Animals ; Base Sequence ; Capsid Proteins ; genetics ; Carcinoma ; Genes, Tumor Suppressor ; Male ; Mice ; Mice, Inbred BALB C ; Mice, Nude ; Molecular Sequence Data ; Nasopharyngeal Neoplasms ; genetics ; therapy

Objective To report the clinical and pathological characteristics of one patient with glycogen storage disease Ⅳ (Anderson disease). Methods The patient was received detailed clinical examinations, ultrasound, electromyography, head MRI and muscle biopsy. Results The onset of the 22 years old male patient was 7yrs. The main symptoms were intolerance and fatigue in proximal limbs muscular movement, cardiopalmus by chance. Abdominal ultrasound examinations showed cirrhosis, portal hypertension, splenomegaly. Echocardiogram showed left ventricular myohypertrophia, mild mitral and tricuspid valve insufficiency. Electrophysiology study revealed widespread myogenic changes. Cranial MRI, MRA and MRS were normal. Muscle biopsy showed basophilic intracytoplasmic material in a lot of fibers deposits, which was intensively PAS-positive material and partially resistant to diastase digestion. In the electron microscope, the storage material consisted of filamentous and finely granular material. Conclusions There was the first case of glycogen storage disease Ⅳ reported in our country, mainly involved skeletal muscle, liver, spleen and cardiac muscle.

This study is to investigate the mechanism of human promyelocytic leukemia HL-60 cells proliferation induced by alteronol in vitro. Human promyelocytic leukemia HL-60 cells cultured in vitro were treated with different concentrations of alteronol. Inhibition rate was detected by SRB assay. Cellular morphological changes were observed by Hoechst and AO/EB (acridine orange/ethidium bromide dye) staining. The apoptosis rate was determined by Annexin V-FITC/PI assay. Cell cycle distribution was determined by flow cytometry. Western blotting analysis was carried out to determine the cell cycle related proteins. The proliferation of HL-60 cells treated with alteronol was inhibited in a concentration-dependent manner. Based on cell viability assay, observation on cell morphology and apoptosis rate, it confirmed that alteronol played an obvious role in proliferation inhibition of human promyelocytic leukemia HL-60 cells, but it did not induce apoptosis in human promyelocytic leukemia HL-60 cells in different concentrations groups. Alteronol could effectively inhibit the proliferation of human promyelocytic leukemia HL-60 cells inducing cell cycle arrest at G1 phase, as well as, alteration expression of cell cycle proteins level of CyclinD1 and pRb.

[Objective] To investigate the role SPAG5 play in ovarian adenocarcinoma cell mitosis,Taxol sensitivity and ovarian high grade serous carcinoma patients' prognosis.[Methods] Transient knockdown of SPAG5 in SKOV3 cell were performed,and MTT assay and cell cycle flow cytometry assay were carried out.IHC staining of SPAG5 protein in 110 high grade serous carcinoma patients' tumor tissues were performed,and the expression were analyzed with clinical data and prognosis.Finally,SPAG5 were knocked down in OVCAR3 A2780 and SKOV3 cells followed by 0.5μM Taxol treatment,MTT assay were performed to detect cell viability.[Results] SPAG5 knockdown inhibited cell mitosis of ovarian adenocarcinoma cell SKOV3 by G2/M arrest.High grade serous carcinoma patients after neoadjuvant chemotherapy gained the expression of SPAG5.Patients without neoadjuvant chemotherapy with low SPAG5 expression have poor progress free survival,especially in early stage patients.Patients with low SPAG5 expression also have poorer overall survival,but the difference was not statistically significant.Furthermore,SPAG5 knockdown in OVCAR3 A2780 and SKOV3 cells reduced Taxol sensitivity.[Conclusion] SPAG5 regulated cell mitosis and promoted cell proliferation in ovarian adenocarcinoma cell lines.Expression of SPAG5 in patients' tumor tissues predicted patients' prognosis and Taxol sensitivity.As the results,individualized treatment of high grade serous carcinoma patients is necessary.

Objective To report the development of clinical symptoms in a Chinese family with autosomal dominant progressive external ophthalmoplegia(adPEO).Methods Electromyologram and muscle biopsy were performed in the proband and 4 family members with the disease.Results The proband was a 57 year-old woman,who developed bilateral ptosis after the age of 30,external ophthalmoplegia after the age of 35 years old,weakness of extremities at the age of 37 years old and bulb palsy with palmus at the age of 47 years old.In the family there were 20 male and female members from five generations.All of them complained about bilateral ptosis between 26—33 years old,external ophthalmoplegia(12/15)and weakness of all extremities(14/15)between 35—45,facial and masticatory weakness(9/9)as well as dysphagia(8/9)between 44—60,accompanied with heart lesions(4/7)after 50 years old.Some patients died due to cardiac impairment.Electromyologram showed myopathic abnormalities in the examined patients. The main myopathological changes were ragged red fibers,cytochrome c oxidase negative fibers and ragged blue fibers in succinate dehydrogenase staining.Conclusions The adPEO started from extra-ocular muscles to limbs,finally facial and bulbar muscles.Heart lesions were presented in late stage and lead to death in some members.The developing process of symptoms suggested that we should pay more attention to cardiac manifestations in this disease.

Objective To analysis the clinical manifestations of mtDNA A3243G mutation in adulthood.Methods The clinical features were investigated in 36 cases (28 patients from 5 families with the mutation and 8 sporadic cases),in whom mtDNA A3243G mutation was confirmed genetically in 23 cases (15 cases from 5 mutation families and 8 sporadic cases).Cranium radiology was performed in 14 cases.Muscal biopsies were performed in l0 cases.Results Among 28 cases in the 5 family,there were 9 cases (32.1%) with stroke like episodes,17 cases (60.7%) with diabetic mellitus and 16 cases (57.1%) with deafness.Such symptoms usually combined with each other and rarely existed alone. Cardiomyopathy and renal failure were uncommon.In the 23 cases with mtDNA A3243G mutation,14 cases (61.0%) had mitochondria] myopathy,encephalopathy,lactic acidosis,and stroke-like episodes (MELAS),mostly presenting cognitive abnormalities,dysarthria or aphasia and headache,3 cases (13.0%) were asymptomatic carriers,2 cases (8.7%) had autonomic dysfunction,2 cases (8.7%) had diabetic mellitus with or without nerve deafness,1 case (4.3%) had diabetic mellitus with infertilitas and cardiomyopathy,respectively.Cranial radiological images revealed the changes more commonly in the temporal and occipital lobes and less frequently in the frontal lobes.Ragged red fibers were confirmed in 9 of 10 cases with muscle biopsies.The proportion of mutant mtDNA A3243C was not significantly different between MEALS (28.75%?13.69%) and non-MELAS (25.08%?11.54%).Conclusions mtDNA A3243G mutation mainly results in the lesions in the central nerve system,pancreatic island and acoustic nerve in adulthood.Heart and kidney are less frequently involved.Cognitive abnormalities,aphasia and headache are the major symptoms of adult MELAS.Families have with more than 1 patient with diabetic mellitus and deafness,indicating that the mutation is other than MELAS mutation.We should pay more attention to the non-MELAS symptoms in the families with mtDNA A3243G mutation.

OBJECTIVETo explore the recording method of the electrophysiological signals in corticospinal tract (CST) of adult rats by plugging microelectrodes and analyze the characteristics of these signals. These could provide some valuable and basic neural electrophysiological information for further research of recovering and refunctioning after spinal cord injury.

METHODSThe microelectrodes were plugged into the corticospinal tract at the T8 spinal section of Sprague-Dawley rats and the neuro-electrical signals were identified and recorded from CST by means of the Cerebus System. The characteristics of the recorded signals were described with the help of the Offline sorter and Neuroexplorer softwares, including the wavelength, amplitude, discharging frequency, the synchrony among the multi-discharging units from the same electrode and two different electrodes, analysis of interspike interval (ISI), etc.

RESULTSThe continuous and steady spontaneous electrophysiological signals were recorded from CST. Three or four types of discharging signals originated from different discharging units were collected with each electrode. The waveform of the signals appeared bidirectional. The wavelengths were 0.6 - 1.3 ms with wave amplitudes at a grade of hundred microvoltage and high signal-noise ratios. The LFB staining proved that the electrodes were accurately plugged into the corticospinal tract.

CONCLUSIONThe neuro-electrical signals at a grade of hundred microvoltage could be recorded stably from the corticospinal tract of rats by the Cerebus System with the microelectrodes, which provided valuable and basic neural electrophysiological information for further research on recovering and refunctioning after spinal cord injury (SCI) by analyzing the characteristics of electrophysiological signals.

Animals ; Electrodes, Implanted ; Electrophysiological Phenomena ; physiology ; Evoked Potentials, Motor ; physiology ; Male ; Microelectrodes ; Pyramidal Tracts ; physiology ; Rats ; Rats, Sprague-Dawley ; Spinal Cord ; physiology ; Spinal Cord Injuries ; physiopathology

Objective To investigate the possibility of increasing R0 resection rate in esophageal carcinoma after neoadjuvant chemotherapy. Methods 30 patients underwent operation after neoadjuvant chemotherapy mainly by paclitaxel combined with cis-platinum, including 18 squamous carcinoma cases. 10 adenocarcinoma cases and 2 small cell carcinoma cases.Results In these 30 patients,the overall effective rate of neoadjuvant chemotherapy is 56.7%(17/30)(CR+PR),R0 resection rate is 100%,and the incidence rate of anastomotic leakage is 3.3%(1/30),without perioperative death.Conclusion The neoadjuvant chemotherapv is significant in improving the R0 resection rate in esophageal carcinoma,and the impact of long-term survival after surgery needs further follow-up.

ObjectiveTo investigate the effect of magnesium sulfate combined with monosialoganglioside on the recovery of motor function after spinal cord injury in rats.Methods48 healthy adult rats were randomly divided into groups A, B, C and D, and SCI was made by Allen's mode(10 g×25 mm) on spinal cord T9 extradually, 12 rats in each group. On the 1st d, 3rd d and 7th d after SCI, the recovery of motor function after spinal cord injury in rats was assessed with Basso-Beattie-Bresnahan(BBB)scale and slanting board test. Thiobarbituric acid was used to detect the concentration of malondialdehyde, and was observed the change of free radicals.ResultsAfter spinal cord injury in rats, BBB scores and slanting board test of groups A, B and C were better than group D. BBB scores and slanting board test of group C was better than groups A and B, which had significant difference on the 3rd d and 7th d after injury(P<0.05). After spinal cord injury in rats, concentration of malondialdehyde of groups A, B and C were lower than group D(P<0.05). Concentration of malondialdehyde of group C was lower than groups A and B, which had significant difference after injury(P<0.05).ConclusionMagnesium sulfate combined with GM1 can promote the recovery of motor function early after spinal cord injury in rats, and is superior to magnesium sulfate or GM1.