Chlorides ; metabolism ; Diarrhea ; congenital ; Humans

No abstract available.
Animal ; Chlorides/metabolism* ; Endometrium/metabolism* ; Female ; Mice ; Sodium/metabolism*

Outwardly rectifying swelling-activated chloride conductance (ICl,Swell) in rabbit heart plays a critical role in cardioprotection following ischemic preconditioning (IP). But the functional characterization and molecular basis of this chloride conductance in rabbit heart ventricular myocytes is not clear. Candidate chloride channel clones (e.g. ClC-2, ClC-3, ClC-4 and ClC-5) were determined using RT-PCR and Western blot analysis. Whole cell ICl,Swell was recorded from isolated rabbit ventricular myocytes using patch clamp techniques during hypo-osmotic stress. The inhibitory effects of 4,4' isothiocyanato-2,2-disulfonic acid (DIDS), 5-nitro-2(3-phenylroylamino) benzoic acid (NPPB) and indanyloxyacetic acid 94 (IAA-94) on ICl,Swell were examined. The expected size of PCR products for ClC-2, ClC-3 and ClC-4 but not for ClC-5 was obtained. ClC-2 and ClC-3 expression was confirmed by automated fluorescent DNA sequencing. RT-PCR and Western blot showed that ClC-4 was expressed in abundance and ClC-2 was expressed at somewhat lower levels. The biological and pharmacological properties of I(Cl,Swell), including outward rectification, activation due to cell volume change, sensitivity to DIDS, IAA-94 and NPPB were identical to those known properties of ICl,Swell in exogenously expressed systems and other mammals hearts. It was concluded that ClC-3 or ClC-4 might be responsible for the outwardly rectifying part of ICl,Swell and may be the molecular targets of cardioprotection associated with ischemic preconditioning or hypo-osmotic shock.
Biophysics/methods ; Chlorides/*chemistry ; Chlorides/metabolism ; DNA Primers/chemistry ; Electrophysiology/methods ; Gene Expression Regulation ; Glycolates/pharmacology ; Heart Ventricles/*cytology ; Ischemic Preconditioning ; Muscle Cells/*cytology ; Osmosis ; Sequence Analysis, DNA

OBJECTIVETo study the salt stress tolerance of Hongxinju, Huangju and F1 seedlings from orthogonal and reciprocal cross under different salt treatments. Grope for transmissibility of salt tolerance between parents and F1 seedlings, and relativity between flavone, chlorogenic acid contents and salt tolerance.

METHODThe materials were put in 5 different concentrations of Hoagland nutrient solution (0, 40, 80, 120, 160 mmol x L(-1)) containing NaCl, keeping grads while raising the consistency of NaCl day by day. The injured leaf area per plant, proline, betaine, MDA, flavones and chlorogenic acid contents were measured and analyzed after treatment.

RESULTAs NaCl concentration was below 120 mmol x L(-1), the salt tolerance of Hongxinju was higher than that of Huangju, the salt tolerance of Hongxinju x Huangju higher than that of parents, the salt tolerance of Huangju x Hongxinju was at the level of parents. As NaCl concentration between 120 to 160 mmol x L(-1), the salt tolerance of Huangju was higher than that of Hongxinju, the salt tolerance of Huangju x Hongxinju higher than that of parents and the salt tolerance of Hongxinju x Huangju was at the level of parents.

CONCLUSIONSalt tolerance of F1 is more influenced by female parent, relativity showed between flavonoids, chlorogenic acid contents and salt tolerance.

Breeding ; China ; Chlorides ; metabolism ; Chrysanthemum ; genetics ; growth & development ; physiology ; Salt-Tolerance ; Seedlings ; genetics ; growth & development ; physiology

The present paper describes two Korean male infants, 1. 16 year old and newly born neonate from two families who were diagnosed and managed for one of very rare inborn errors of metabolism, congenital chloridorrhea (Darrow-Gamble syndrome). The diagnosis was suggested by one of the authors (HRM) from the unusual combination of metabolic alkalosis with severe gastrointestinal disorder presenting with chronic, profuse watery diarrhea in the newborn period in the first patient; and the maternal polyhydramnios, the appearance of dilated fetal bowel loops on prenatal ultrasonography and profuse watery diarrhea beginning at birth without passage of meconium in the second patient. The diagnosis was confirmed in both patients by examination of the stool chloride concentration which revealed extremely high exceeding the sum of sodium and potassium concentrations. Serum electrolytes and arterial blood gas analyses revealed hyponatremia, hypokalemia and hypochloremia with elevated bicarbonate. With replacement of fluid and electrolyte deficit and adequate dietary supplements of potassium and chloride, both patients remained well although the character of the stools waxed and waned. This is the first reported case of congenital chloridorrhea in korean population.
Adolescent ; Chlorides/*metabolism ; Diagnosis, Differential ; Diarrhea/*congenital/diagnosis/ethnology ; Diarrhea, Infantile/*diagnosis/ethnology/metabolism ; Humans ; Infant, Newborn ; Korea ; Male

The present paper describes two Korean male infants, 1. 16 year old and newly born neonate from two families who were diagnosed and managed for one of very rare inborn errors of metabolism, congenital chloridorrhea (Darrow-Gamble syndrome). The diagnosis was suggested by one of the authors (HRM) from the unusual combination of metabolic alkalosis with severe gastrointestinal disorder presenting with chronic, profuse watery diarrhea in the newborn period in the first patient; and the maternal polyhydramnios, the appearance of dilated fetal bowel loops on prenatal ultrasonography and profuse watery diarrhea beginning at birth without passage of meconium in the second patient. The diagnosis was confirmed in both patients by examination of the stool chloride concentration which revealed extremely high exceeding the sum of sodium and potassium concentrations. Serum electrolytes and arterial blood gas analyses revealed hyponatremia, hypokalemia and hypochloremia with elevated bicarbonate. With replacement of fluid and electrolyte deficit and adequate dietary supplements of potassium and chloride, both patients remained well although the character of the stools waxed and waned. This is the first reported case of congenital chloridorrhea in korean population.
Adolescent ; Chlorides/*metabolism ; Diagnosis, Differential ; Diarrhea/*congenital/diagnosis/ethnology ; Diarrhea, Infantile/*diagnosis/ethnology/metabolism ; Humans ; Infant, Newborn ; Korea ; Male

Objective: To investigate the expression of cation chloride cotransporter (NKCC1/KCC2) in the neurons from cerebral lesions of children with focal cortical dysplasia (FCD) type Ⅱ, to provide a morphological basis for revealing the possible mechanism of epilepsy. Methods: Eight cases of FCD type Ⅱ diagnosed at Beijing Haidian Hospital, Beijing, China and 12 cases diagnosed at Xuanwu Hospital, Capital Medical University, Beijing, China from February 2017 to December 2019 were included. The expression of NKCC1 and KCC2 in FCD type Ⅱa and FCD type Ⅱb was detected using immunohistochemistry and double immunohistochemical stains. The average optical density of NKCC1 in dysmorphic neurons and normal neurons was also determined using immunohistochemical staining in FCD type Ⅱa (10 cases). Results: The patients were all younger than 14 years of age. Ten cases were classified as FCD type IIa, and 10 cases as FCD type Ⅱb. NKCC1 was expressed in the cytoplasm of normal cerebral cortex neurons and KCC2 expressed on cell membranes. In dysmorphic neurons of FCD type Ⅱa, expression of NKCC1 increased, which was statistically higher than that of normal neurons (P<0.01). Aberrant expression of KCC2 in dysmorphic neurons was also noted in the cytoplasm. In the FCD Ⅱb type, the expression pattern of NKCC1/KCC2 in dysmorphic neurons was the same as that of FCD type Ⅱa. The aberrant expression of NKCC1 in balloon cells was negative or weakly positive on the cell membrane, while the aberrant expression of KCC2 was absent. Conclusions: The expression pattern of NKCC1/KCC2 in dysmorphic neurons and balloon cells is completely different from that of normal neurons. The NKCC1/KCC2 protein-expression changes may affect the transmembrane chloride flow of neurons, modify the effect of inhibitory neurotransmitters γ-aminobutyric acid and increase neuronal excitability. These effects may be related to the occurrence of clinical epileptic symptoms.
Child ; Humans ; Brain/pathology* ; Cations/metabolism* ; Chlorides/metabolism* ; Epilepsy/metabolism* ; Malformations of Cortical Development, Group I/metabolism* ; Solute Carrier Family 12, Member 2/metabolism* ; Symporters/metabolism*

OBJECTIVETo investigate the effect of aluminum trichloride on the abnormal phosphorylation of tau protein in SH-SY5Y cells.

METHODSSH-SY5Y cells were assigned to control group and aluminum trichloride exposure groups (200, 400, and 800 µmol/L Al(3+)). The cell morphology was observed after 48 hours of exposure; the cell viability was measured by CCK-8 assay; total protein was extracted from the cells, and the expression of phospho-tau (p-tau) 181, 231, 262, and 396 and tau 5 was measured by Western blot.

RESULTSAs the Al(3+) concentration rose, the number of living SH-SY5Y cells decreased, and the synapses of the cells retracted. The viability of cells exposed to 800 µmol/L Al(3+) was significantly lower than that of the control group (P < 0.05). The 200, 400, and 800 µmol/L Al(3+) exposure groups showed significantly higher expression of p-tau 181, 231, and 396 and tau5 than the control group (P < 0.05), and the 800 µmol/L Al(3+) exposure group showed significantly higher expression of p-tau 262 than the control group (P < 0.05).

CONCLUSIONUnder the present experimental conditions, aluminum trichloride has toxic effect on SH-SY5Y cells and can lead to abnormal expression of p-tau 181, 231, and 396 and tau 5 at low Al(3+) concentration.

Aluminum Compounds ; toxicity ; Cell Line, Tumor ; Cell Survival ; drug effects ; Chlorides ; toxicity ; Humans ; Phosphorylation ; tau Proteins ; metabolism

Aluminum Compounds ; pharmacology ; Animals ; Bone and Bones ; drug effects ; metabolism ; Chlorides ; pharmacology ; Decalcification Technique ; methods ; Formates ; chemistry ; Humans

We investigated the effects of simulated ischemia on intracellular Cl- concentration ([Cl-]i) in guinea pig ventricular myocardial cells and possible role of the [Cl-]i on the ischemia/reperfusion-induced arrhythmias in perfused rat hearts. Our results provided direct evidence that the [Cl-]i in ventricular muscle was increased under ischemic conditions, which suggested that activation of the Cl-(-)HCO3- exchanger by ischemia would partially contribute to the elevation of [Cl-]i. Application of stilbene derivatives or lowering Cl- concentration in perfusion solution delayed the onset of ischemia-induced deterioration in action potentials, pHi, [Cl-]i, and suppressed the incidence of ischemia/reperfusion-induced arrhythmias. The conclusion was made to emphasize the important role of intracellular Cl- homeostasis in cardiac physiology and pathogenesis of myocardial ischemia/reperfusion injury.
Action Potentials ; Animals ; Arrhythmias, Cardiac ; etiology ; metabolism ; Chlorides ; metabolism ; Guinea Pigs ; Hydrogen-Ion Concentration ; Microelectrodes ; Myocardial Reperfusion Injury ; complications ; metabolism ; Myocytes, Cardiac ; metabolism ; Papillary Muscles ; cytology