Spinal and bulbar muscular atrophy (SBMA) is an inherited adult onset motor ne uron disease caused by the expansion of a polyglutamine (polyQ) tract within the androgen receptor (AR), affecting only males. The charact...Spinal and bulbar muscular atrophy (SBMA) is an inherited adult onset motor ne uron disease caused by the expansion of a polyglutamine (polyQ) tract within the androgen receptor (AR), affecting only males. The characteristic pathological f inding is nuclear inclusions (NIs) consisting of mutant AR with an expanded poly Q in residual motor neurons, and in certain visceral organs. We immunohistochemi cally examined 11 SBMA patients at autopsy with 1C2, an antibody that specifical ly recognizes expanded polyQ.Our study demonstrated that diffuse nuclear accumul ation of mutant AR was far more frequent and extensive than NIs being distribute d in a wide array of CNS nuclei, and in more visceral organs than thus far belie ved. Mutant AR accumulation was also present in the cytoplasm, particularly in t he Golgi apparatus; nuclear or cytoplasmic predominance of accumulation was tiss ue specific. Furthermore,the extent of diffuse nuclear accumulation of mutant AR in motor and sensory neurons of the spinal cord was closely related to CAG repe at length. Thus, diffuse nuclear accumulation of mutant AR apparently is a cardi nal pathogenetic process underlying neurological manifestations, as in SBMA tran sgenic mice, while cytoplasmic accumulation may also contribute to SBMA pathophy siology.展开更多
Developing advanced cleaning systems for the removal of unwanted layers from painted surfaces is still a challenging issue that needs to be addressed in the field of cultural heritage.In this work,we proposed the use ...Developing advanced cleaning systems for the removal of unwanted layers from painted surfaces is still a challenging issue that needs to be addressed in the field of cultural heritage.In this work,we proposed the use of novel polyelectrolyte hydrogels prepared via free radical polymerization and freeze-thaw methods.Zwitterionic sulfobetaine methacrylate(SBMA)was crosslinked with methylacryloyloxyethyl trimethylammonium(DMC)through covalent bonding to yield zwitterionic polyelectrolyte poly(SBMA-co-DMC)(PSD).Introducing PSD into the polyvinyl alcohol/carboxymethyl chitosan(PVA/CMC)hydrogel network enhanced the intermolecular electrostatic interactions and changed the porous structure and swelling behaviors,thus leading to improved mechanical properties.The adsorption ability of the PVA/CMC/PSD polyelectrolyte hydrogels toward dyes(rhodamine B and congo red)and metal ions(Cu^(2+)and K^(+))varied with increasing amounts of PSD,which could be attributed to cationic PSD tuning the net charges in the hydrogel.The cleaning effectiveness of the PVA/CMC/PSD polyelectrolyte hydrogels was assessed on wall painting mock-ups coated with soil,red paint,and soluble salts,using microscopic and spectroscopic analyses.The results showed that the hydrogel adhered to rough surfaces and removed unwanted substances effectively without leaving determinable residues,providing a possible safe and efficient method for the cleaning of important artifacts.展开更多
文摘Spinal and bulbar muscular atrophy (SBMA) is an inherited adult onset motor ne uron disease caused by the expansion of a polyglutamine (polyQ) tract within the androgen receptor (AR), affecting only males. The characteristic pathological f inding is nuclear inclusions (NIs) consisting of mutant AR with an expanded poly Q in residual motor neurons, and in certain visceral organs. We immunohistochemi cally examined 11 SBMA patients at autopsy with 1C2, an antibody that specifical ly recognizes expanded polyQ.Our study demonstrated that diffuse nuclear accumul ation of mutant AR was far more frequent and extensive than NIs being distribute d in a wide array of CNS nuclei, and in more visceral organs than thus far belie ved. Mutant AR accumulation was also present in the cytoplasm, particularly in t he Golgi apparatus; nuclear or cytoplasmic predominance of accumulation was tiss ue specific. Furthermore,the extent of diffuse nuclear accumulation of mutant AR in motor and sensory neurons of the spinal cord was closely related to CAG repe at length. Thus, diffuse nuclear accumulation of mutant AR apparently is a cardi nal pathogenetic process underlying neurological manifestations, as in SBMA tran sgenic mice, while cytoplasmic accumulation may also contribute to SBMA pathophy siology.
基金supported by the National Natural Science Foundation of China(Grant No.31900104)the Conservation Science and Technology Project of Zhejiang Provincial Administration of Cultural Heritage(Grant No.2023011)the Fundamental Research Funds for the Central Universities(Grant Nos.S20220161,S20220105)。
文摘Developing advanced cleaning systems for the removal of unwanted layers from painted surfaces is still a challenging issue that needs to be addressed in the field of cultural heritage.In this work,we proposed the use of novel polyelectrolyte hydrogels prepared via free radical polymerization and freeze-thaw methods.Zwitterionic sulfobetaine methacrylate(SBMA)was crosslinked with methylacryloyloxyethyl trimethylammonium(DMC)through covalent bonding to yield zwitterionic polyelectrolyte poly(SBMA-co-DMC)(PSD).Introducing PSD into the polyvinyl alcohol/carboxymethyl chitosan(PVA/CMC)hydrogel network enhanced the intermolecular electrostatic interactions and changed the porous structure and swelling behaviors,thus leading to improved mechanical properties.The adsorption ability of the PVA/CMC/PSD polyelectrolyte hydrogels toward dyes(rhodamine B and congo red)and metal ions(Cu^(2+)and K^(+))varied with increasing amounts of PSD,which could be attributed to cationic PSD tuning the net charges in the hydrogel.The cleaning effectiveness of the PVA/CMC/PSD polyelectrolyte hydrogels was assessed on wall painting mock-ups coated with soil,red paint,and soluble salts,using microscopic and spectroscopic analyses.The results showed that the hydrogel adhered to rough surfaces and removed unwanted substances effectively without leaving determinable residues,providing a possible safe and efficient method for the cleaning of important artifacts.
