RESUMO
To establish and identify induced pluripotent stem cells (iPSCs) derived from patients with Aicardi-Goutières syndrome (AGS) with TREX1 gene 667G>A mutation, and obtain a specific induced pluripotent stem cell model for Aicardi-Goutières syndrome (AGS-iPSCs). A 3-year-old male child with Aicardi-Goutieres syndrome was admitted to Zhongshan People's Hospital in December 2020. After obtaining the informed consent of the patient's family members, 5 ml peripheral blood samples from the patient were collected, and mononuclear cells were isolated. Then,the peripheral blood mononuclear cells(PBMCs) were transduced with OCT3/4, SOX2, c-Myc and Klf4 by using Sendai virus, and PBMCs were reprogrammed into iPSCs. The pluripotency and differentiation ability of the cells were identified by cellular morphological analysis, real-time PCR, alkaline phosphatase staining (AP), immunofluorescence, teratoma formation experiments in mice. The results showed that the induced pluripotent stem cell line of Aicardi-Goutieres syndrome was successfully constructed and showed typical embryonic stem-like morphology after stable passage, RT-PCR showed mRNA expression of stem cell markers, AP staining was positive, OCT4, SOX2, NANOG, SSEA4, TRA-1-81 and TRA-1-60 pluripotency marker proteins were strongly expressed. In vivo teratoma formation experiments showed that iPSCs differentiate into the ectoderm (neural tube like tissue), mesoderm (vascular wall tissue) and endoderm (glandular tissue). Karyotype analysis also confirmed that iPSCs still maintained the original karyotype (46, XY). In conclusion, induced pluripotent stem cell line for Aicardi-Goutières syndrome was successfully established using Sendai virus, which provided an important model platform for studying the pathogenesis of the disease and for drug screening.
Assuntos
Doenças Autoimunes do Sistema Nervoso , Malformações do Sistema Nervoso , Animais , Pré-Escolar , Humanos , Masculino , Camundongos , Doenças Autoimunes do Sistema Nervoso/metabolismo , Doenças Autoimunes do Sistema Nervoso/patologia , Diferenciação Celular , Linhagem Celular , Células-Tronco Pluripotentes Induzidas/metabolismo , Células-Tronco Pluripotentes Induzidas/patologia , Leucócitos Mononucleares , Malformações do Sistema Nervoso/metabolismo , Malformações do Sistema Nervoso/patologiaRESUMO
Recent studies have revealed the significant influence of finite resistivity on high-energy-density plasmas, contrary to the previous findings of Jukes [J. Fluid Mech. 16, 177 (1963)0022-112010.1017/S0022112063000677]. This paper reexamines Jukes' theory in the context of magneto-Rayleigh-Taylor instability in magnetohydrodynamics with finite resistivity represented by η. The inadequacy of Jukes' approach due to an erroneous boundary condition is demonstrated, and it is shown that although the theory provides some physical insights, it fails to capture crucial features. The dispersion relation proposed in this study highlights that larger growth rates tend to diffuse the magnetic field rapidly, negating its suppressive effect. Moreover, the Atwood number has a significant influence on the growth-rate curves' shape, which differs from those of viscous or elastic flows and ideal magnetohydrodynamics. Additionally, long wavelengths grow proportionally to η^{1/3}, while α indicating growth rates behaves classically when the magnetic field is entirely diffused.
RESUMO
In this paper, the rotational part of the disturbance flow field caused by viscous Rayleigh-Taylor instability (RTI) at the cylindrical interface is considered, and the most unstable mode is revealed to be three-dimensional for interfaces of small radii R. With an increase in R, the azimuthal wave number of the most unstable mode increases step by step, and the corresponding axial wave number increases as well at each step of the azimuthal wave number. When the amplitude of the wave-number vector is much larger or much smaller than 1/R, the cylindrical RTI is close to the semi-infinite planar viscous RTI limit or the finite-thickness creeping-flow RTI limit, respectively. The effect of the viscosity ratio is double-edged; it may enhance or suppress the cylindrical RTI, depending on R and the amplitude range of the wave-number vector.
RESUMO
An electroosmotic pump flow injection system is introduced in this paper. According to electroosmotic theory, the pump's properties were described. A large flow range (mul min(-1)-ml min(-1)), moderate carrier pressure (>0.15 MPa), reduced performance voltage (<500 V) and stable flow rate (RSD<3.0% in 4 h) are the main properties of the pump. NH(4)OH (0.35 mM) was used as carrier for improving the pump's flow stability. The electroosmotic efficiency of the pump's medium, porous core, can be recovered and regenerated. A sandwich zone was used for sample and reagent introduction in order to adapt to the pump performance. Flow injection-spectrophotometry was employed for the determination of Cr(VI) in waste-water, based on the formation of the complex with 1,5-diphenylcarbazide and absorbance measurement at 540 nm. Within the calibration range of 0-7.0 mg l(-1) of Cr(VI), the RSD was 0.4% (n=5). The recovery of 0.70 mg l(-1) Cr(VI) added to the waste-water sample was 94.5+/-2.0% (n=5).