BACKGROUND Adult neuronal ceroid lipofuscinosis(ANCL)can be caused by compound heterozygous recessive mutations in CLN6.The main clinical features of the disease are neurodegeneration,progressive motor dysfunction,sei...BACKGROUND Adult neuronal ceroid lipofuscinosis(ANCL)can be caused by compound heterozygous recessive mutations in CLN6.The main clinical features of the disease are neurodegeneration,progressive motor dysfunction,seizures,cognitive decline,ataxia,vision loss and premature death.CASE SUMMARY A 37-year-old female presented to our clinic with a 3-year history of limb weakness and gradually experiencing unstable walking.The patient was diagnosed with CLN6 type ANCL after the identification of mutations in the CLN6 gene.The patient was treated with antiepileptic drugs.The patient is under ongoing followup.Unfortunately,the patient’s condition has deteriorated,and she is currently unable to care for herself.CONCLUSION There is presently no effective treatment for ANCL.However,early diagnosis and symptomatic treatment are possible.展开更多
Intervertebral disc(IVD)degenerative diseases are a common problem in the world,and they cause substantial social and economic burdens for people.The current methods for treating IVD degenerative diseases mainly inclu...Intervertebral disc(IVD)degenerative diseases are a common problem in the world,and they cause substantial social and economic burdens for people.The current methods for treating IVD degenerative diseases mainly include surgery and conservative treatment,which cannot fundamentally restore the normal structure of the disc.With continuous research on the mechanism of degeneration and the development of regenerative medicine,rapid progress has been made in the field of regenerative medicine regarding the use of stem cell-derived exosomes,which are active biological substances used in intercellular communication,because they show a strong effect in promoting tissue regeneration.The study of exosomes in the field of IVD degeneration has just begun,and many surprising achievements have been made.This paper mainly reviews the biological characteristics of exosomes and highlights the current status of exosomes in the field of IVD degeneration,as well as future developments regarding exosomes.展开更多
Organic photochromic materials have drawn considerable attention for their potential applications in large-scale and low-cost optoelectronics owing to unique tunable physicochemical properties.For organic photodetecto...Organic photochromic materials have drawn considerable attention for their potential applications in large-scale and low-cost optoelectronics owing to unique tunable physicochemical properties.For organic photodetectors,photochromic materials have realized optical and electrical engineering of semiconductor layers,which incorporate not only tunable performance,but also functionalities to optoelectronic devices.However,the essential challenge is to assemble large-area photochromic micro-and nanostructure arrays with controllable geometry and precise alignment,which restricts the integration of multifunctional optoelectronic devices.Herein,we fabricate organic photochromic one-dimensional(1D)arrays via a feasible solution process through the confined crystallization of organic molecules.By modulating and controlling the photoisomerization behaviors,these 1D photochromic arrays possess broad spectral tunability,which ensure tunable photoresponse.Furthermore,we investigate the crystallographic transition and electronic performance variation of these 1D photochromic arrays.By adjusting the dwell time of ultraviolet(UV)irradiation,the UV photochromic photodetectors realize tunable and repeatable responsivity from 85.6 to 0.709 mA/W.Our work provides new possibilities for optical and electrical engineering of photochromic microwires towards the integration of multifunctional optoelectronic devices.展开更多
In 1992, Branner and Hubbard raised a conjecture that the Julia set of a polynomial is a Cantor set if and only if each critical component of its filled-in Julia set is not periodic. This conjecture was solved recentl...In 1992, Branner and Hubbard raised a conjecture that the Julia set of a polynomial is a Cantor set if and only if each critical component of its filled-in Julia set is not periodic. This conjecture was solved recently. In this paper, we generalize this result to a class of rational functions.展开更多
文摘BACKGROUND Adult neuronal ceroid lipofuscinosis(ANCL)can be caused by compound heterozygous recessive mutations in CLN6.The main clinical features of the disease are neurodegeneration,progressive motor dysfunction,seizures,cognitive decline,ataxia,vision loss and premature death.CASE SUMMARY A 37-year-old female presented to our clinic with a 3-year history of limb weakness and gradually experiencing unstable walking.The patient was diagnosed with CLN6 type ANCL after the identification of mutations in the CLN6 gene.The patient was treated with antiepileptic drugs.The patient is under ongoing followup.Unfortunately,the patient’s condition has deteriorated,and she is currently unable to care for herself.CONCLUSION There is presently no effective treatment for ANCL.However,early diagnosis and symptomatic treatment are possible.
文摘Intervertebral disc(IVD)degenerative diseases are a common problem in the world,and they cause substantial social and economic burdens for people.The current methods for treating IVD degenerative diseases mainly include surgery and conservative treatment,which cannot fundamentally restore the normal structure of the disc.With continuous research on the mechanism of degeneration and the development of regenerative medicine,rapid progress has been made in the field of regenerative medicine regarding the use of stem cell-derived exosomes,which are active biological substances used in intercellular communication,because they show a strong effect in promoting tissue regeneration.The study of exosomes in the field of IVD degeneration has just begun,and many surprising achievements have been made.This paper mainly reviews the biological characteristics of exosomes and highlights the current status of exosomes in the field of IVD degeneration,as well as future developments regarding exosomes.
基金acknowledge the National Natural Science Foundation(51922012,21633014)the MOST of China(Grant Nos.:2017YFA0204504,2018YFA0208502,,2018YFA0704803)+1 种基金Beijing Municipal Science and Technology(Z181100004418014)Youth Innovation Promotion Association CAS(2018034).
文摘Organic photochromic materials have drawn considerable attention for their potential applications in large-scale and low-cost optoelectronics owing to unique tunable physicochemical properties.For organic photodetectors,photochromic materials have realized optical and electrical engineering of semiconductor layers,which incorporate not only tunable performance,but also functionalities to optoelectronic devices.However,the essential challenge is to assemble large-area photochromic micro-and nanostructure arrays with controllable geometry and precise alignment,which restricts the integration of multifunctional optoelectronic devices.Herein,we fabricate organic photochromic one-dimensional(1D)arrays via a feasible solution process through the confined crystallization of organic molecules.By modulating and controlling the photoisomerization behaviors,these 1D photochromic arrays possess broad spectral tunability,which ensure tunable photoresponse.Furthermore,we investigate the crystallographic transition and electronic performance variation of these 1D photochromic arrays.By adjusting the dwell time of ultraviolet(UV)irradiation,the UV photochromic photodetectors realize tunable and repeatable responsivity from 85.6 to 0.709 mA/W.Our work provides new possibilities for optical and electrical engineering of photochromic microwires towards the integration of multifunctional optoelectronic devices.
文摘In 1992, Branner and Hubbard raised a conjecture that the Julia set of a polynomial is a Cantor set if and only if each critical component of its filled-in Julia set is not periodic. This conjecture was solved recently. In this paper, we generalize this result to a class of rational functions.
