing to consumers without losing quality.
PubMed: 37372505
DOI: 10.3390/foods12122293 -
Molecules (Basel, Switzerland) Dec 2023The flotation agent is an important collector in the production of potassium chloride and is brought into the crystallization stage with the reflux of the mother liquor....
The flotation agent is an important collector in the production of potassium chloride and is brought into the crystallization stage with the reflux of the mother liquor. Octadecylamine Hydrochloride (ODA), 1-Dodecylamine Hydrochloride (DAH) and Sodium 1-dodecanesulfonate (SDS) were selected to study their effect on the nucleation of potassium chloride. Focused Beam Reflectance Measurement was used to collect the nucleation-induced periods of KCl in the presence of flotation agents at different supersaturations. Then, empirical equations, classical nucleation theory and growth mechanism equations were employed for data analysis. It was found that the presence of flotation agents increased the nucleation sequence , and (ODA) > (SDS) > (DAH) > (HO). In addition, the interfacial energy data obtained using classical nucleation theory suggest that the flotation agents used in our paper promoted the homogeneous nucleation of KCl (reduced from 5.3934 mJ·m to 5.1434 mJ·m) and inhibited the heterogeneous nucleation of KCl (increased from 2.8054 mJ·m to 3.6004 mJ·m). This investigation also revealed that the growth of potassium chloride was consistent with the 2D nucleation-mediated growth mechanism, and the addition of flotation agent did not change the growth mechanism of potassium chloride. Finally, the particle size distribution results were exactly consistent with the order of nucleation order . The study of nucleation kinetics and growth mechanisms of different flotation agents on potassium chloride can provide guidance for optimizing the production process of potassium chloride and developing new flotation agents.
PubMed: 38067652
DOI: 10.3390/molecules28237923 -
Journal of the American Chemical Society Aug 2022Chalcogen bonding (ChB) is rapidly rising to prominence in supramolecular chemistry as a powerful sigma (σ)-hole-based noncovalent interaction, especially for...
Chalcogen bonding (ChB) is rapidly rising to prominence in supramolecular chemistry as a powerful sigma (σ)-hole-based noncovalent interaction, especially for applications in the field of molecular recognition. Recent studies have demonstrated ChB donor strength and potency to be remarkably sensitive to local electronic environments, including redox-switchable on/off anion binding and sensing capability. Influencing the unique electronic and geometric environment sensitivity of ChB interactions through simultaneous cobound metal cation recognition, herein, we present the first potassium chloride-selective heteroditopic ion-pair receptor. The direct conjugation of benzo-15-crown-5 ether (B15C5) appendages to Te centers in a bis-tellurotriazole framework facilitates alkali metal halide (MX) ion-pair binding through the formation of a cofacial intramolecular bis-B15C5 M (M = K, Rb, Cs) sandwich complex and bidentate ChB···X formation. Extensive quantitative H NMR ion-pair affinity titration experiments, solid-liquid and liquid-liquid extraction, and U-tube transport studies all demonstrate unprecedented KCl selectivity over all other group 1 metal chlorides. It is demonstrated that the origin of the receptor's ion-pair binding cooperativity and KCl selectivity arises from an electronic polarization of the ChB donors induced by the cobound alkali metal cation. Importantly, the magnitude of this switch on Te-centered electrophilicity, and therefore anion-binding affinity, is shown to correlate with the inherent Lewis acidity of the alkali metal cation. Extensive computational DFT investigations corroborated the experimental alkali metal cation-anion ion-pair binding observations for halides and oxoanions.
Topics: Anions; Cations; Chalcogens; Chlorides; Metals, Alkali; Potassium Chloride
PubMed: 35930460
DOI: 10.1021/jacs.2c05333 -
PloS One 2023To investigate the effect of the addition of a low concentration of sodium chloride (NaCl) and potassium chloride (KCl) solutions on the tear ferning (TF) patterns of...
PURPOSE
To investigate the effect of the addition of a low concentration of sodium chloride (NaCl) and potassium chloride (KCl) solutions on the tear ferning (TF) patterns of tears collected from humans.
METHODS
A tear sample (20 μL) was collected from the right eye of 23 males and 7 females (25.4 ± 6.6 years). The tears were collected in one sitting for healthy subjects (N = 13). For dry eye participants (N = 17), the tear samples were collected in two separate settings with five minutes gap in between. A sample (1 μL) from each tear was dried on a glass slide, and the obtained ferns were observed using a microscope and graded using the five-point TF grading scale. Mixtures of tear samples (0.5 μL) and different volumes (0.5-2.5 μL) of each electrolyte (10-30 mg in 100 mL of water) solution were prepared, and their TF patterns were recorded and compared with those of the corresponding pure tears.
RESULTS
Significant improvements (Wilcoxon test, P < 0.001) have been seen in the TF grades of the tear samples after the addition of NaCl and KCl solutions. A significant difference (Wilcoxon test, P = 0.016) was found between the TF grades when NaCl and KCl solutions were added to the tear samples. The TF grades of pure tears collected from dry-eye subjects ranged from 2.1 to 3.5, based on the five points grading scale, and decreased to be in the range of 0.4 to 1.6 after the addition of electrolyte solutions. While the TF grades of pure tears collected from normal-eye ranged from 1.2 to 1.9 and improved after the addition of electrolyte solution to be in the range of 0.4 to 1.5.
CONCLUSIONS
The TF test was used in vitro to assess the impact of the addition of a low concentration of sodium and potassium chloride solutions on tears collected from humans. The TF grades of human tears significantly improved after the addition of either sodium or potassium chloride solution. The mechanism for the improvement in TF grades due to the addition of electrolyte solutions must be investigated.
Topics: Male; Female; Humans; Sodium Chloride; Potassium Chloride; Dry Eye Syndromes; Lacerations; Electrolytes; Tears; Sodium
PubMed: 36735697
DOI: 10.1371/journal.pone.0280853 -
Journal of the American College of... Aug 2020
Topics: Aged; Diuretics; Furosemide; Heart Failure; Humans; Prescriptions; Sodium Potassium Chloride Symporter Inhibitors
PubMed: 32762902
DOI: 10.1016/j.jacc.2020.06.044 -
JMIR Public Health and Surveillance Jul 2021Regular salt is about 100% sodium chloride. Low-sodium salts have reduced sodium chloride content, most commonly through substitution with potassium chloride. Low-sodium...
BACKGROUND
Regular salt is about 100% sodium chloride. Low-sodium salts have reduced sodium chloride content, most commonly through substitution with potassium chloride. Low-sodium salts have a potential role in reducing the population's sodium intake levels and blood pressure, but their availability in the global market is unknown.
OBJECTIVE
The aim of this study is to assess the availability, formulation, labeling, and price of low-sodium salts currently available to consumers worldwide.
METHODS
Low-sodium salts were identified through a systematic literature review, Google search, online shopping site searches, and inquiry of key informants. The keywords "salt substitute," "low-sodium salt," "potassium salt," "mineral salt," and "sodium reduced salt" in six official languages of the United Nations were used for the search. Information about the brand, formula, labeling, and price was extracted and analyzed.
RESULTS
A total of 87 low-sodium salts were available in 47 out of 195 (24%) countries worldwide, including 28 high-income countries, 13 upper-middle-income countries, and 6 lower-middle-income countries. The proportion of sodium chloride varied from 0% (sodium-free) to 88% (as percent of weight; regular salt is 100% sodium chloride). Potassium chloride was the most frequent component with levels ranging from 0% to 100% (potassium chloride salt). A total of 43 (49%) low-sodium salts had labels with the potential health risks, and 33 (38%) had labels with the potential health benefits. The median price of low-sodium salts in high-income, upper-middle-income, and lower-middle-income countries was US $15.00/kg (IQR 6.4-22.5), US $2.70/kg (IQR 1.7-5.5), and US $2.90/kg (IQR 0.50-22.2), respectively. The price of low-sodium salts was between 1.1 and 14.6 times that of regular salts.
CONCLUSIONS
Low-sodium salts are not widely available and are commonly more expensive than regular salts. Policies that promote the availability, affordability, and labeling of low-sodium salts should increase uptake, helping populations reduce blood pressure and prevent cardiovascular diseases.
INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID)
RR2-10.1111/jch.14054.
Topics: Blood Pressure; Diet, Sodium-Restricted; Humans; Sodium; Sodium Chloride; Sodium Chloride, Dietary
PubMed: 33985938
DOI: 10.2196/27423 -
Molecular Neurobiology Feb 2023The mechanisms of treatment-resistant depression (TRD) are not clear and are difficult to study. An animal model resembling human TRD is the Wistar Kyoto rat strain. In...
The mechanisms of treatment-resistant depression (TRD) are not clear and are difficult to study. An animal model resembling human TRD is the Wistar Kyoto rat strain. In the present study, we focused on selecting miRNAs that differentiate rats of the WKY strain from Wistar Han (WIS) rats in two divisions of the habenula, the lateral and medial (LHb and MHb, respectively). Based on our preliminary study and literature survey, we identified 32 miRNAs that could be potentially regulated in the habenula. Six miRNAs significantly differentiated WKY rats from WIS rats within the MHb, and three significantly differentiated WKY from WIS rats within the LHb. Then, we selected relevant transcripts regulated by those miRNAs, and their expression in the habenular nuclei was investigated. For mRNAs that differentiated WKY rats from WIS rats in the MHb (Cdkn1c, Htr7, Kcnj9, and Slc12a5), their lower expression correlated with a higher level of relevant miRNAs. In the LHb, eight mRNAs significantly differentiated WKY from WIS rats (upregulated Htr4, Drd2, Kcnj5, and Sstr4 and downregulated Htr2a, Htr7, Elk4, and Slc12a5). These data indicate that several important miRNAs are expressed in the habenula, which differentiates WKY rats from WIS rats and in turn correlates with alterations in the expression of target transcripts. Of particular note are two genes whose expression is altered in WKY rats in both LHb and MHb: Slc12a5 and Htr7. Regulation of KCC2 via the 5-HT7 receptor may be a potential target for the treatment of TRD.
Topics: Animals; Rats; Depression; G Protein-Coupled Inwardly-Rectifying Potassium Channels; Habenula; MicroRNAs; Phenotype; Rats, Inbred WKY
PubMed: 36344870
DOI: 10.1007/s12035-022-03103-y -
Scientific Reports Oct 2020The measurement of electric potential and resistance reflect the transport of sodium and chloride ions which take place in keratinocytes and is associated with skin...
The measurement of electric potential and resistance reflect the transport of sodium and chloride ions which take place in keratinocytes and is associated with skin response to stimuli arising from external and internal environment. The aim of the study was to assess changes in electrical resistance and the transport of chloride and sodium ions, under iso-osmotic conditions and following the use of inhibitors affecting these ions' transport, namely amiloride (A) and bumetanide (B). The experiment was performed on 104 fragments of rabbit skin, divided into three groups: control (n = 35), A-inhibited sodium transport (n = 33) and B-inhibited chloride transport (n = 36). Measurement of electrical resistance (R) and electrical potential (PD) confirmed tissue viability during the experiment, no statistically significant differences in relation to control conditions were noted. The minimal and maximal PD measured during stimulation confirmed the repeatability of the recorded reactions to the mechanical and mechanical-chemical stimulus for all examined groups. Measurement of PD during stimulation showed differences in the transport of sodium and chloride ions in each of the analyzed groups relative to the control. The statistical analysis of the PD measured in stationary conditions and during mechanical and/or mechanical-chemical stimulation proved that changes in sodium and chloride ion transport constitute the physiological response of keratinocytes to changes in environmental conditions for all applied experimental conditions. Assessment of transdermal ion transport changes may be a useful tool for assessing the skin condition with tendency to pain hyperactivity and hypersensitivity to xenobiotics.
Topics: Amiloride; Animals; Bumetanide; Chlorides; Electrophysiology; Epithelial Sodium Channel Blockers; Ion Transport; Rabbits; Skin; Sodium; Sodium Potassium Chloride Symporter Inhibitors
PubMed: 33093644
DOI: 10.1038/s41598-020-75275-3 -
International Journal of Environmental... Jan 2023The effects of salinization on freshwater ecosystems have been estimated by testing sodium chloride (NaCl) since it is the most widely used salt as a deicing agent and...
The effects of salinization on freshwater ecosystems have been estimated by testing sodium chloride (NaCl) since it is the most widely used salt as a deicing agent and Na and Cl ions are the most representative in seawater composition. However, calcium, magnesium, and/or potassium are starting to be proposed as potential surrogates for NaCl, but for which ecotoxicological effects are less explored. This study aimed to identify (i) the less toxic salt to freshwater biota to be suggested as a safer alternative deicer and (ii) to contribute to the lower tiers of salinity risk assessment frameworks by identifying a more suitable surrogate salt than NaCl. The battery of ecotoxicity assays with five key trophic level species showed that among the tested salts (MgCl, CaCl, and KCl), KCl and CaCl seemed to induce the highest and lowest toxicity, respectively, compared with NaCl. CaCl is suggested as a safer alternative for use as a deicer and KCl as a surrogate for the risk assessment of seawater intrusion in coastal regions. These results enrich the salt toxicity database aiming to identify and propose more suitable surrogate salts to predict the effects of salinization to a broader extent.
Topics: Sodium Chloride; Salts; Ecosystem; Calcium Chloride; Salinity; Fresh Water; Cations; Biota
PubMed: 36767106
DOI: 10.3390/ijerph20031741