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1.
The great majority of genetic disorders are caused by defects in the nuclear genome. However, some significant diseases are the result of mitochondrial mutations. Because of the unique features of the mitochondria, these diseases display characteristic modes of inheritance and a large degree of phenotypic variability. Recent studies have suggested that mitochondrial dysfunction plays a central role in a wide range of age-related disorders and various forms of cancer.  相似文献   

2.
Objective: Leber's hereditary optic neuropathy (LHON) is a maternally inherited degeneration of the optic nerve caused by point mutations of mitochondrial DNA (mtDNA). Many unsolved questions regarding the penetrance and pathophysiological mechanism of LHON demand efficient and reliable mutation testing. This study aims to develop a minor groove binder (MGB) probe assay for rapid detection of mtDNA11778 mutation and heteroplasmy in Chinese LHON patients by real-time polymerase chain reaction (PCR). Methods: Forty-eight patients suspected of having LHON and their maternal relatives underwent a molecular genetic evaluation, with 20 normal individuals as a control group at the same time. A real-time PCR involving two MGB probes was used to detect the mtDNA 1 1778 mutation and heteroplasmy. A linear standard curve was obtained by pUCmLHONG and pUCmLHONA clones. Results: All 48 LHON patients and their maternal relatives were positive for rntDNA11778 mutation in our assay, 27 heteroplasmic and 21 homoplasmic. Eighteen cases did not show an occurrence of the disease, while 9 developed the disease among the 27 heteroplasmic mutation cases. Eleven did not show an occurrence of the disease, while 10 cases developed the disease among 21 homoplasmic mutation cases. There was a significant difference in the incidence between the heteroplasmic and the homoplasmic mutation types. The time needed for running a real-time PCR assay was only 80 min. Conclusion: This real-time PCR assay is a rapid, reliable method for mtDNA mutation detection as well as heteroplasmy quantification. Detecting this ratio is very important for predicting phenotypic expression of unaffected carriers.  相似文献   

3.
A sudden increase in permeability of the inner mitochondrial membrane, the so-called mitochondrial permeability transition, is a common feature of apoptosis and is mediated by the mitochondrial permeability transition pore (mtPTP). It is thought that the mtPTP is a protein complex formed by the voltage-dependent anion channel, members of the pro- and anti-apoptotic BAX-BCL2 protein family, cyclophilin D, and the adenine nucleotide (ADP/ATP) translocators (ANTs). The latter exchange mitochondrial ATP for cytosolic ADP and have been implicated in cell death. To investigate the role of the ANTs in the mtPTP, we genetically inactivated the two isoforms of ANT in mouse liver and analysed mtPTP activation in isolated mitochondria and the induction of cell death in hepatocytes. Mitochondria lacking ANT could still be induced to undergo permeability transition, resulting in release of cytochrome c. However, more Ca2+ than usual was required to activate the mtPTP, and the pore could no longer be regulated by ANT ligands. Moreover, hepatocytes without ANT remained competent to respond to various initiators of cell death. Therefore, ANTs are non-essential structural components of the mtPTP, although they do contribute to its regulation.  相似文献   

4.
5.
以黄芪和党参为原料组方制成芪参补气药茶(ACT),探索ACT对线粒体的保护作用及其机制。分别用AlCl3比色法和硫酸苯酚法测定ACT的功能因子总黄酮、总糖及总多糖含量。用Ca2+诱导肝线粒体通透性转换(MPT),分光光度法测MPT程度;以Fe2+/维生素C诱发肝线粒体脂质过氧化,采用硫代巴比妥酸显色法测定丙二醛(MDA)含量;测定ACT对Fe2+螯合能力及还原力的影响。实验测得ACT总糖含量为(26 ± 1.3)mg·mL-1,总多糖含量为(12.5 ± 0.8)mg·mL-1,总黄酮含量为(121 ± 8.5)μg·mL-1。结果表明:ACT可抑制Ca2+引起的MPT,可一定程度上抑制线粒体MDA生成,具有较弱的Fe2+螯合能力及一定的还原力。能通过温和的抗氧化和清除活性氧作用,一定的还原力及抑制MPT来保护线粒体,从而维持机体氧化与抗氧化平衡,促进机体健康。  相似文献   

6.
Mitochondria play a critical role in mediating both apoptotic and necrotic cell death. The mitochondrial permeability transition (mPT) leads to mitochondrial swelling, outer membrane rupture and the release of apoptotic mediators. The mPT pore is thought to consist of the adenine nucleotide translocator, a voltage-dependent anion channel, and cyclophilin D (the Ppif gene product), a prolyl isomerase located within the mitochondrial matrix. Here we generated mice lacking Ppif and mice overexpressing cyclophilin D in the heart. Ppif null mice are protected from ischaemia/reperfusion-induced cell death in vivo, whereas cyclophilin D-overexpressing mice show mitochondrial swelling and spontaneous cell death. Mitochondria isolated from the livers, hearts and brains of Ppif null mice are resistant to mitochondrial swelling and permeability transition in vitro. Moreover, primary hepatocytes and fibroblasts isolated from Ppif null mice are largely protected from Ca2+-overload and oxidative stress-induced cell death. However, Bcl-2 family member-induced cell death does not depend on cyclophilin D, and Ppif null fibroblasts are not protected from staurosporine or tumour-necrosis factor-alpha-induced death. Thus, cyclophilin D and the mitochondrial permeability transition are required for mediating Ca2+- and oxidative damage-induced cell death, but not Bcl-2 family member-regulated death.  相似文献   

7.
It has been shown that Bid and its truncated form tBid could induce cytochrome c (cyt c) release without impacting on PTP. We first show that Bid BH3 peptide, but not its mutant form of Bid BH3 peptide G94E, which is unable to bind to Bcl-xL, induces permeability transition pore (PTP) opening in a dose dependent manner. Bid BH3 peptide also induces the reduction of mitochondria membrane potential (ψm) and cyt c release from mitochondrial in vitro.PTP opening and the loss of ψm were inhibited by Bcl-xL,cyclosporin A and ruthenium red, and the latter was an inhibitor of Ca2+ uniporter in the mitochondrial membrane.These results indicate that Bid BH3 peptide could antagonize Bcl-xL to induced PTP opening and mitochondrial dysfunction.  相似文献   

8.
The entire mitochondrial DNA sequence (mitogenome) of Russell's snapper (Lutjanus russellii) was determined using long PCR and primer-walking methodology, representing the first complete mitogenome accessioned for Lutjanid fishes (16,505 bp, GenBank Accession No. EF514208). The mitogenome was similar in gene composition and order to those of other vertebrates, having 37 structural genes, i.e., two ribosomal RNAs, 22 transfer RNAs, and 13 protein-coding genes. Phylogenetic analyses based on the mtDNA sequence of Russell's snapper supported a close relationship between Lutjaninae and Caesioninae, consistent with taxonomic hypotheses based on morphology. More studies utilizing mitogenomes are needed to resolve high-level relationships among snappers.  相似文献   

9.
由于多尺度孔隙存在纳米尺度到宏观尺度的过渡,气体在页岩中流动的主导机制会在达西流和努森扩散等运移机理之间发生转换,该现象结合页岩基质内的气体吸附作用使得页岩气渗透率的测算变得更为复杂.因此,在实验室尺度确定有效应力及气体运移方式对渗透率的综合影响力对实际页岩气开采中的渗透率及产量评估准确性有着不可忽视的影响.采用脉冲衰减渗透率测算法,在不同围压条件下,对氦气和二氧化碳在富含有机质的页岩薄片中的气体渗透率进行测量.结果表明:努森扩散作用在低孔压时对页岩气渗透率有显著的正面影响,其强度与孔压成反比.在较大围压作用下的高孔压下的超临界二氧化碳可导致页岩基质最大吸附量降低,进而增加表观渗透率.表观渗透率随着有效应力的变化在不同孔压区间有着截然相反的趋势,有效应力系数此时出现非单一值,其主要是由于孔压变化带来的气体流动机理变化而引起的.  相似文献   

10.
Dehydration melting of subducted continental crust is significant during exhumation, and its study from both experimental and petrological observations is of great importance to our understanding of continental geodynamics. Dehydration melting experi- ments were carried out on ultrahigh-pressure (UHP) eclogite from Bixiling in the Dabie orogen using a piston cylinder at 1.5-3.0 GPa and 800-950℃ to investigate partial melting of eclogite induced by phengite breakdown. The phengite-bearing eclogite started to melt at T≤800-850℃ and P=1.5-2.0 GPa and produced about 3% granitic melt. The products of dehydration melting vary with temperature and pressure. Such results provide valuable constraints on the micro-texture related to partial melting of UHP rocks in the Dabie-Sulu orogenic belt. Three types of polyphase inclusions were identified in garnet from the Shuanghe UHP eclogite. K-feldspar and quartz inclusions are interpreted to represent the products of segregation and crystallization of minor amounts of melt that formed during dehydration melting of phengite by the inferred reaction Phengite+Omphacite+Quartz Amphibole_+Garnet+Melt (K-feldspar+Quartz+Plagioclase). Polyphase inclusions of phengite and K-feldspar+Quartz inclusions were also found in zoisite/clinozoisite and garnet from the Shuanghe garnet-bearing paragneiss. These polyphase inclusions pro- vide evidence for a continuous process from sub-solidus dehydration to partial melting within the UHP gneissic rocks. The com- positional variation of garnets demonstrates that breakdown of epidote-group minerals may have played a crucial role during de- hydration melting reaction of phengite. The Ti-in-zircon thermometry and Si content of phengite in zircon suggest that partial melting would occur at 783-839℃ and 2.0-2.5 GPa. Therefore, both experimental results and petrological observations indicate that dehydration melting and fluid activity within the Dabie UHP rocks at micro-scale are controlled by the breakdown of phengite.  相似文献   

11.
B3lyp/6-311++g** and mp2/6-311++g** calculations were used to analyze the interaction between CH3SO and HOCl. Nine (complex A: S1A-S9A) and five (complex B: S4B-S7B and S10B) minima were localized on the potential energy surface of CH3SO…HOCl complexes at b3lyp/6-311++g** and mp2/ 6-311++g** computational levels, respectively. The AIM and NBO theories were also applied to explain the nature of the complexes. Bonding energy of complexes A and B corrected with BSSE falls in the ranges of -0.4―-41.4 kJ·mol-1 and -6.9―-35.8 kJ·mol-1 at mp2/6-311++g** level, respectively. The re- sults show that a novel oxygen bond complex (S6) exists in the system, besides hydrogen bond and chlorine bond. Especially, S6B-F, S6B-Br and S7B are blue shifted complexes compared with red shifted S6A, because the electron transfer occurs between LP1(S8) and σ*(O5-Cl7), resulting in the increase of O5-Cl7 and the decrease of vibrational frequency. The complex of S10B has characteristics of both red shift and blue shift.  相似文献   

12.
Phytic acid (PA) is the primary storage compound of phosphorus in seeds accounting for up to 80% of the total seed phosphorus and contributing as much as 1.5% to the seed dry weight. The negatively charged phosphate in PA strongly binds to metallic cations of Ca, Fe, K, Mg, Mn and Zn making them insoluble and thus unavailable as nutritional factors. Phytate mainly accumulates in protein storage vacuoles as globoids, predominantly located in the aleurone layer (wheat, barley and rice) or in the embryo (maize). During germination, phytate is hydrolysed by endogenous phytase(s) and other phosphatases to release phosphate, inositol and micronutrients to support the emerging seedling. PA and its derivatives are also implicated in RNA export, DNA repair, signalling, endocytosis and cell vesicular trafficking. Our recent studies on purification of phytate globoids, their mineral composition and dephytinization by wheat phytase will be discussed. Biochemical data for purified and characterized phytases isolated from more than 23 plant species are presented, the dephosphorylation pathways of phytic acid by different classes of phytases are compared, and the application of phytase in food and feed is discussed.  相似文献   

13.
Objective: This study aimed to explore the effects of different types of palatal lateral excisions on the growth and development of the maxilla and dental arch, and to investigate the underlying mechanisms. Methods: A total of 112 3-week-old Sprague-Dawley (SD) male rats were randomly divided into a control and 3 experimental groups: the mucoperiosteal denudation group, the mucosal flap excision group, and the periosteum excision group. In the experimental groups, bilateral mucoperiosteal, mucosal flap and periosteum were excised respectively in the lateral one half of the palate. Four rats in each group were randomly chosen for sacrifice every two weeks. The maxilla was dissected following the excision. The widths of the maxilla and dental arch were measured and the histological phenomena were investigated at different phases. At the same time, 12 animals in each group were sequentially injected with calcein every two weeks. Three animals in each group, whose fluorescent labeling was used, were sacrificed for investigating bone formation at Week 8 following injection. Results: (1) Each experimental group presented the constriction of the maxilla and dental arch. The upper first molars in the experimental groups inclined medially. The mucoperiosteal denudation group showed the largest degree of effect followed by the periosteum excision group. The indices of the mucosal flap excision group, which retained the structures of the periosteum layer, had the most approximate values to the control group; (2) Different histological changes among the experimental groups were detected. The fibers penetrated into the palatal bone as Sharpey's fibers in the mucoperiosteal denudation group. The pattern of bone deposition was the bundle type. Sharpey's fibers were not found in the mucosal flap and periosteum excision groups and the depositions of palatal bone were the lamellar type as those in the control group; (3) The rates of bone deposition in the experimental groups decreased compared with the  相似文献   

14.
In this paper,a portable 2.42 GHz transmitter for wireless communication systems,with 8dBm output power and small size is proposed.Several novel features exist in this transmitter.First,power consumption and output are balanced by introducing a differential oscillator with input signal controlled biasing,which acts as both a carrier generator and an OOK modulator.Then,power consumption of the transmitter is reduced by the OOK modulated signal via switching the oscillator and the power amplifier at the same time.Furthermore,the area size is also reduced by a class-AB power amplifier,which uses the PCB antenna as the resonance inductance.With these features,the total chip area is reduced to 670μm×740μm(In a 0.18μm CMOS process).  相似文献   

15.
The modelling and simulation of deformable objects is a challenging topic in the field of haptic rendering between human and virtual environment. In this paper, a novel and efficient layered rhombus-chain-connected haptic deformation model based on physics is proposed for an excellent haptic rendering. During the modelling, the accumulation of relative displacements in every chain structure unit in each layer is equal to the deformation on the virtual object surface, and the resultant force of corresponding springs is equivalent to the external force. The layered rhombus-chain-con- nected model is convenient and fast to calculate, and can satisfy real-time requirement due to its simple nature. Simulation experiments in virtual human liver based on the proposed model are con- ducted, and the results demonstrate that our model provides stable and realistic haptic feeling in real time. Meanwhile, the display result is vivid.  相似文献   

16.
The hepatoprotective potential of earthworm extract (EE) (Lampito mauritii, Kinberg) was evaluated against paracetamol-induced liver injury in Wistar albino rat, in comparison with silymarin, the standard hepatoprotective drug. We observed a reduction in liver antioxidants, such as glutathione (GSH), superoxide dismutase (SOD), glutathione peroxidase (GPx), and catalase (CAT) and in serum total protein, and an increase in serum alkaline phosphatase (ALP), serum aspertate ami- notranferase (AST), serum alanine aminotranferase (ALT), bilirubin and liver thiobarbituric acid reactive substances (TBARS) due to liver injury in the paracetamol-administered rats (2 g/kg). On the contrary, increased activities of liver GSH, SOD, GPx, CAT and serum total protein level, and decrease in the contents of serum ALP, AST, ALT, bilirubin and liver TBARS were observed in rats administered with different doses of EE (100, 200 and 300 mg/kg), which are similar to the activities of hepato- protective drug silymarin (150 mg/kg). The mode of action of EE as evidenced by the above parameters may suggest that EE, on the one hand, prevents the formation of the reactive oxygen groups, or scavenges these groups, thereby preventing the damage on the hepatic cells, and, on the other hand, modulates the genes responsible for synthesis of antioxidant enzymes such as GPx, CAT and SOD in liver tissue and decreases the serum enzymatic activities such as ALP, AST and ALT.  相似文献   

17.
Environmental pollution affects the quality ofpedosphere, hydrosphere, atmosphere, lithosphere and biosphere. Great efforts have been made in the last two decades to reduce pollution sources and remedy the polluted soil and water resources. Phytoremediation, being more cost-effective and fewer side effects than physical and chemical approaches, has gained increasing popularity in both academic and practical circles. More than 400 plant species have been identified to have potential for soil and water remediation. Among them, Thlaspi, Brassica, Sedum alfredii H., and Arabidopsis species have been mostly studied. It is also expected that recent advances in biotechnology will play a promising role in the development of new hyperaccumulators by transferring metal hyperaccumulating genes from low biomass wild species to the higher biomass producing cultivated species in the times to come. This paper attempted to provide a brief review on recent progresses in research and practical applications of phytoremediation for soil and water resources.  相似文献   

18.
In the traditional dual-frequency radar ranging system, there is a contradiction between ranging precision and ranging distance. So a tri-frequency ranging method is proposed. The principle of the tri-frequency ranging is that the distance can be achieved by transmitting the three frequencies and then measuring the three phase difference and calculating the number of wavelength. What' s more, range ambiguity will not occur within a certain distance. The experimental results show that, if the waveband is 0.47 -0.57m, the measurement of unambiguous distance of 25 -500m can be realized. If the wavelength tolerance error is less than ± 10^-7 and the phase error is less than ± 10 ^-3, the precision of the distance ranging can reach 10^-6.  相似文献   

19.
Objective: This prospective randomized clinical trial was conducted to evaluate the necessity of drainage after total thyroidectomy or lobectomy for benign thyroidal disorders. Methods: A total of 116 patients who underwent total thyroidectomy or lobectomy for benign thyroidal disorders were randomly allocated to be drained or not. Operative and postoperative outcomes including operating time, postoperative pain assessed by visual analogue scale (VAS), total amount of intramuscular analgesic administration, hospital stay, complications, necessity for re-operation and satisfaction of patients were all assessed. Results: The mean operating time was similar between two groups (the drained and non-drained groups). The mean VAS score was found to be significantly low in the non-drained group patients in postoperative day (POD) 0 and POD 1. The mean amount of intramuscular analgesic requirement was significantly less in the non-drained group. One case of hematoma, two cases ofseroma and three cases of transient hypoparathyroidism occurred in the non-drained group, whereas one case of hematoma, two cases of seroma, two cases of wound infections and two cases of transient hypoparathyroidism occurred in the drained group. No patient needed re-operation for any complication. The mean hospital stay was significantly shorter and the satisfaction of patients was superior in the non-drained group. Conclusion: These findings suggest that postoperative complications cannot be prevented by using drains after total thyroidectomy or lobectomy for benign thyroid disorders. Furthermore, the use of drains may increase postoperative pain and the analgesic requirement, and prolong the hospital stay. In the light of these findings, the routine use of drains might not be necessary after thyroid surgery for benign disorders.  相似文献   

20.
Excessive dexamethasone (Dex) administrated into pregnant mice during critical periods of palatal development can produce a high incidence of cleft palate. Its mechanisms remain unknown. Vitamin B12 has been shown to antagonize the teratogenic effects of Dex, which, however, remains controversial. In this study, we investigated the effects of Dex and vitamin B12 on murine embryonic palatal shelf fusion using organ culture of murine embryonic shelves. The explanted palatal shelves on embryonic day 14 (E14) were cultured for 24, 48, 72 or 96 h in different concentrations of Dex and/or vitamin B 12. The palatal shelves were examined histologically for the morphological alterations on the medial edge epithelium (MEE) and fusion rates among different groups. It was found that the palatal shelves were not fused at 72 h or less of culture in Dex group, while they were completely fused in the control and vitamin B 12-treated groups at 72 and 96 h, respectively. The MEE still existed and proliferated. In Dex+vitamin B12 group the palatal shelves were fused at each time point in a similar rate to controls. These results may suggest that Dex causes teratogenesis of murine embryonic palatal shelves and vitamin B12 prevents the teratogenic effect of Dex on palatogenesis on murine embryos in vitro.  相似文献   

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