Spinocerebellar ataxias （SCAs） are a group of genetic disorders characterized by slowly progressive incoordina- tion of gait and are often associated with poor coordination of the hands, speech, and eye movements. F...Spinocerebellar ataxias （SCAs） are a group of genetic disorders characterized by slowly progressive incoordina- tion of gait and are often associated with poor coordination of the hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. The genetic forms of ataxia are diagnosed by family history, physical examina- tion, neuroimaging, and molecular genetic testing. At present, 36 SCA subtypes including 27 pathogenic genes have been identified [1]. Different subtypes of SCAs have clear distribution differences among ethnic populations, and SCA8 is an infrequent entity worldwide, which has mostly been reported in Japanese, but has never been reported in Chinese [2]. SCAB involves bidirectional expression based on the total number of both the （CTA）n and （CTG）n expansion transcripts in ATXN8OS. The pathogenesis of this disorder is complex and the spectrum of clinical presentations is broad. It is predominantly characterized by drawn-out slowness of speech and gait instability, followed by slowly progressive ataxia, with disease onset typically occurring in adulthood [3]. How- ever, the lowest full-penetrance allele for SCA8 onset remains elusive and the current understanding of the phenotypic and genotypic features of SCA8 is limited. Since SCA8 has not yet been reported in the Chinese population and is scantily reported in a small proportion of pedigrees so far, clinical knowledge is still developing. Moreover, the boundary between the normal and patho- genic alleles of SCA8 is uncertain. Here we report the clinical and molecular genetic characteristics of 3 Chinese SCA8 families and have identified 51 CTA/CTG repeats within ATXN8OS, probably the shortest pathogenic allele for SCA8.展开更多
Dear Ayi,I think my son’s elementary teachers are irresponsible!He always comes home with his water bottle still full,despite my repeated messages to his teachers reminding them to encourage him to drink more water.S...Dear Ayi,I think my son’s elementary teachers are irresponsible!He always comes home with his water bottle still full,despite my repeated messages to his teachers reminding them to encourage him to drink more water.Should I move him to another school next year?展开更多
Dear Ayi,I tried to avoid crowds this Labor Day holiday by visiting a small village in the middle of nowhere.Iended up bored out of my mind.Where can I go that's quiet and fun?
I’m crazy sick.I eat a lot of煎饼(ji`nbing,Chinese pancakes),like,every day for breakfast.Do you think they are using gutter oil or something like that?It’s really annoying.Scot Scott Well,of course it’s the煎饼.Yo...I’m crazy sick.I eat a lot of煎饼(ji`nbing,Chinese pancakes),like,every day for breakfast.Do you think they are using gutter oil or something like that?It’s really annoying.Scot Scott Well,of course it’s the煎饼.You don’t have enough experience eating Chinese food.You can’t just have some spicy煎饼every day.Try another Chinese breakfast,something more bland,展开更多
I’ve been binge-eating all through the Spring Festival and gained so much weight.I hate myself.Dear Ayi,how can I lose it quickly?Stuffed Steve Back in my day,we starved for two days and ate a single meal of gruel ma...I’ve been binge-eating all through the Spring Festival and gained so much weight.I hate myself.Dear Ayi,how can I lose it quickly?Stuffed Steve Back in my day,we starved for two days and ate a single meal of gruel made of corn on Spring Festival Eve.Look at展开更多
Dear Agony Ayi,How do I break up with my boyfriend who I’ve had for three years?His family loves me and treats me like their daughter.Sincerely,Ms.Beijing Brokenheart毒的女人 (dú de nǔrén, evil woman). ...Dear Agony Ayi,How do I break up with my boyfriend who I’ve had for three years?His family loves me and treats me like their daughter.Sincerely,Ms.Beijing Brokenheart毒的女人 (dú de nǔrén, evil woman). 你怎么敢 (ni zenme gan, how dare you). You are a monster. That is, unless he’s a 屌丝男 (diao sinan, a poor and ugly guy ). In which case: run. Just run away. Yes, yes, the 傻子 (shazi,展开更多
I am a Rabbit and my husband is an Ox.Can you tell me our fortune in the coming Year of the Rooster?Zodiac Zelda Rabbit and Ox?It’s not exactly a天作之合(tianzuo zhi he,a match made in heaven).He may have seemed stea...I am a Rabbit and my husband is an Ox.Can you tell me our fortune in the coming Year of the Rooster?Zodiac Zelda Rabbit and Ox?It’s not exactly a天作之合(tianzuo zhi he,a match made in heaven).He may have seemed steady when you first met,but he’ll turn out as stubborn as,well,an ox.And you?You are too timid to challenge him.More bad news:III feng展开更多
I’m worried my husband—who is quite a bit older than me—is flirting with his Chinese coworkers.They are very young and pretty.We have two sons,and now I don’t trust him anymore.Help.Yankee Eighty-TwoWell,this is i...I’m worried my husband—who is quite a bit older than me—is flirting with his Chinese coworkers.They are very young and pretty.We have two sons,and now I don’t trust him anymore.Help.Yankee Eighty-TwoWell,this is inevitable.Chinese women展开更多
I lost all my money in the stock market,what should I do?I haven’t even told my wife.Broke BrokerI know what people usually do;they跳楼(tiaolou,jump from a height)to一了百了(yiliao bai liao,end it for good).May I sug...I lost all my money in the stock market,what should I do?I haven’t even told my wife.Broke BrokerI know what people usually do;they跳楼(tiaolou,jump from a height)to一了百了(yiliao bai liao,end it for good).May I suggest a building so tall that you展开更多
Where can I learn to make really good General Tso’s chicken?It was my favorite dish in America,and I’d like to make it here.Uncle SamanthaGeneral Tso?General Tso?.First of all,it’s左宗棠,not'General Tso'.He...Where can I learn to make really good General Tso’s chicken?It was my favorite dish in America,and I’d like to make it here.Uncle SamanthaGeneral Tso?General Tso?.First of all,it’s左宗棠,not'General Tso'.He was a great general—or a bad one.I don’t know.It’s not important—but it is important that he commanded soldiers,not a chicken.展开更多
I am torn between two men.One earns 400,000 RMB a year,but only has a high-school diploma;the other is my college classmate,who is doing an unpaid internship.With the first man,I worry about our kids'future educat...I am torn between two men.One earns 400,000 RMB a year,but only has a high-school diploma;the other is my college classmate,who is doing an unpaid internship.With the first man,I worry about our kids'future education,but with the second,I feel we would have to work hard in the next few years to have the life I want.Dear Ayi,which man should I pick?展开更多
Why do people have to talk so loud on their phones when they are on the train?It bothers me.I plan to stand up next time and ask them to keep it to themselves.What do you recommend?Mobile Man Shut up and be a good倾听...Why do people have to talk so loud on their phones when they are on the train?It bothers me.I plan to stand up next time and ask them to keep it to themselves.What do you recommend?Mobile Man Shut up and be a good倾听者(qingtingzhe,listener).These passengers are nice enough to开诚布公(kaicheng bugong,speak frankly and sincerely)in front a group of total陌生人(moshengren,strangers).The least you can do is satisfy展开更多
So,I just found out my brother’s gay.My mom is Chinese and doesn’t approve.She says it’s wrong,and that she wants him to get married.How can I help my poor brother?Mary Guo l&M lbo11,glass,meaning gay),huh?Well...So,I just found out my brother’s gay.My mom is Chinese and doesn’t approve.She says it’s wrong,and that she wants him to get married.How can I help my poor brother?Mary Guo l&M lbo11,glass,meaning gay),huh?Well,it is all okay nowadays.fs]]t-(tongzhi)used to mean comrade,and now it means that you are Hfr-ffi(dudnxiu,cut展开更多
Chongqing Sanxia Paints Co., Ltd. (Chongqing Sanxia, SZ: 000565) has recently an-nounced that because of China’s glycine prices being lower than expected, the startup ofwhole glycine project in the company will proba...Chongqing Sanxia Paints Co., Ltd. (Chongqing Sanxia, SZ: 000565) has recently an-nounced that because of China’s glycine prices being lower than expected, the startup ofwhole glycine project in the company will probably be delayed.展开更多
The fuzzy comfortability of a wind-sensitive super-high tower crane is critical to guarantee occupant health and improve construction efficiency.Therefore,the wind-resistant fuzzy comfortability of a super-high tower ...The fuzzy comfortability of a wind-sensitive super-high tower crane is critical to guarantee occupant health and improve construction efficiency.Therefore,the wind-resistant fuzzy comfortability of a super-high tower crane in the Ma’anshan Yangtze River(MYR)Bridge site is analyzed in this paper.First,the membership function model that represents fuzzy comfortability is introduced in the probability density evolution method(PDEM).Second,based on Fechner’s law,the membership function curves are constructed according to three acceleration thresholds in ISO 2631.Then,the fuzzy comfortability for the super-high tower crane under stochastic wind loads is assessed on the basis of different cut-set levelsλ.Results show that the comfortability is over 0.9 under the required maximum operating wind velocity.The low sensitivity toλcan be observed in the reliability curves of ISOⅡandⅢmembership functions.The reliability of the ISOⅠmembership function is not sensitive toλwhenλ<0.7,whereas it becomes sensitive toλwhenλ>0.7.展开更多
To ensure the structural integrity of life-limiting component of aeroengines,Probabilistic Damage Tolerance(PDT)assessment is applied to evaluate the failure risk as required by airworthiness regulations and military ...To ensure the structural integrity of life-limiting component of aeroengines,Probabilistic Damage Tolerance(PDT)assessment is applied to evaluate the failure risk as required by airworthiness regulations and military standards.The PDT method holds the view that there exist defects such as machining scratches and service cracks in the tenon-groove structures of aeroengine disks.However,it is challenging to conduct PDT assessment due to the scarcity of effective Probability of Detection(POD)model and anomaly distribution model.Through a series of Nondestructive Testing(NDT)experiments,the POD model of real cracks in tenon-groove structures is constructed for the first time by employing the Transfer Function Method(TFM).A novel anomaly distribution model is derived through the utilization of the POD model,instead of using the infeasible field data accumulation method.Subsequently,a framework for calculating the Probability of Failure(POF)of the tenon-groove structures is established,and the aforementioned two models exert a significant influence on the results of POF.展开更多
基金supported by grants from the National Natural Science Foundation of China(81301486 and81672095)
文摘Spinocerebellar ataxias （SCAs） are a group of genetic disorders characterized by slowly progressive incoordina- tion of gait and are often associated with poor coordination of the hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. The genetic forms of ataxia are diagnosed by family history, physical examina- tion, neuroimaging, and molecular genetic testing. At present, 36 SCA subtypes including 27 pathogenic genes have been identified [1]. Different subtypes of SCAs have clear distribution differences among ethnic populations, and SCA8 is an infrequent entity worldwide, which has mostly been reported in Japanese, but has never been reported in Chinese [2]. SCAB involves bidirectional expression based on the total number of both the （CTA）n and （CTG）n expansion transcripts in ATXN8OS. The pathogenesis of this disorder is complex and the spectrum of clinical presentations is broad. It is predominantly characterized by drawn-out slowness of speech and gait instability, followed by slowly progressive ataxia, with disease onset typically occurring in adulthood [3]. How- ever, the lowest full-penetrance allele for SCA8 onset remains elusive and the current understanding of the phenotypic and genotypic features of SCA8 is limited. Since SCA8 has not yet been reported in the Chinese population and is scantily reported in a small proportion of pedigrees so far, clinical knowledge is still developing. Moreover, the boundary between the normal and patho- genic alleles of SCA8 is uncertain. Here we report the clinical and molecular genetic characteristics of 3 Chinese SCA8 families and have identified 51 CTA/CTG repeats within ATXN8OS, probably the shortest pathogenic allele for SCA8.
文摘Dear Ayi,I think my son’s elementary teachers are irresponsible!He always comes home with his water bottle still full,despite my repeated messages to his teachers reminding them to encourage him to drink more water.Should I move him to another school next year?
文摘Dear Ayi,I tried to avoid crowds this Labor Day holiday by visiting a small village in the middle of nowhere.Iended up bored out of my mind.Where can I go that's quiet and fun?
文摘I’m crazy sick.I eat a lot of煎饼(ji`nbing,Chinese pancakes),like,every day for breakfast.Do you think they are using gutter oil or something like that?It’s really annoying.Scot Scott Well,of course it’s the煎饼.You don’t have enough experience eating Chinese food.You can’t just have some spicy煎饼every day.Try another Chinese breakfast,something more bland,
文摘I’ve been binge-eating all through the Spring Festival and gained so much weight.I hate myself.Dear Ayi,how can I lose it quickly?Stuffed Steve Back in my day,we starved for two days and ate a single meal of gruel made of corn on Spring Festival Eve.Look at
文摘Dear Agony Ayi,How do I break up with my boyfriend who I’ve had for three years?His family loves me and treats me like their daughter.Sincerely,Ms.Beijing Brokenheart毒的女人 (dú de nǔrén, evil woman). 你怎么敢 (ni zenme gan, how dare you). You are a monster. That is, unless he’s a 屌丝男 (diao sinan, a poor and ugly guy ). In which case: run. Just run away. Yes, yes, the 傻子 (shazi,
文摘I am a Rabbit and my husband is an Ox.Can you tell me our fortune in the coming Year of the Rooster?Zodiac Zelda Rabbit and Ox?It’s not exactly a天作之合(tianzuo zhi he,a match made in heaven).He may have seemed steady when you first met,but he’ll turn out as stubborn as,well,an ox.And you?You are too timid to challenge him.More bad news:III feng
文摘I’m worried my husband—who is quite a bit older than me—is flirting with his Chinese coworkers.They are very young and pretty.We have two sons,and now I don’t trust him anymore.Help.Yankee Eighty-TwoWell,this is inevitable.Chinese women
文摘I lost all my money in the stock market,what should I do?I haven’t even told my wife.Broke BrokerI know what people usually do;they跳楼(tiaolou,jump from a height)to一了百了(yiliao bai liao,end it for good).May I suggest a building so tall that you
文摘Where can I learn to make really good General Tso’s chicken?It was my favorite dish in America,and I’d like to make it here.Uncle SamanthaGeneral Tso?General Tso?.First of all,it’s左宗棠,not'General Tso'.He was a great general—or a bad one.I don’t know.It’s not important—but it is important that he commanded soldiers,not a chicken.
文摘I am torn between two men.One earns 400,000 RMB a year,but only has a high-school diploma;the other is my college classmate,who is doing an unpaid internship.With the first man,I worry about our kids'future education,but with the second,I feel we would have to work hard in the next few years to have the life I want.Dear Ayi,which man should I pick?
文摘Why do people have to talk so loud on their phones when they are on the train?It bothers me.I plan to stand up next time and ask them to keep it to themselves.What do you recommend?Mobile Man Shut up and be a good倾听者(qingtingzhe,listener).These passengers are nice enough to开诚布公(kaicheng bugong,speak frankly and sincerely)in front a group of total陌生人(moshengren,strangers).The least you can do is satisfy
文摘So,I just found out my brother’s gay.My mom is Chinese and doesn’t approve.She says it’s wrong,and that she wants him to get married.How can I help my poor brother?Mary Guo l&M lbo11,glass,meaning gay),huh?Well,it is all okay nowadays.fs]]t-(tongzhi)used to mean comrade,and now it means that you are Hfr-ffi(dudnxiu,cut
文摘Chongqing Sanxia Paints Co., Ltd. (Chongqing Sanxia, SZ: 000565) has recently an-nounced that because of China’s glycine prices being lower than expected, the startup ofwhole glycine project in the company will probably be delayed.
基金The National Natural Science Foundation of China(No.52108274,52208481,52338011)State Scholarship Fund of China Scholarship Council(No.202306090285).
文摘The fuzzy comfortability of a wind-sensitive super-high tower crane is critical to guarantee occupant health and improve construction efficiency.Therefore,the wind-resistant fuzzy comfortability of a super-high tower crane in the Ma’anshan Yangtze River(MYR)Bridge site is analyzed in this paper.First,the membership function model that represents fuzzy comfortability is introduced in the probability density evolution method(PDEM).Second,based on Fechner’s law,the membership function curves are constructed according to three acceleration thresholds in ISO 2631.Then,the fuzzy comfortability for the super-high tower crane under stochastic wind loads is assessed on the basis of different cut-set levelsλ.Results show that the comfortability is over 0.9 under the required maximum operating wind velocity.The low sensitivity toλcan be observed in the reliability curves of ISOⅡandⅢmembership functions.The reliability of the ISOⅠmembership function is not sensitive toλwhenλ<0.7,whereas it becomes sensitive toλwhenλ>0.7.
基金supported by the National Major Science and Technology Project,China(No.J2019-Ⅳ-0007-0075)the Fundamental Research Funds for the Central Universities,China(No.JKF-20240036)。
文摘To ensure the structural integrity of life-limiting component of aeroengines,Probabilistic Damage Tolerance(PDT)assessment is applied to evaluate the failure risk as required by airworthiness regulations and military standards.The PDT method holds the view that there exist defects such as machining scratches and service cracks in the tenon-groove structures of aeroengine disks.However,it is challenging to conduct PDT assessment due to the scarcity of effective Probability of Detection(POD)model and anomaly distribution model.Through a series of Nondestructive Testing(NDT)experiments,the POD model of real cracks in tenon-groove structures is constructed for the first time by employing the Transfer Function Method(TFM).A novel anomaly distribution model is derived through the utilization of the POD model,instead of using the infeasible field data accumulation method.Subsequently,a framework for calculating the Probability of Failure(POF)of the tenon-groove structures is established,and the aforementioned two models exert a significant influence on the results of POF.
