This paper explores the socio-cultural dimensions of the Tiji Festival by delving into its historical origins,symbolic meanings,and cultural relevance to establish social harmony among the people.The Tiji Festival,an ...This paper explores the socio-cultural dimensions of the Tiji Festival by delving into its historical origins,symbolic meanings,and cultural relevance to establish social harmony among the people.The Tiji Festival,an annual three-day ritual celebrated in Lo(Mustang),Nepal,holds deep cultural-historical significance as it embodies the socio-cultural and religious ethos of the region.Rooted in Tibetan Buddhist traditions,it embodies the triumph of good over evil forces through the re-enactment of the legendary battle between Dorje Jono and the demonic forces.Situated in the trans-Himalayan region,Mustang has been a cultural crossroad for centuries,blending Tibetan Buddhist customs with indigenous practices.Beyond its religious significance,the festival functions as a platform for communal gathering,and the preservation of intangible cultural heritage.Despite modern influences,the resilience of Tiji highlights the dynamic interplay between tradition and adaptation.By unlocking the socio-cultural perspective of this vibrant festival,this study underscores its role in maintaining Mustang’s historical legacy while evolving within contemporary socio-cultural contexts.It reflects this unique synthesis,serving as a means of social cohesion,identity reinforcement,and spiritual renewal for the local community.The elaborate masked dances,sacred chants,and ritualistic performances not only preserve the ancient oral traditions but also convey ethical teachings vital to Mustang’s collective consciousness.The acceptance of creative potential factor of Tiji festival is analyzed through the lenses of cultural theorists that justify call for historical practices of it.展开更多
Panchen Rinpoche Sends Greetings for Tibetan New Year 2025 On the Tibetan New Year,Feb.28,Panchen Erdeni Chos-kyi rGyal-po,one of the most influential figures in Tibetan Buddhism,extended greetings to Tibetans at home...Panchen Rinpoche Sends Greetings for Tibetan New Year 2025 On the Tibetan New Year,Feb.28,Panchen Erdeni Chos-kyi rGyal-po,one of the most influential figures in Tibetan Buddhism,extended greetings to Tibetans at home and abroad.In the speech,he prayed for the young to be wise and for the elderly to enjoy peace and wellbeing,wished for the country's prosperity and world peace,and wished that everything would go well in the Year of the Wood Snake and the virtuous be blessed.展开更多
Set against the cultural and historical backdrop of 18th-century Venice,Giulia Elisabetta Lama’s(1681-1747)remarkable paintings reveal intriguing connections to mythology and science.Lama’s notable depiction of Satu...Set against the cultural and historical backdrop of 18th-century Venice,Giulia Elisabetta Lama’s(1681-1747)remarkable paintings reveal intriguing connections to mythology and science.Lama’s notable depiction of Saturn Devouring His Child,painted in oils in 1729 and now in a private collection,draws significant attention.展开更多
LAMA2-related congenital muscular dystrophy(LAMA2-CMD),characterized by laminin-α2 deficiency,is debilitating and ultimately fatal.To date,no effective therapy has been clinically available.Laminin-a1,which shares si...LAMA2-related congenital muscular dystrophy(LAMA2-CMD),characterized by laminin-α2 deficiency,is debilitating and ultimately fatal.To date,no effective therapy has been clinically available.Laminin-a1,which shares significant similarities with laminin-a2,has been proven as a viable compensatory modifier.To evaluate its clinical applicability,we establish a Lama2 exon-3-deletion mouse model(dy^(H)/dy^(H)).The dy^(H)/dy^(H)mice exhibit early lethality and typical LAMA2-CMD phenotypes,allowing the evaluation of various endpoints.In dy^(H)/dy^(H)mice treated with synergistic activation mediator-based CRISPRa-mediated Lama1 upregulation,a nearly doubled median survival is observed,as well as improvements in weight and grip.Significant therapeutical effects are revealed by MRl,serum biochemical indices,and muscle pathology studies.Treating LAMA2-CMD with LAMA1 upregulation is feasible,and early intervention can alleviate symptoms and extend lifespan.Additionally,we reveal the limitations of LAMA1 upregulation,including high-dose mortality and non-sustained expression,which require further optimization in future studies.展开更多
BACKGROUND We report a rare case of primary clinical presentation featuring elevated creatine kinase(CK)levels in a neonate,which is associated with the LAMA2 gene.In this case,a heterozygous mutation in exon5 of the ...BACKGROUND We report a rare case of primary clinical presentation featuring elevated creatine kinase(CK)levels in a neonate,which is associated with the LAMA2 gene.In this case,a heterozygous mutation in exon5 of the LAMA2 gene,c.715C>G(resulting in a change of nucleotide number 715 in the coding region from cytosine to gua-nine),induced an amino acid alteration p.R239G(No.239)in the patient,repre-senting a missense mutation.This observation may be elucidated by the neonatal creatine monitoring mechanism,a phenomenon not previously reported.CASE SUMMARY We analysed the case of a neonate presenting solely with elevated CK levels who was eventually discharged after supportive treatment.The chief complaint was identification of increased CK levels for 15 d and higher CK values for 1 d.Ad-mission occurred at 18 d of age,and despite prolonged treatment with creatine and vitamin C,the elevated CK levels showed limited improvement.Whole exo-me sequencing revealed the presence of a c.715C>G mutation in LAMA2 in the newborn,correlating with a clinical phenotype.However,the available informa-tion offers insufficient evidence for clinical pathogenicity.CONCLUSION Mutations in LAMA2 are associated with the clinical phenotype of increased neonatal CK levels,for which no specific treatment exists.Whole genome sequen-cing facilitates early diagnosis.展开更多
The 17th century was a time during which a numerous secret biographies occurred,and the writing of the elaborate secret biographies started.A Sealed Secret Biography of the 5th Dalai Lama(gsang mam rgy can ma in Tibet...The 17th century was a time during which a numerous secret biographies occurred,and the writing of the elaborate secret biographies started.A Sealed Secret Biography of the 5th Dalai Lama(gsang mam rgy can ma in Tibetan)was a well-known work in the time,yet to be popularized so far,and the academic research on the biography is not fruitful.This article,applying the method of academic research of Philology,discusses the writing style of the A Sealed Secret Biography of the 5th Dalai Lama in general.Specifically,it is found that the concerned biography is written in the writing style of prophesy,and the political history,social situation,the interrelationship among the important figures,and the solidarity of the territory were written in a metaphoric style.In terms of actual writing style,besides this biography was under the influence of A Sealed Secret Biography of Gongthang Lama Zhang(gong thang bla ma zhang gig sang mam bkav rgya ma in Tibetan)in the 12th century and A Secret Biography of Lhadrak Namkha Gyentsen(lho brag nam mkav rgyan mtshn gyi gsang bavi mam thar in Tibetan)in the 14th century,A Sealed Secret Biography of the 5th Dalai Lama displays its unique writing characteristics,which in turned started the secret biography writing of Nyingmapa scholars such as the works of Rikzin Gyurme Doije,the founding father of Mindroling.展开更多
文摘This paper explores the socio-cultural dimensions of the Tiji Festival by delving into its historical origins,symbolic meanings,and cultural relevance to establish social harmony among the people.The Tiji Festival,an annual three-day ritual celebrated in Lo(Mustang),Nepal,holds deep cultural-historical significance as it embodies the socio-cultural and religious ethos of the region.Rooted in Tibetan Buddhist traditions,it embodies the triumph of good over evil forces through the re-enactment of the legendary battle between Dorje Jono and the demonic forces.Situated in the trans-Himalayan region,Mustang has been a cultural crossroad for centuries,blending Tibetan Buddhist customs with indigenous practices.Beyond its religious significance,the festival functions as a platform for communal gathering,and the preservation of intangible cultural heritage.Despite modern influences,the resilience of Tiji highlights the dynamic interplay between tradition and adaptation.By unlocking the socio-cultural perspective of this vibrant festival,this study underscores its role in maintaining Mustang’s historical legacy while evolving within contemporary socio-cultural contexts.It reflects this unique synthesis,serving as a means of social cohesion,identity reinforcement,and spiritual renewal for the local community.The elaborate masked dances,sacred chants,and ritualistic performances not only preserve the ancient oral traditions but also convey ethical teachings vital to Mustang’s collective consciousness.The acceptance of creative potential factor of Tiji festival is analyzed through the lenses of cultural theorists that justify call for historical practices of it.
文摘Panchen Rinpoche Sends Greetings for Tibetan New Year 2025 On the Tibetan New Year,Feb.28,Panchen Erdeni Chos-kyi rGyal-po,one of the most influential figures in Tibetan Buddhism,extended greetings to Tibetans at home and abroad.In the speech,he prayed for the young to be wise and for the elderly to enjoy peace and wellbeing,wished for the country's prosperity and world peace,and wished that everything would go well in the Year of the Wood Snake and the virtuous be blessed.
文摘Set against the cultural and historical backdrop of 18th-century Venice,Giulia Elisabetta Lama’s(1681-1747)remarkable paintings reveal intriguing connections to mythology and science.Lama’s notable depiction of Saturn Devouring His Child,painted in oils in 1729 and now in a private collection,draws significant attention.
基金This study received support from the following grants:National Natural Science Foundation of China(82171393 to H.X.)National High Level Hospital Clinical Research Funding(High Quality Clinical Research Project of Peking University First Hospital)(2022CR69 to H.X.)+4 种基金Natural Science Foundation of Beijing Municipality(7212116 to H.X.)National Key Research and Development Program of China(2016YFC0901505 to H.X.)Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases(BZ0317 to H.X.)Research Foundation for Youth Talents of the First Affiliated Hospital of Nanchang University(YFYPY202223 to D.T.)Natural Science Foundation of Beijing Municipality(7242149 to H.L.).
文摘LAMA2-related congenital muscular dystrophy(LAMA2-CMD),characterized by laminin-α2 deficiency,is debilitating and ultimately fatal.To date,no effective therapy has been clinically available.Laminin-a1,which shares significant similarities with laminin-a2,has been proven as a viable compensatory modifier.To evaluate its clinical applicability,we establish a Lama2 exon-3-deletion mouse model(dy^(H)/dy^(H)).The dy^(H)/dy^(H)mice exhibit early lethality and typical LAMA2-CMD phenotypes,allowing the evaluation of various endpoints.In dy^(H)/dy^(H)mice treated with synergistic activation mediator-based CRISPRa-mediated Lama1 upregulation,a nearly doubled median survival is observed,as well as improvements in weight and grip.Significant therapeutical effects are revealed by MRl,serum biochemical indices,and muscle pathology studies.Treating LAMA2-CMD with LAMA1 upregulation is feasible,and early intervention can alleviate symptoms and extend lifespan.Additionally,we reveal the limitations of LAMA1 upregulation,including high-dose mortality and non-sustained expression,which require further optimization in future studies.
基金Supported by The Suzhou Science and Technology Development Plan Guiding Project,No.SZSYYXH-2023-YB5The Suzhou Science and Technology Development Plan Project,No.SKY2023002The Suzhou Key Laboratory of Children's Structural Deformities,No.SZS2022018.
文摘BACKGROUND We report a rare case of primary clinical presentation featuring elevated creatine kinase(CK)levels in a neonate,which is associated with the LAMA2 gene.In this case,a heterozygous mutation in exon5 of the LAMA2 gene,c.715C>G(resulting in a change of nucleotide number 715 in the coding region from cytosine to gua-nine),induced an amino acid alteration p.R239G(No.239)in the patient,repre-senting a missense mutation.This observation may be elucidated by the neonatal creatine monitoring mechanism,a phenomenon not previously reported.CASE SUMMARY We analysed the case of a neonate presenting solely with elevated CK levels who was eventually discharged after supportive treatment.The chief complaint was identification of increased CK levels for 15 d and higher CK values for 1 d.Ad-mission occurred at 18 d of age,and despite prolonged treatment with creatine and vitamin C,the elevated CK levels showed limited improvement.Whole exo-me sequencing revealed the presence of a c.715C>G mutation in LAMA2 in the newborn,correlating with a clinical phenotype.However,the available informa-tion offers insufficient evidence for clinical pathogenicity.CONCLUSION Mutations in LAMA2 are associated with the clinical phenotype of increased neonatal CK levels,for which no specific treatment exists.Whole genome sequen-cing facilitates early diagnosis.
文摘The 17th century was a time during which a numerous secret biographies occurred,and the writing of the elaborate secret biographies started.A Sealed Secret Biography of the 5th Dalai Lama(gsang mam rgy can ma in Tibetan)was a well-known work in the time,yet to be popularized so far,and the academic research on the biography is not fruitful.This article,applying the method of academic research of Philology,discusses the writing style of the A Sealed Secret Biography of the 5th Dalai Lama in general.Specifically,it is found that the concerned biography is written in the writing style of prophesy,and the political history,social situation,the interrelationship among the important figures,and the solidarity of the territory were written in a metaphoric style.In terms of actual writing style,besides this biography was under the influence of A Sealed Secret Biography of Gongthang Lama Zhang(gong thang bla ma zhang gig sang mam bkav rgya ma in Tibetan)in the 12th century and A Secret Biography of Lhadrak Namkha Gyentsen(lho brag nam mkav rgyan mtshn gyi gsang bavi mam thar in Tibetan)in the 14th century,A Sealed Secret Biography of the 5th Dalai Lama displays its unique writing characteristics,which in turned started the secret biography writing of Nyingmapa scholars such as the works of Rikzin Gyurme Doije,the founding father of Mindroling.
