Amino Acid Metabolism, Inborn Errors ; Humans ; Mitochondrial Diseases ; Sarcosine Dehydrogenase ; deficiency

Bone and Bones ; pathology ; Humans ; Immunologic Deficiency Syndromes ; diagnosis ; Osteochondrodysplasias ; diagnosis

The human ovarian mucinous cystadenocarcinoma (hOMC) cells were co-cultured with antisense oligodeoxynucleotide (antisense ODN), nonsense ODN, and follicle-stimulating hormone (FSH) at different concentrations for the purpose of observing the effects of antisense ODN to FSH receptor (FSHR) on the proliferation and apoptosis of cultured hOMC cells in vitro. The inhibitory rates of growth were measured by using MTT method on the 2nd, 4th, 6th, 8th and 10th days after the interference of antisense ODN, nonsense ODN, and FSH, respectively. The apoptotic rates and the cell cycles were determined by means of flow cytometry, the apoptosis indexes were detected by using TUNEL, and the expression of caspase-3 was measured by using SP immunohistochemistry. Compared with that in the control group, the proliferative activity of hOMC cells was increased obviously in FSH groups (P<0.05 or P<0.01), decreased distinctly in antisense ODN groups (P<0.05 or P<0.01), and unchanged in nonsense ODN groups, respectively. Meanwhile, antisense ODN could significantly antagonize the FSH-promoted cell proliferative activity (P<0.01). Compared with those in the control group, the apoptotic rates and the expression of caspase-3 were dramatically increased in the mid-and high-dose antisense ODN groups (P<0.05 or P<0.01), while the number of cells in G(1)/G(0) phase was significantly decreased and that in S phase distinctly increased (P<0.01). There was no change in nonsense ODN groups (P>0.05). It was suggested that FSH may improve the development of hOMC cells. However, antisense ODN could inhibit proliferative activity and the FSH-promoted proliferative activity in hOMC cells, at the same time, antisense ODN could inhibit hOMC cell growth by inducing apoptosis.

AIM: To explore if metabotropic glutamate receptors (mGluRs) i nduces neuroprotection against 6-hydroxydopamine (6-OHDA) neurotoxicity in PC1 2 cells. METHODS: The alteration of the glutamate in extracellul ar fluid of PC12 cells was detected by high performance liquid chromatography (H PLC) with a fluorescent detector. The cytotoxic activity of PC12 cells was assay ed by means of MTT colorimetric method. RESULTS: 6-OHDA dose-d ependently increased glutamate release and decreased cell activity of PC12 cells , and the mGluRs ligands had no significant effect on that. CONCLUSION: The mGluR3 ligands has no protective effect on neurotoxicity of PC12 cel ls induced by 6-OHDA.

AIM To study the relationship between the activity of glutamate transporters and Parkinson's disease (PD), examine whether a novel ATP sensitive potassium (K ATP) channel opener Iptakalim (Ipt) hydrochloride enhances glutamate uptake activity, and to investigate its mechanisms. METHODS Rats were stereotaxically injected with 6 hydroxydopamine (6 OHDA) in SNpc. The synaptosomes from normal and PD rats were isolated, and [ 3H] glutamate uptake in synaptosomes was measured by using liquid scintillation counting. RESULTS [ 3H] glutamate uptake by synaptosomes from striatum and cerebral cortex of PD rats decreased and the redution was recovered by administration with Ipt (10, 50, 100 ?mol?L -1 ). The protective effect of Ipt was blocked by co adiministration with glibenclamide (20 ?mol?L -1 ), an inhibitor of sulphonylurea receptors. CONCLUSION Our results demonstrate for the first time the protective role of K ATP channel in glutamate uptake of synaptosomes and conceptually support the view that Ipt may have potential and feasibility in therapy for PD.

The numerous noxious reactions caused by misusing medicinal plant ingredients have been widely concerned at home and abroad. The problems of safe drug use needed to be solved. DNA barcode is a powerful molecular identification method, which can cover the shortage of tradditional morphological and chemical identification. In recent years, DNA barcode provided new aspects for the identification of Chinese herbal medicines and have obtained outstanding achievement. Several popular DNA regions ( ITS, ITS2, ps-bA-trnH, rbcL and matK) have been used for the identification of Chinese herbs.

Objective To discuss the clinical manifestations,imaging features and prognosis of children with mild encephalitis/encephalopathy with a reversible splenial lesion(MERS).Methods Twenty-five patients with MERS admitted to Beijing Children′s Hospital,Capital Medical University,between November 2013 and March 2016 were enrolled and their clinical and imaging data were retrospectively analyzed.Ages of onset of these 25 cases were from 6 months to 13 years old.Because of different clinical manifestations in different onset ages,these 25 cases were divided into 2 groups:≤6 years old group (20 cases),with the onset age of 6 months to 3 years and 9 months old(average 2 years and 2 months);>6 years old group(5 cases),with the onset age of 9 years 3 months to 13 years old (average 10 years and 10 months).Results Nineteen cases among the 25 patients had infection history before onset,including 10 cases of digestive tract infection(all were ≤6 years old children),9 cases of respiratory tract infection(6 children ≤6 years old and 3 children >6 years old).The main clinical manifestations included convulsion (18/25 cases,72.0%),fever (17/25 cases,68.0%),vomiting (11/25 cases,44.0%),and disturbance of consciousness (11/25 cases,44.0%).The main clinical manifestation of ≤6 years old group was convulsion (18/20 cases,90.0%),while the main clinical manifestations of the>6 years old group were fever(3/5 cases,60.0%),headache and dizziness(2/5 cases,40.0%),and none of the patients in >6 years old group had convulsion.Eight cases had liver function injury,myocardial enzymes increased in 10 cases,and hyponatremia occurred in 9 cases.Magnetic resonance imaging (MRI) showed 21 cases were type Ⅰ MERS(only involving corpus callosum),and 4 cases of type Ⅱ MERS which involved corpus callosum as well as deep brain white matter,subcortical white matter (centrum semiovale).MRI lesions disappeared after 8-56 days (average 16.5 days) of anti-infection and reducing intracranial pressure treatment.Conclusion MERS is more common in ≤6 years old children,and digestive tract infection is common in ≤6 years old children,while respiratory tract infection is common in >6 years old children.The symptoms in children are mainly manifested as fever,convulsion,vomiting,conscious disturbance,and so on.Infection and hyponatremia are the main causes of MERS in children.MRI is the first choice of imaging examination methods.

OBJECTIVE:To observe the curative effect and safety of compound glycyrrhizin injection in the treatment of neonatal hepatitis syndrome(NHS).METHODS:68 neonates with NHS were randomly divided into treatment and control gro_up(n=34),undertaking intravenous administration of glycyrrhizin injection and shengmai injection respectively,both at a dose of 3ml/(kg?d) for 2 weeks consecutively.RESULTS:The liver function in the treatment group after treatment was significantly better than that in the control group(P

Objective: To analyze the clinical and genetic features of progressive cavitating leukoencephalopathy (PCL). Method: The data of clinical and genetic features of 4 PCL patients diagnosed by Beijing Children′s Hospital between January 2015 and January 2016 were analyzed. The cases with complete clinical data retrieved on literature search at China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform and PubMed (up to August 2016) by using search terms of"NDUFV1" ,"NDUFS1" , or"leukoencephalopathy" , were summarized. Result: There were three females and one male, two of which were compatriots. The age of onset ranged from 6 months to 15 months. All four children′s first symptoms were motor development regression, and the developmental milestones were almost normal before the onset. Of the 4 patients, 3 had cognitive impairment, 1 had seizures, 4 had dystonia and pyramidal impairment, 2 had emaciation, and 1 had nystagmus. The lactate concentrations of 4 patients were normal in blood. One patient had lactaciduria in the urinary organic acid analysis. Cranial magnetic resonance imaging (MRI) of all patients showed leukoencephalopathy, involved in the corpus callosum, and three patients accompanied by cystic lesions. Follow up for 2-13 years showed that the physical and language development were improved. Genetic analysis revealed that mutations in NDUFS1 were found in three patients and NDUFV1 mutation was found in one patient. All six mutations (p.Arg377Cys and p. Arg377His in NDUFV1; p. Arg482Glyfs^*5, p.Thr368Pro, p.Tyr454X and p. Asp565Gly in NDUFS1) are novel. Five English case reports including 10 PCL patients were collected. Together with this group of 4 cases, a total of 14 cases were involved. All 14 children patients had motor development regression, 11 cases had cognitive impairment and dystonia, 6 cases had pyramidal impairment, 5 cases had irritability, 4 cases had epilepsy and nystagmus, 3 cases had strabismus and swallowing difficulty. Cranial MRI showed patchy leukoencephalopathy with cavities, involved in the corpus callosum. Follow up for 19 months-15 years that the neurology development were improved slowly in all patients. Conclusion NDUFS1 and NDUFV1 gene mutation screening should be performed firstly in patients with PCL clinical and imaging feature.

OBJECTIVETo investigate the representation methods of dissolubility property of total alkaloid extract from Peganum hamala in different solvents, and to investigate the evaluation method of the dissolubility property of extracts from traditional Chinese medicine.

METHODThe dissolubility property of the whole extract and markers of harmaline and harmine, as well as the particle diameter distribution of the extract in different solvents were evaluated by precipitation method, solubility test, and the particle diameter test.

RESULTBoth the alkaloid extract and it's index ingredients had good solubility in absolute ethanol, 95% ethanol, and 80% ethanol, while the solubility in 60% ethanol was poor, and worst in water. The sequence of particle diameter of extract in solvents was in the following order water > 95% ethanol > 60% ethanol > 60% ethanol > 80% ethanol.

CONCLUSIONThe extract has good solubility in the ethanol solution whose concentration is over 80%. The results between precipitation method and index components method have certain correlation. The particle diameter method can provide distribution information of the extract in different solvents. Combination of those three methods could reflect the dissolubility property of extracts from traditional Chinese medicine more comprehensively.

Alkaloids ; chemistry ; isolation & purification ; Chemical Fractionation ; methods ; Particle Size ; Peganum ; chemistry ; Plant Extracts ; chemistry ; isolation & purification ; Solubility ; Solvents ; chemistry