As one of the most serious hereditary neuromuscular disease, spinal muscular atrophy (SMA) is caused by the loss or mutation of survival motor neuron 1 (SMN1) gene. It leads to a decrease in the level of SMN protein and a consequent loss of alpha neurons and progressive muscle atrophy resulting in the progressive muscle weakness, the significant disability and the shortened lifespan. Up till now, only three drugs have been approved for SMA, including the gene therapy drug onasemnogene abeparvovec. The antisense oligonucleotide drug nusinersen and and the small molecule chemical drug risdiplam were briefly introduced. Some representative samples of the small molecule chemical drugs and antisense oligonucleotide drugs targeting SMN2 in the clinical trial or preclinical research phases were also reviewed.

Objective To establish human choriocarcinoma JeG-3 cell line resistant to floxuridine (FUDR)and describe the characteristics of this FUDR-resistant subline.The thymidylate synthase (TS) expression level in FUDR-resistant subline was also discussed.Methods The FUDR-resistant sub-line JeG-3/FUDRA was established by intermitted exposure to grads increased FUDR.Reversed microscope was used to observe the morphological changes in FUDR-resistant sub-line.Population doubling time was calculated and compared based on the growth curve of these two cell lines,cell cycles and chromosomal ploidy were assayed with flow cytometry methods.The chemo-luminescence assay was used to detect the hormone secretion by two kinds of cell lines.The resistant index (RI) was measured by cell counting kit-8 (CCK-8)assay.Quantitative RT-PCR was used to detect the mRNA expression level of TS and we also detected the TS mRNA expression level in different doses exposed subline.Results The RI of JeG-3/FUDRA was 31.62.Compared with the JeG-3 cell,the FUDR-resistant cell line had gross changes in morphological,cell growth,cell cycles and chromosomal numbers.The ability of human chorionic gonadotrop(hCG) and progesterone secretion was lower in JeG-3/FUDRA subline.The trend of TS mRNA expression was:while exposed to low concentration of FUDR,the TS mRNA expression level was downregulated,then followed the increasing dose of the drug,the expression level of TS mRNA ascended gradually.When the terminal concentration was reached,the expression level of TS mRNA in JeG-3/FUDRA subline was higher than that of JeG-3 cell line (P＜0.05).Conclusions We established the FUDR-resistant subline of JeG-3 successfully.The TS mRNA expression level is stage-related to the different concentration and different phase in FUDR exposure.Our data suggested that TS mRNA expression level may not be used as a biomarker to predict the chemosensitivity in FUDR-based chemotherapy.

Adolescent ; Adult ; Angiography, Digital Subtraction ; Child ; Decompression, Surgical ; methods ; Female ; Humans ; Magnetic Resonance Angiography ; Male ; Middle Aged ; Optic Nerve Injuries ; diagnostic imaging ; pathology ; Young Adult

Objective:To obtain a large amount of erythromycin B and to investigate the activity site in eryK. Methods:The key sequence of the BC loop region in eryK gene was knocked out and the eryK gene with 101 bp deleted was amplified by overlapping PCR,and cloned into vector pWHM3 to construct recombinant plasmid. The Saccharopolyspora erythraea mutant AK17 was constructed through chromosomal homologous recombination technique.Results and Conclusions:The S.erythraea mutant AK17 was constructed. The results of TCL and MS analysis showed that the major fermentation product of AK17 is erythromycin B.

Adult ; Air Pollutants ; adverse effects ; Humans ; Male ; Military Medicine ; Monitoring, Physiologic ; Noise, Occupational ; adverse effects

BACKGROUNDPost and post-core systems are used to restore extensively damaged teeth. Among these systems, cast alloy post and core, prefabricated threaded alloy post and prefabricated simple alloy post are most frequently applied in China nowadays. In Europe and North America a combined application of the fiber post, resin-core and crown has been applied to restore seriously destructed teeth with satisfactory results in recent years. This study was intended to evaluate the clinical effect of carbon fiber post system on restoration of child anterior tooth defect after root canal therapy, based on 3 - 5 years' observation.

METHODSOne hundred and six children with incompletely established occlusion were observed and followed for an average of 42 months (ranging from 36 to 60 months). Eighty-five upper teeth and forty-one lower teeth were restored with carbon fiber post system and composite jacket crown. Periodic check-up was conducted for periodontal condition and restoration effect.

RESULTSOne hundred and twenty-one (96.2%) restorations were successful. Four jacket crowns (3.0%) were lost. One tooth (0.8%) had slight gingival inflammation. Tooth root or post fracture and gingival stain were not observed. X-ray showed there was no obvious change in aspects including the width of periodontal membrane, the density of alveolar bone and the height of alveolar ridge crest.

CONCLUSIONSCarbon-fiber post system can satisfy the clinical requirements of young patients who have residual anterior crown and root caused by trauma or caries, and have incomplete occlusion and have completed root canal therapy. This system helps realize good esthetic result for patients and easy practice for dentists. Carbon fiber post is safe and convenient, especially for sick children.

Adolescent ; Carbon ; Child ; Dental Restoration, Permanent ; Humans ; Post and Core Technique ; Root Canal Therapy

BACKGROUNDAlternatives to the conventional auro-galvanoforming ceramic bridge need to be evaluated in long-term longitudinal studies before being recommended for restoration of dentition defects. This study aimed to evaluate the clinical performance of a ceramic bridge with auro-galvanoforming primary coping and Ni-Cr pontic for restoration of dentition defects.

METHODSIn total, 114 ceramic bridges with auro-galvanoforming (primary coping and Ni-Cr pontic) were placed in 112 patients with dentition defects. Among them, 83 bridges in 82 patients were available for follow-up after 7 years. We evaluated marginal integrity, color match, gingival inflammation, fracture of ceramic bridges, and recurrent caries.

RESULTSMarginal integrity of the ceramic bridges was perfect in all patients. Most ceramic bridges maintained their original color. Fracture was found in three bridges; the rest were free of fracture, breakage, and loosening. Recurrent abutment caries were not found. Ceramic bridges with auro-galvanoforming (primary coping and Ni-Cr pontic) produced satisfactory clinical outcomes - equivalent to the conventional porcelain-fused-to-metal restoration. This technique is especially useful for dentition defects of the molar area and is cheaper than restoration with noble metal bridges.

CONCLUSIONSCeramic bridges with auro-galvanoforming (primary coping and Ni-Cr pontic) performed well for the restoration of dentition defects during this follow-up period. More studies are warranted to further evaluate this technique as an alternative to the conventional porcelain-fused-to-metal restoration.

Adult ; Aged ; Cementation ; Ceramics ; Dental Materials ; Dental Occlusion ; Dental Prosthesis ; methods ; Denture Design ; Denture, Partial, Fixed ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Young Adult

In this paper 3 different media (A,for yeast cultivation ; B, for laccase producing; D, for white rot fungi cultivation) were compared in enriching and screening decolorizing fungi culture using Reactive Black 5 (RB5) and Reactive Red (M-3BE) from the following three points: decolorization effects, abilities of producing enzymes and diversity of microbial community. 11 groups of fungi with obvious decolorization effects were obtained after enrichment for near one month. Among them, 6 groups came from medium D, the other two 3 groups from medium A and B, respectively. However, the 3 groups from medium A exhibited the highest microbial diversity and best decolorization results with 99.53% and 97.42% color removal rate of Reactive Red M-3BE and Acid Red. From them, 16 strains of fungi were isolated and primarily identified as Saprolegniaceae, Eurotiaceae (Monascus went), Erysiphaceae and Physodermataceae. Fungi groups from medium B and D exhibited a bit lower color removal rate of various dyes and only 3 and 2 isolates primarily classified as Saccharomycetaceae and Eurotiaceae (Penicillium) were obtained from them. Fungi cultures in medium A and B could produce lignin peroxidase, and those in medium D could be detected higher activity of laccase. All the fungal cultures exhibited very weak activity of manganese dependant peroxidase.

Tumor cell energy metabolism is dependent on glycolysis and oxidative phosphorylation.Tumor cells,because of its rapid growth,often show increased intake of glucose and other nutrients,increased glycolysis and so on.In recent years,the study on energy metabolism of tumor cells has received extensive attention.This paper summarizes the required nutrients,regulatory networks and therapeutic targets in the energy metabolism of tumor cells,and provides important reference for future research and clinical treatment.

Objective To assess the long efficacy and safety of Lamotrigine(LTG) monotherapy and add-on therapy different types of epileptic seizures in children.Methods According to the classification of the 1981 and 1989 International Union of Antiepileptic for epileptic seizure,a total of 124 cases with epilepsy were included in the study and divided into non-refractory group with 93 cases and refractory group with 31 cases.LTG treatment only or add-on were used.Original drug dosage was not changed and LTG was added slowly and carefully untill the terrible side effect appeared.The average monthly seizure frequency with baseline in the last 3 months was compared and the side effect was observed.Results Total efficiency was 72.6%,control rate was 51.6%.Total efficiency and control rate of the non-refractory group was 81.0% and 61.3%,which was significantly higher than those of refractory group(48.4%,22.6%).Total efficiency and control rate of the combination group with LTG and valproate sodium(VPA) was 78.4% and 54.5%,which was significantly higher than those of the group of LTG only(61.0%,44.0%).Clinical results was different significantly between the course of the observation period within and over 5 years(P