Objective To investigate the clinical, electromyographic and cervical magnetic resonance imaging (MRI) characteristics of Hirayama disease. Methods Fifteen patients with Hirayama disease were observed for special clinical manifestations and underwent electromyographic examination of the bilateral distal upper limb muscles and peripheral nerve conduction velocity. MRI of the neck in neutral and fully flexed positions was performed to identify potential lower cervical cord atrophy and cervical curvature anomalies. Results All the 15 male patients had disease onset during puberty with asymmetric muscular atrophy and weakness of the hands and forearms. Concentric needle electromyography revealed prolonged duration and large amplitude of the motor unit potentials in the compromised distal limb muscles with also increased polyphasic potentials and poor recruitment, involving mainly the C7, C8 and T1 myotomes. In neutral neck position, MRI identified lower cervical cord atrophy in 9 patients, occurring mainly at C5, C6 levels;in fully flexed position, all patients showed forward displacement and flattening of the lower cervical cord, occurring mostly at C6 level. Conclusion Hirayama disease occurs mainly in puberty in young male patients, whose clinical features and electromyographic examination often indicate localized anterior horn anomalies in the lower cervical cord. MRI of the neck in neutral and fully flexed position can provide valuable assistance in the diagnosis of this disease.

The effect of magnetic Fe3O4 particles on cellulase in the enzymatic hydrolysis of sunflower seed hull was studied in different adding ways and additive amount. In the process of enzymatic hydrolysis of sunflower seed hull, the variations of cellulase activity, reducing sugar concentration and cellulose conversion were evaluated. After the reaction, the analysis of pH and surface tension of hydrolysate were also used to determine the mechanisms of cellulase by the magnetic effect. The results indicated that after adding magnetic Fe3O4, the cellulase activity, reducing sugar concentration and conversion of cellulose had an increased between the 0.5 g/L and 2.0 g/L cases after 48 h. When the additive amount of magnetic Fe3O4 was 2 g/L, the cellulase activity at 60 h was improved significantly by 25.9%. It was found that the concentration of reducing sugar was increased from 6.950 mg/mL to 8.775 mg/mL with magnetic Fe3O4 1.5 g/L. Simultaneously, compared with the blank, which the conversion of cellulose was 47.932%, the maximum celluloseconversion of samples with adding magnetic Fe3O4 was 60.531%. Besides, the stability of cellulase activity adding in times was better than in one time. After the reaction, the final surface tension of hydrolysate with 1.5 g/L magnetic Fe3O4 was the lowest in comparison with the blank. However, no significant differences were observed in the final pH of the hydrolysate.

OBJECTIVETo express and purify of the BC097361 recombinant protein, and to prepare the BC097361 specific rabbit polyclonal antibody.

METHODSBC097361 cDNA was ligated into the prokaryotic expressive vector pET-32a (+), and the resulting plasmid was transformed into E.coli BL21 (DE3). The protein expression was induced with IPTG and the protein was analyzed with SDS-PAGE and western blotting. The expressed product was purified using Ni+ affinity column chromatography.Then the purified pET-32a (+) -BC097361 fusion protein was used to immunize New Zealand rabbits to gain polyclonal antibody. The specificity and potency of polyclonal antibody were evaluated by Western blot and ELISA.

RESULTSThe BC097361 fusion protein was highly expressed.The protein was mainly in the inclusion body. ELISA indicated the titer of polyclonal antibody more than 1:320000. The high specificity was confirmed with Western blot.

CONCLUSIONSThe recombinant BC097361 fusion protein and the BC097361 specific polyclonal antibody will be valuable tools for the investigation on the biological function of BC097361.

Angiotensin II ; genetics ; Animals ; Antibodies ; immunology ; isolation & purification ; metabolism ; Antibody Specificity ; Blotting, Western ; Cloning, Molecular ; Escherichia coli ; genetics ; metabolism ; Gene Expression ; Genetic Vectors ; genetics ; Liver Cirrhosis ; genetics ; Male ; Plasmids ; genetics ; Rabbits ; Recombinant Fusion Proteins ; biosynthesis ; immunology ; Reverse Transcriptase Polymerase Chain Reaction

AIMTo study the effect of chronic hypoxic hypercapnia on expression of COX-2 mRNA in pulmonary arterioles.

METHODSSD rats were randomly divided into two groups: control group and hypoxic hypercapnic group. COX-2 mRNA was observed in pulmonary arterioles by the technique of in situ hybridization.

RESULTSmPAP, weight ratio of right ventricle (RV) to left ventricle plus septum (LV + S) and COX-2 mRNA in pulmonary arterioles were much higher in rats of hypoxic hypercapnic group than those of control group. Light microscopy showed that vessel smooth muscle cell hypertrophy and vessel cavity straightness were found in hypoxic hypercapnic group.

CONCLUSIONChanges of expressions of COX-2 mRNA may regulate hypoxic hypercapnic pulmonary hypertension.

Animals ; Cyclooxygenase 2 ; genetics ; metabolism ; Hypercapnia ; metabolism ; physiopathology ; Hypoxia ; metabolism ; physiopathology ; Male ; Pulmonary Artery ; metabolism ; physiopathology ; Rats ; Rats, Sprague-Dawley

Coal ; Dust ; analysis ; Gases ; adverse effects ; Humans ; Male ; Middle Aged ; Occupational Exposure ; analysis ; Pulmonary Alveolar Proteinosis ; etiology

OBJECTIVETo analyze the effect of endophytic fungi on expression amount of key enzyme genes SS (squalene synthase gene), SE (squalene epoxidase gene) and bAS (beta-amyrin synthase gene) in saponin biosynthesis and saponins content in Eleutherococcus senticosus.

METHODWound method was used for back meeting the endophytic fungi to E. senticosus. With GAPDH as internal control gene, the expression of key enzyme genes was detected by real time PCR method. E. senticosus saponins content was measured by spectrophotometry method.

RESULTWhen wound method back meeting P116-1a and P116-1b after 30 d, the expression content of SS improved significantly (P < 0.05), however the back meeting of P109-4 and P312-1 didnt change the expression of SS. After that SS expression showed reduction-equality-reduction varying trend. Thirty days after back meeting P312-1, the expression content of SE improved significantly (P < 0.05). Ninty days after back meeting P116-1b and P312-1, the expression content of SE improved significantly to 130%,161%, respectively (P < 0.05). After 120 d, back meeting four endophytic fungi, the expression of SE were significantly higher than the control (P < 0.05). Back meeting four endophytic fungi form 60 d to 120 d, the expression of bAS was significantly higher than the control (P < 0.05). The back meeting four endophytic fungi improved E. senticosus saponins content significantly (P < 0.05).

CONCLUSIONEndophytic fungi P116-1a, P116-1b, P1094 and P312-1 significantly effected the expression of key enzyme genes SS, SE and bAS and then affected E. senticosus saponins content. Among the genes, bAS was key target gene.

Eleutherococcus ; chemistry ; metabolism ; microbiology ; Endophytes ; physiology ; Farnesyl-Diphosphate Farnesyltransferase ; genetics ; Fungi ; physiology ; Gene Expression Regulation, Enzymologic ; Gene Expression Regulation, Plant ; Intramolecular Transferases ; genetics ; Saponins ; analysis ; biosynthesis ; Squalene Monooxygenase ; genetics

OBJECTIVETo clone farnesyl diphosphate synthase (FPS) gene from Eleutherococcus senticosus and analyze the bioinformatics and expression of the gene.

METHODThe FPS full length cDNA was cloned by rapid amplification of cDNA ends (RACE). The data was analyzed by bioinformatics method, the structure and function of FPS was deduced. The expression of FPS in different organ of E. senticosus was detected by RT-PCR.

RESULTThe full length of FPS cDNA was 1 499 bp containing a 1 029 bp ORF that encoded 342 amino acids. The deduced protein sequence exhibited two Asp riches conserved motifs (DDXXD). Without transmembrane domain, FPS was located in cytoplasm. RT-PCR result showed that FPS gene expressed in different organs of E. senticosus. The expression amounts of FPS in different organs were different significantly (P < 0.05).

CONCLUSIONThe FPS gene of E. senticosus was successfully cloned for the first time, and provided a stable foundation for studying on its effect and expression control on E. senticosus saponins biosynthesis.

Amino Acid Sequence ; Cloning, Molecular ; Computational Biology ; Conserved Sequence ; Eleutherococcus ; enzymology ; genetics ; Gene Expression Regulation, Plant ; Geranyltranstransferase ; chemistry ; genetics ; metabolism ; Models, Molecular ; Molecular Sequence Data ; Phylogeny ; Protein Conformation

AIMTo study the effect of aspirin on chronic hypoxia and hypercapnic pulmonary hypertension.

METHODSSD rats were randomly divided into normal control group (A), hypoxic hypercapnic group (B), hypoxic hypercapnia + aspirin group (C). The concentration of TXB2 and 6-keto-PGF1alpha in plasma and in lung were detected by the technique of radioimmunology.

RESULTS(1) mPAP was significantly higher in B group than those of A and C group. Differences of mCAP were not significant in three groups. (2) Light microscopy showed that WA/TA (vessel wall area/total area) and PAMT (the thickness of medial smooth cell layer) were significantly higher in B group than those of A and C group. (3) The concentration of TXB2 and 6-keto-PGF1alpha in plasma and lung as well as the ratio of TXB2/6-keto-PGF1alpha were significantly higher in rats of B group than those of A and C group.

CONCLUSIONAspirin may inhibit hypoxic hypercapnia pulmonary hypertension and pulmonary vessel remodeling.

6-Ketoprostaglandin F1 alpha ; metabolism ; Animals ; Aspirin ; pharmacology ; Carotid Arteries ; pathology ; physiopathology ; Epoprostenol ; metabolism ; Hypercapnia ; physiopathology ; Hypertension, Pulmonary ; metabolism ; pathology ; physiopathology ; Hypoxia ; physiopathology ; Male ; Pulmonary Artery ; pathology ; physiopathology ; Rats ; Rats, Sprague-Dawley ; Thromboxane A2 ; metabolism