AIM:To report a phenotypic variant pedigree of lattice corneal dystrophy(LCD)associated with two mutations,R124C and A546 D,in the transforming growth factor betainduced gene(TGFBI).METHODS:A detailed ocular exa...AIM:To report a phenotypic variant pedigree of lattice corneal dystrophy(LCD)associated with two mutations,R124C and A546 D,in the transforming growth factor betainduced gene(TGFBI).METHODS:A detailed ocular examination was taken for all participants of a LCD family. Peripheral blood leukocytes from each participant were extracted to obtain the DNA. Polymerase chain reaction(PCR)of all seventeen exons of TGFBI gene was performed. The products were sequenced and analyzed. Histological examination was carried out after a penetrating keratoplasty from the right eye of proband. RESULTS:Genetic analysis showed that the proband and all 6 affected individuals harbored both a heterozygous CGC to TGC mutation at codon 124 and a heterozygous GCC to GAC mutation at codon 546 of TGFBI. None of the 100 control subjects and unaffected family members was positive for these two mutations. Ocular examination displayed multiple refractile lattice-like opacities in anterior stroma of the central cornea and small granular deposits in the peripheral cornea. The deposits were stained positively with Congo red indicating be amyloid in nature and situated mainly in the anterior and middle stroma. CONCLUSION:We observed a novel LCD family which carried two pathogenic mutations(R124C and A546D)in the TGFBI gene. The phenotypic features were apparently different from those associated with corresponding single mutations. The result reveals that although the definite mutation is the most important genetic cause of the disease,some different modifier alleles may influence the phenotype.展开更多
Simulation models of traction driver systems were established using SIMULINK,according to the actual structure and parameters of China Railway High-Speed 2 (CRH2) and China Railway High-Speed 3 (CRH3) trains.In these ...Simulation models of traction driver systems were established using SIMULINK,according to the actual structure and parameters of China Railway High-Speed 2 (CRH2) and China Railway High-Speed 3 (CRH3) trains.In these models,the traction motor adopts transient current control and an indirect rotor magnetic field orientation vector control strategy,and the traction converter uses sinusoidal pulse width modulation (SPWM) and space vector pulse width modulation (SVPWM) methods.After these models are transformed in VC++ program,and a friendly interface and data processing system are constructed,simulation software is obtained for CRH2 and CRH3 traction driver systems.On this basis,the operational performance of a traction converter was simulated and analyzed at different train speeds and in different conditions.The simulation results can provide a reference for the actual design and production of a traction converter.展开更多
Compared to the current eddy braking patterns using a single magnetic source,hybrid excitation rail eddy brakes have many advantages,such as controllability,energy saving,and various operating models.Considering the l...Compared to the current eddy braking patterns using a single magnetic source,hybrid excitation rail eddy brakes have many advantages,such as controllability,energy saving,and various operating models.Considering the large braking power consumption of the high-speed train,a hybrid excitation rail eddy brake system,which is based on the principle of electromagnetic field,is proposed to fulfill the needs of safety and reliability.Then the working processes of the mechanical lifting system and electromagnetic system are demonstrated.With the electromagnetic system analyzed using the finite element method,the factors such as speed,air gap,and exciting current have influences on the braking force and attractive force.At last,the structure optimization of the brake system is discussed.展开更多
A switched-mode unit used in electric locomotive generates a strong high frequency conducted electromagnetic interference (EMI),which radiates electromagnetic energy through railway lines.Evaluation of magnetic field ...A switched-mode unit used in electric locomotive generates a strong high frequency conducted electromagnetic interference (EMI),which radiates electromagnetic energy through railway lines.Evaluation of magnetic field using analytical technique based on contour integral is presented,in order to assess the electromagnetic environment around a high-speed railway.Actual railway multiconductor finitely long overhead lines are represented by an infinitely long single line above two-layered earth,whose characteristic is different from homogeneous earth.Owing to the constraint of the GB/T 24338-2009 and the high frequency investigated (a few MHz),only the magnetic fields are examined.The magnetic fields consist of four components:the direct wave,the ideal reflected wave or image wave,the trapped surface wave,and the lateral wave.The calculation results proved that due to the presence of the trapped surface wave,the magnetic field of the observer point on the interface is strongly influenced,when the line is on or closed to the interface.展开更多
基金Supported by the Ph.D.Programs Foundation of Heilongjiang Province(No.LBH-Q13126)the Research Foundation of the First Affiliated Hospital,Harbin Medical University(No.2011BS017)
文摘AIM:To report a phenotypic variant pedigree of lattice corneal dystrophy(LCD)associated with two mutations,R124C and A546 D,in the transforming growth factor betainduced gene(TGFBI).METHODS:A detailed ocular examination was taken for all participants of a LCD family. Peripheral blood leukocytes from each participant were extracted to obtain the DNA. Polymerase chain reaction(PCR)of all seventeen exons of TGFBI gene was performed. The products were sequenced and analyzed. Histological examination was carried out after a penetrating keratoplasty from the right eye of proband. RESULTS:Genetic analysis showed that the proband and all 6 affected individuals harbored both a heterozygous CGC to TGC mutation at codon 124 and a heterozygous GCC to GAC mutation at codon 546 of TGFBI. None of the 100 control subjects and unaffected family members was positive for these two mutations. Ocular examination displayed multiple refractile lattice-like opacities in anterior stroma of the central cornea and small granular deposits in the peripheral cornea. The deposits were stained positively with Congo red indicating be amyloid in nature and situated mainly in the anterior and middle stroma. CONCLUSION:We observed a novel LCD family which carried two pathogenic mutations(R124C and A546D)in the TGFBI gene. The phenotypic features were apparently different from those associated with corresponding single mutations. The result reveals that although the definite mutation is the most important genetic cause of the disease,some different modifier alleles may influence the phenotype.
基金Project supported by the National Natural Science Foundation of China(No.50877070)the National Key Technology R&D Program of China(No.2009BAG12A01-A04-2)+1 种基金the Technological R&D Programs of the Ministry of Chinese Railways(No.2010J011-E)the Fundamental Research Funds for the Central Universities(No.2009QNA4016),China
文摘Simulation models of traction driver systems were established using SIMULINK,according to the actual structure and parameters of China Railway High-Speed 2 (CRH2) and China Railway High-Speed 3 (CRH3) trains.In these models,the traction motor adopts transient current control and an indirect rotor magnetic field orientation vector control strategy,and the traction converter uses sinusoidal pulse width modulation (SPWM) and space vector pulse width modulation (SVPWM) methods.After these models are transformed in VC++ program,and a friendly interface and data processing system are constructed,simulation software is obtained for CRH2 and CRH3 traction driver systems.On this basis,the operational performance of a traction converter was simulated and analyzed at different train speeds and in different conditions.The simulation results can provide a reference for the actual design and production of a traction converter.
基金Project supported by the National Natural Science Foundation of China(Nos.50877070 and 51105331)the Special Financial Grant from the China Postdoctoral Science Foundation(Nos.201104720 and 201104721)
文摘Compared to the current eddy braking patterns using a single magnetic source,hybrid excitation rail eddy brakes have many advantages,such as controllability,energy saving,and various operating models.Considering the large braking power consumption of the high-speed train,a hybrid excitation rail eddy brake system,which is based on the principle of electromagnetic field,is proposed to fulfill the needs of safety and reliability.Then the working processes of the mechanical lifting system and electromagnetic system are demonstrated.With the electromagnetic system analyzed using the finite element method,the factors such as speed,air gap,and exciting current have influences on the braking force and attractive force.At last,the structure optimization of the brake system is discussed.
基金Project supported by the National Natural Science Foundation of China(Nos.50877070 and 51105331)the Technological Research and Development Programs of the Ministry of Chinese Railways(Nos.2009J006-L and 2010J011-E)
文摘A switched-mode unit used in electric locomotive generates a strong high frequency conducted electromagnetic interference (EMI),which radiates electromagnetic energy through railway lines.Evaluation of magnetic field using analytical technique based on contour integral is presented,in order to assess the electromagnetic environment around a high-speed railway.Actual railway multiconductor finitely long overhead lines are represented by an infinitely long single line above two-layered earth,whose characteristic is different from homogeneous earth.Owing to the constraint of the GB/T 24338-2009 and the high frequency investigated (a few MHz),only the magnetic fields are examined.The magnetic fields consist of four components:the direct wave,the ideal reflected wave or image wave,the trapped surface wave,and the lateral wave.The calculation results proved that due to the presence of the trapped surface wave,the magnetic field of the observer point on the interface is strongly influenced,when the line is on or closed to the interface.