|
|
|
题名
|
作者
|
年代
|
出处
|
被引量
|
| 1 | Genome Editing Enables Next-Generation Hybrid Seed Production Technology显示文摘The next-generation hybrid seed technology enables the successful production of sortable hybrid seeds from genic male sterile(GMS)lines and maintainers;however,it requires multiple laborious and complicated steps.Here,we designed a simple next-generation hybrid seed production strategy that takes advantage of the CRISPR/Cas9 technology to create a Manipulated GMS Maintainer(MGM)system via a single transformation.Under this schema,the maize male fertility gene ZmMS26 was nullified by removal of its fifth exon using the CRISPR/Cas9 system on a vector,and a second vector carrying a functional ZmMS26 cDNA was co-transformed to restore fertility.The second vector also contains a male gametophyte inactivation gene(ZmAA1)encoding maizeα-amylase driven by the pollen-specific promoter PG47 and an endosperm fluorescent marker(DsRED)driven by the barley endosperm aleurone-specific promoter Ltp2.The derived single-copy hemizygous MGM lines bore a mutated MS26 gene,leading to complete male sterility but normal vegetative growth and grain yield.The MGM system could prevent genetic transmission of the MGM elements via male gametophytes,providing an efficient method for sorting maintainer seeds labeled by DsRED.This strategy can be extended to any GMS gene and to hybrid crops other than maize. | Xiantao Qi Congsheng Zhang Jinjie Zhu Changlin Liu Changling Huang Xinhai Li Chuanxiao Xie | 2020 | Molecular Plant2020,13,9: | 18 |
| 2 | Global overview of research progress and development of precision maize planters显示文摘Maize is the most important crop for food and widely used for industrial materials,leading to its increasing demand all over the world.Precision planting is the effective method to increase maize yield.To meet the agronomic requirement of precision planting,different kinds of precision maize planters were developed.However,because of the difference of geographical environment,cropping system,farm scale and economic status among different countries,types of maize planters are various and the technologies involved are at different levels.This paper summarizes the precision maize planters currently available in the world and classifies them into four types:precision planters for tilled-land,minimum/no tilled-land,hilly&small land,and cold&arid land.Detailed characteristics have been provided for some typical precision planters and comparisons were made as to their suitability under particular working conditions.High-efficiency and high-accuracy are the main features of precision planters for tilled-land,while the ability to clean residue from seed rows and prevent planters to be blocked are the important function of precision planters for minimum/no tilled-land.To fit for hilly&small land,planters should be light-weighted and small-sized,and to warm up soil and keep moisture,planters for cold&arid land should be equipped with plastic-film mulching mechanism.Finally,developing trend of precision planting technology was analyzed and suggestions,including policy support and technical improvement,were made for developing countries to make suitable precision maize planters according to the local geographical conditions and cropping systems. | Yang Li Yan Bingxin Cui Tao Yu Yiming He Xiantao Liu Quanwei Liang Zhijie Yin Xiaowei Zhang Dongxing | 2016 | International Journal of Agricultural and Biological Engineering2016,9,1: | 17 |
| 3 | Efficient random saliency map detection显示文摘Most image retargeting algorithms rely heavily on valid saliency map detection to proceed. However, the inefficiency of high quality saliency map detection severely restricts the application of these image retargeting methods. In this paper, we propose a random algorithm for efficient context-aware saliency map detection. Our method is a multiple level saliency map detection algorithm that integrates multiple level coarse saliency maps into the resulting saliency map and selectively updates unreliable regions of the saliency map to refine detection results. Because of the randomized search, our method requires very little additional memory beyond that for the input image and result map, and does not need to build auxiliary data structures to accelerate the saliency map detection. We have implemented our algorithm on a GPU and demonstrated the performance for a variety of images and video sequences, compared with state-of-the-art image processing. | HUANG ZhiYong HE FaZhi CAI XianTao ZOU ZhengQin LIU Jing LIANG MingMing & CHEN Xiao | 2011 | Science China(Information Sciences)2011,54,6: | 11 |
| 4 | Experimental Investigation and Optimization Design of Multi-Support Pipeline System显示文摘The design of aircraft hydraulic pipeline system is limited by many factors,such as the integrity of aviation structure or narrow installation space,so the limited clamp support position should be considered.This paper studied the frequency adjustment and dynamic responses reduction of the multi-support pipeline system through experiment and numerical simulation.To avoid the resonance of pipeline system,we proposed two different optimization programs,one was to avoid aero-engine working range,and another was to avoid aircraft hydraulic pump pulsation range.An optimization method was introduced in this paper to obtain the optimal clamp position.The experiments were introduced to validate the optimization results,and the theoretical optimization results can agree well with the test.With regard to avoiding the aero-engine vibration frequency,the test results revealed that the first natural frequency was far from the aero-engine vibration frequency.And the dynamic frequency sweep results showed that no resonance occurred on the pipeline in the engine vibration frequency range after optimization.Additionally,with regard to avoiding the pump vibration frequency,the test results revealed that natural frequencies have been adjusted and far from the pump vibration frequency.And the dynamic frequency sweep results showed that pipeline under optimal clamp position cannot lead to resonance.The sensitivity analysis results revealed the changing relationships between different clamp position and natural frequency.This study can provide helpful guidance on the analysis and design of practical aircraft pipeline. | Xiantao Zhang Wei Liu Yamei Zhang Yujie Zhao | 2021 | Chinese Journal of Mechanical Engineering2021,34,2: | 9 |
| 5 | Systematic identification of endogenous RNA polymeraseⅢpromoters for efficient RNA guidebased genome editing technologies in maize显示文摘Single-guide RNA(sg RNA) is one of the two core components of the CRISPR(clustered regularly interspaced short palindromic repeat)/Cas(CRISPR-associated) genome-editing technology. We established an in vitro Traffic Light Reporter(TLR) system, which is designated as the same colors as traffic lights such as green, red and yellow were produced in cells. The TLR can be readily used in maize mesophyll protoplast for a quick test of promoter activity. The TLR assay indicates the variation in transcription activities of the seven Pol III promoters, from 3.4%(U6-1) to over 21.0%(U6-6). The U6-2 promoter, which was constructed to drive sg RNA expression targeting the Zm Wx1 gene, yielded mutation efficiencies ranging from 48.5% to 97.1%. Based on the reported and unpublished data, the in vitro TLR assay results were confirmed to be a readily system and may be extended to other plant species amenable to efficient genome editing via CRISPR/Cas. Our efforts provide an efficient method of identifying native Pol III-recognized promoters for RNA guide-based genome-editing systems in maize. | Xiantao Qi Le Dong Changlin Liu Long Mao Fang Liu Xin Zhang Beijiu Cheng Chuanxiao Xie | 2018 | The Crop Journal2018,6,3: | 7 |
| 6 | Graphene/phosphorene nano-heterojunction:facile synthesis, nonlinear optics, and ultrafast photonics applications with enhanced performance显示文摘Owing to its thickness-modulated direct energy band gap, relatively strong light–matter interaction, and unique nonlinear optical response at a long wavelength, few-layer black phosphorus, or phosphorene, becomes very attractive in ultrafast photonics applications. Herein, we synthesized a graphene/phosphorene nano-heterojunction using a liquid phase-stripping method. Tiny lattice distortions in graphene and phosphorene suggest the formation of a nano-heterojunction between graphene and phosphorene nanosheets. In addition, we systematically investigate their nonlinear optical responses at different wavelength regimes. Our experiments indicate that the combined advantages of ultrafast relaxation, broadband response in graphene, and the strong light–matter interaction in phosphorene can be combined together by nano-heterojunction. We have further fabricated two-dimensional(2D) nano-heterojunction based optical saturable absorbers and integrated them into an erbium-doped fiber laser to demonstrate the generation of a stable ultrashort pulse down to 148 fs. Our results indicate that a graphene/phosphorene nano-heterojunction can operate as a promising saturable absorber for ultrafast laser systems with ultrahigh pulse energy and ultranarrow pulse duration. We believe this work opens up a new approach to designing 2D heterointerfaces for applications in ultrafast photonics and other research.The fabrication of a 2D nano-heterojunction assembled from stacking different 2D materials, via this facile and scalable growth approach, paves the way for the formation and tuning of new 2D materials with desirable photonic properties and applications. | SHUNXIANG LIU ZHONGJUN LI YANQI GE HUIDE WANG RUI YUE XIANTAO JIANG JIANQING LI QIAO WEN HAN ZHANG | 2017 | Photonics Research2017,5,6: | 6 |
| 7 | miR-27 inhibits adipocyte differentiation via suppressing CREB expression显示文摘miR-27 在 adipogenesis 的规定起一个否定作用。然而,分子的机制尚待是 clarified.In 现在的学习,我们发现 miR-27 由镇压部分禁止 adipogenesis 由直接指向它的 3 untranslated 区域的早 adipogenic transcriptionfactor 营地反应元素绑定蛋白质。另外,我们 demonstratedthat 肿瘤坏死因素 --(TNF-) 通过 NF-B 小径的处理起来调整 miR-27。而且, anti-miR-27 reducesthe TNF -- adipogenesis 的导致的抑制。同时, miR-27 表示的层次在成熟 adipocytesof 被减少肥胖的老鼠什么时候与瘦老鼠相比。我们的数据在 adipogenesis 的规定揭示了 miR-27 的新奇机制。 | Yingjie Zhu Xiaodong Zhang Xingpo Ding Huichao Wang Xiantao Chen Hanzheng Zhao Yudong Jia Sanhong Liu Youwen Liu | 2014 | Acta Biochimica et Biophysica Sinica2014,46,7: | 5 |
| 8 | Paleozoic post-collisional magmatism and high-temperature granulite-facies metamorphism coupling with lithospheric delamination of the East Kunlun Orogenic Belt,NW China显示文摘Lithosphere extension and upwelling of asthenosphere at post-collisional stage of an orogenic cycle generally induce diverse magmatism and/or associated high-temperature metamorphism. Nevertheless, the intimate coexistence of post-collisional magmatic activity and high-temperature metamorphism is rare.In this contribution, a lithological assemblage composing of diverse magmatic rocks deriving from distinct magma sources and coeval high-temperature metamorphism was identified in eastern Kunlun.Petrography, ages, mineral chemistry and whole-rock geochemistry demonstrated that those intimately coexistent diverse rocks were genetically related to post-collisional extension. The garnet-bearing mafic granulites in Jinshuikou area interior of the East Kunlun Orogenic Belt are mainly composed of garnet,orthopyroxene, and plagioclase, with peak metamorphic P–T conditions of ~ 701–756 ℃and 5.6–7.0 kbar,representing a granulite-facies metamorphism at 409.7 ± 1.7 Ma. The diverse contemporaneous magmatic rocks including hornblendites, gabbros and granites yield zircon U–Pb ages of 408.6 ± 2.5 Ma,413.4 ± 4.6 Ma, and 387–407 Ma, respectively. The hornblendites show N-MORB-like REE patterns with(La/Sm)Nvalues of 0.85–0.94. They have positive zircon εHf(t) values of 0.1–4.9 and whole-rock εNd(t) values of 3.9–4.7 but relatively high(^(87)Sr/^(86)Sr)_(i)values of 0.7081 to 0.7088. These features demonstrate that the hornblendites derived from a depleted asthenospheric mantle source with minor continental crustal materials in source. As for the gabbros, they exhibit arc-like elemental signatures, low zircon εHf(t) values(-4.3 to 2.5) and variable whole-rock εNd(t) values(-4.9 to 1.2) as well as high(^(87)Sr/86 Sr)ivalues(0.7068 to 0.7126), arguing for that they were originated from partial melting of heterogeneous lithospheric mantle anteriorly metasomatized by subducted-sediment released melts. Geochemistry of the granites defines their strongly peraluminous S-type signatures. Zircons from the granites yield a large range of εHf(t) values ranging from -30.8 to -5.1, while the whole-rock samples yield consistent(^(87)Sr/86 Sr)ivalues(0.7301 to 0.7342) and negative εNd(t) values(-10.1 to -12.4). These features indicate that the S-type granites could be generated by reworking of an ancient crust. Taken together, the penecontemporaneous magmatism and metamorphic event, demonstrated the early-middle Devonian transition from crustal thickening to extensional collapse. The post-collisional mantle-derived magmas serve as an essential driving force for the high-temperature granulite-facies metamorphism and anataxis of the crust associated with formation of S-type granite. This study not only constructs a more detail Proto-Tethys evolution process of the eastern Kunlun, but also sheds new light on better understanding the intimate relationship between magmatism and metamorphism during post-collisional extensional collapse. | Qian Wang Jiao Zhao Chuanlin Zhang Shengyao Yu Xiantao Ye Xiaoqiang Liu | 2022 | Geoscience Frontiers2022,13,1: | 5 |
| 9 | Design and experimental study of seed precise delivery mechanism for high-speed maize planter显示文摘Aiming at improving the seed spacing uniformity of maize planter at high forward speed,a seed precise delivery mechanism driven by electric motor has been developed and evaluated in laboratory.The mechanism was designed to deliver single seed from seed meter to furrow.Seed’s movement from the seed release point in a seed meter to the seed delivery cavity in the belt was analyzed,the mathematical model of seed movement in the seed precise delivery mechanism was established based on seed delivery time analysis.A mechanical prototype was designed and associated control system was developed.The performance contrast experiment was conducted between a traditional seed tube and newly developed seed precise delivery mechanism,and test results indicated that the qualities of feed index of the seed precise delivery mechanism were higher than those of the traditional seed tube delivery mechanism,and the coefficients of variation of the seed precise delivery mechanism were lower than those of the traditional seed tube delivery mechanism when the forward speed of planter was set at 10 km/h,12 km/h and 14 km/h,respectively.It indicated the seed precise delivery mechanism significantly improved seed spacing uniformity of maize planter compared with the traditional seed tube delivery mechanism. | Quanwei Liu Tao Cui Dongxing Zhang Li Yang Yunxia Wang Xiantao He Mantao Wang | 2018 | International Journal of Agricultural and Biological Engineering2018,11,4: | 4 |
| 10 | Conversion of a normal maize hybrid into a waxy version using in vivo CRISPR/Cas9 targeted mutation activity显示文摘Waxy maize is a specialty maize that produces mainly amylopectin starch with special food or industrial values. The objective of this study was to overcome the limitations of wx mutant allele acquisition and breeding efficiency by conversion of parental lines from normal to waxy maize. The intended mutation activity was achieved by in vivo CRISPR/Cas9 machinery involving desired-target mutation of the Wx locus in the ZC01 background,abbreviated as ZC01-DTM^(wx). Triple selection was applied to segregants to obtain high genome background recovery with transgene-free wx mutations. The targeted mutation was identified, yielding six types of mutations among progeny crossed with ZC01-DTM^(wx).The amylopectin contents of the endosperm starch in mutant lines and hybrids averaged94.9%, while those of the wild-type controls were significantly(P < 0.01) lower, with an average of 76.9%. Double selection in transgene-free lines was applied using the Bar strip test and Cas9 PCR screening. The genome background recovery ratios of the lines were determined using genome-wide SNP data. That of lines used as male parents was as high as98.19% and that of lines used as female parents was as high as 86.78%. Conversion hybrids and both parental lines showed agronomic performance similar to that of their wild-type counterparts. This study provides a practical example of the efficient extension of CRISPR/Cas9 targeted mutation to industrial hybrids for transformation of a recalcitrant species. | Xiantao Qi Hao Wu Haiyang Jiang Jinjie Zhu Changling Huang Xin Zhang Changlin Liu Beijiu Cheng | 2020 | The Crop Journal2020,8,3: | 4 |
| 11 | Establishment of an efficient seed fluorescence reporter-assisted CRISPR/Cas9 gene editing in maize显示文摘Genome editing by clustered regularly interspaced short palindromic sequences(CRISPR)/CRISPRassociated protein 9(Cas9)has revolutionized functional gene analysis and genetic improvement.While reporter-assisted CRISPR/Cas systems can greatly facilitate the selection of genome-edited plants produced via stable transformation,this approach has not been well established in seed crops.Here,we established the seed fluorescence reporter(SFR)-assisted CRISPR/Cas9 systems in maize(Zea mays L.),using the red fluorescent Ds RED protein expressed in the endosperm(En-SFR/Cas9),embryos(Em-SFR/Cas9),or both tissues(Em/En-SFR/Cas9).All three SFRs showed distinct fluorescent patterns in the seed endosperm and embryo that allowed the selection of seeds carrying the transgene of having segregated the transgene out.We describe several case studies of the implementation of En-SFR/Cas9,Em-SFR/Cas9,and Em/En-SFR/Cas9 to identify plants not harboring the genomeediting cassette but carrying the desired mutations at target genes in single genes or in small-scale mutant libraries,and report on the successful generation of single-target mutants and/or mutant libraries with En-SFR/Cas9,Em-SFR/Cas9,and Em/En-SFR/Cas9.SFR-assisted genome editing may have particular value for application scenarios with a low transformation frequency and may be extended to other important monocot seed crops. | Yuanyuan Yan Jinjie Zhu Xiantao Qi Beijiu Cheng Changlin Liu Chuanxiao Xie | 2021 | Journal of Integrative Plant Biology2021,63,9: | 3 |
| 12 | Quasi-plane-hypothesis of strain coordination for RC beams seismically strengthened with externally-bonded or near-surface mounted fiber reinforced plastic显示文摘The application of fiber reinforced plastic(FRP),including carbon FRP and glass FRP,for structural repair and strengthening has grown due to their numerous advantages over conventional materials such as externally bonded reinforcement(EBR) and near-surface mounted(NSM) strengthening techniques.This paper summarizes the results from 21 reinforced concrete beams strengthened with different methods,including externally-bonded and near-surface mounted FRP,to study the strain coordination of the FRP and steel rebar of the RC beam.Since there is relative slipping between the RC beam and the FRP,the strain of the FRP and steel rebar of the RC beam satisfy the quasi-plane-hypothesis;that is,the strain of the longitudinal fiber that parallels the neutral axis of the plated beam within the scope of the effective height(h 0) of the cross section is in direct proportion to the distance from the fiber to the neutral axis.The strain of the FRP and steel rebar satisfies the equation:ε FRP =βε steel,and the value of β is equal to 1.1-1.3 according to the test results. | Ren Zhenhua Zeng Xiantao Liu Hanlong Zhou Fengjun | 2013 | Earthquake Engineering and Engineering Vibration2013,12,1: | 3 |
| 13 | Systematic reviews on reports of hip fractures in Web of Science:a bibliometric analysis of publication activity显示文摘 | Mao Zhi Wang Guoqi Mei Xifan Chen Shuo Liu Xiaoxie Zeng Xiantao Long Anhua Zhang Licheng Zhang Lihai Tang Peifu | 2014 | Chinese Medical Journal2014,,13: | 2 |
| 14 | Integration of high-performance spin-orbit torque MRAM devices by 200-mm-wafer manufacturing platform显示文摘We demonstrate in-plane field-free-switching spin-orbit torque(SOT)magnetic tunnel junction(MTJ)devices that are capable of low switching current density,fast speed,high reliability,and,most importantly,manufactured uniformly by the 200-mm-wafer platform.The performance of the devices is systematically studied,including their magnetic properties,switch-ing behaviors,endurance and data retention.The successful integration of SOT devices within the 200-mm-wafer manufactur-ing platform provides a feasible way to industrialize SOT MRAMs.It is expected to obtain excellent performance of the devices by further optimizing the MTJ film stacks and the corresponding fabrication processes in the future. | Hongchao Zhang Xiangyue Ma Chuanpeng Jiang Jialiang Yin Shuqin Lyu Shiyang Lu Xiantao Shang Bowen Man Cong Zhang Dandan Li Shuhui Li Wenjing Chen Hongxi Liu Gefei Wang Kaihua Cao Zhaohao Wang Weisheng Zhao | 2022 | Journal of Semiconductors2022,43,10: | 2 |
| 15 | Isolated atomic catalysts encapsulated in MOF for ultrafast water pollutant treatment显示文摘Atomic noble metals stand as one of the most advanced catalysts because of their unique properties and interaction with the reactants.However,due to their high activity,noble atomic catalysts tend to aggregate and deactivate in practical application.Moreover,supports aimed to disperse these atomic catalysts often suffer from weak confinement and poor porosity,thus limited the catalytic efficiency of noble atoms.Here,we report the facile encapsulation of atomic noble catalyst in cheap cerous metal-organic framework(Ce-MOF)crystals to create a robust catalyst that could deliver high catalytic performance for the reduction of 4-nitrophenol without decay in long-term cycling test.Specifically,Au atoms encapsulated in Ce-MOF exhibited ultrahigh turnover frequency(TOF)of 131 min−1 for the reduction of 4-nitrophenol in minutes,consuming only 10%precious metals compared with state-of-the-art catalysts operated under same condition. | Shuailong Guo Hao Yuan Wei Luo Xiaoqing Liu Xiantao Zhang Haoqing Jiang Feng Liu Gary J.Cheng | 2021 | Nano Research2021,14,5: | 2 |
| 16 | Effect of travel speed on seed spacing uniformity of corn seed meter显示文摘Both seeding performance of seed metering unit and travel speed of seed planter have significant effects on seeding quality,thereby affecting crop growth and yields.In order to determine the effects of different travel speeds on seed spacing uniformity,four different types of seed meters were evaluated at five different travel speeds on seed meter test bench and in field.The tested seed meters included a finger pickup seed meter,a scoop-wheel seed meter,an air-pressure type seed meter and an air-blowing type seed meter.The seeding performance of the horizontal distribution of seeds within a row was described by using the coefficient of variation,the quality of feed index,the multiple index and the miss-seeding index.Experiments were performed in laboratory and field,respectively.Results indicated that different travel speeds have statistically significant effects on seed spacing uniformity.The four types of seed meters performed better on the seed meter test bench than in the field.Coefficient of variation increases and quality of feed index decreases as the travel speed of seed planter increases.The best seed spacing uniformity was obtained with the air-pressure type seed meter,followed with the air-blowing type seed meter,the finger pickup seed meter and the scoop-wheel seed meter.There were considerable differences between the performances of the scoop-wheel seed meter in the bench test and field test;the seeding qualities were much better in the bench test than in the field test.The scoop-wheel seed meter is more sensitive to vibration than the other types of seed meters. | Liu Quanwei He Xiantao Yang Li Zhang Dongxing Cui Tao Qu Zhe Yan Bingxin Wang Mantao Zhang Tianliang | 2017 | International Journal of Agricultural and Biological Engineering2017,10,4: | 2 |
| 17 | Multiple criteria linear programming approach to data mining: Models, algorithm designs and software development 显示文摘 | KOU Gang LIU Xiantao PENG Yi | 2003 | Optimization Methods and Software2003,18,4: | 1 |
| 18 | Structural elucidation and immunological activity of a polysaccharide from the fruiting body of Armillaria mellea显示文摘 | Yongxu Sun Haitao Liang Xiantao Zhang Haibin Tong Jicheng Liu | 2008 | Bioresource Technology2008,,5: | 1 |
| 19 | CRISPR/dCas-mediated gene activation toolkit development and its application for parthenogenesis induction in maize显示文摘Clustered regularly interspaced short palindromic repeats(CRISPR)-Cas systems can be engineered as programmable transcription factors to either activate(CRISPRa)or inhibit transcription.Apomixis is extremely valuable for the seed industry in breeding clonal seeds with pure genetic backgrounds.We report here a CRISPR/dCas9-based toolkit equippedwith dCas9-VP64 andMS2-p65-HSF1 effectors that may specifically target genes with high activation capability.We explored the application of in vivo CRISPRa targeting of maize BABY BOOM2(ZmBBM2),acting as a fertilization checkpoint,as a means to engineer parthenogenesis.We detected ZmBBM2 transcripts only in egg cells but not in other maternal gametic cells.Activation of ZmBBM2 in egg cells in vivo caused maternal cell-autonomous parthenogenesis to produce haploid seeds.Our work provides a highly specific gene-activation CRISPRa technology for target cells and verifies its application for parthenogenesis induction in maize. | Xiantao Qi Huimin Gao Renyao Lv Wenbo Mao Jinjie Zhu Changling Liu Long Mao Xinhai Li Chuanxiao Xie | 2023 | Plant Communications2023,4,2: | 1 |
| 20 | HPDL deficiency causes a neuromuscular disease by impairing the mitochondrial respiration显示文摘Mitochondrial diseases are caused by variants in both mitochondrial and nuclear genomes.A nuclear gene HPDL(4-hydroxyphenylpyruvate dioxygenase-like),which encodes an intermembrane mitochondrial protein,has been recently implicated in causing a neurodegenerative disease characterized by pediatric-onset spastic movement phenotypes.Here,we report six Chinese patients with bi-allelic HPDL pathogenic variants from four unrelated families showing neuropathic symptoms of variable severity,including developmental delay/intellectual disability,spasm,and hypertonia.Seven different pathogenic variants are identified,of which five are novel.Both fibroblasts and immortalized lymphocytes derived from patients show impaired mitochondrial respiratory function,which is also observed in HPDL-knockdown(KD)He La cells.In these He La cells,overexpression of a wild-type HPDL gene can rescue the respiratory phenotype of oxygen consumption rate.In addition,a decreased activity of the oxidative phosphorylation(OXPHOS)complex II is observed in patient-derived lymphocytes and HPDL-KD He La cells,further supporting an essential role of HPDL in the mitochondrial respiratory chain.Collectively,our data expand the clinical and mutational spectra of this mitochondrial neuropathy and further delineate the possible disease mechanism involving the impairment of the OXPHOS complex II activity due to the bi-allelic inactivations of HPDL. | Yu Sun Xiujuan Wei Fang Fang Yiping Shen Haiyan Wei Jiuwei Li Xianglai Ye Yongkun Zhan Xiantao Ye Xiaomin Liu Wei Yang Yuhua Li Xiangju Geng Xuelin Huang Yiyan Ruan Zailong Qin Shang Yi Jianxin Lyu Hezhi Fang Yongguo Yu | 2021 | Journal of Genetics and Genomics2021,48,8: | 1 |