Schwann-like adipose-derived stem cells and nerve injury:Peripheral nerve injuries(PNIs)are a common clinical problem usually as a consequence of trauma.Despite optimal surgical management,PNI has a lifelong impact on...Schwann-like adipose-derived stem cells and nerve injury:Peripheral nerve injuries(PNIs)are a common clinical problem usually as a consequence of trauma.Despite optimal surgical management,PNI has a lifelong impact on function and wellbeing of the patient.The peripheral nervous system(PNS)has regenerative capability,in contrast to the central nervous system(CNS),and is dependent on the plasticity of the peripheral glia,Schwann cells(SCs).Despite this regenerative capability,PNI recovery of sensorial and motor function is always incomplete causing pain,cold intolerance,paralysis and impairment of activities of daily living.展开更多
Frontotemporal lobar degeneration(FTLD)represents a group of clinically,neuropathologically and genetically heterogeneous disorders with plenty of overlaps between the neurodegenerative mechanism and the clinical phen...Frontotemporal lobar degeneration(FTLD)represents a group of clinically,neuropathologically and genetically heterogeneous disorders with plenty of overlaps between the neurodegenerative mechanism and the clinical phenotype.FTLD is pathologically characterized by the frontal and temporal lobar atrophy.Frontotemporal dementia(FTD)clinically presents with abnormalities of behavior and personality and language impairments variants.The clinical spectrum of FTD encompasses distinct canonical syndromes:behavioural variant of FTD(bvFTD)and primary progressive aphasia.The later includes nonfluent/agrammatic variant PPA(nfvPPA or PNFA),semantic variant PPA(svPPA or SD)and logopenic variant PPA(lvPPA).In addition,there is also overlap of FTD with motor neuron disease(FTD-MND or FTD-ALS),as well as the parkinsonian syndromes,progressive supranuclear palsy(PSP)and corticobasal syndrome(CBS).The FTLD spectrum disorders are based upon the predominant neuropathological proteins(containing inclusions of hyperphosphorylated tau or ubiquitin protein,e.g transactive response(TAR)DNA-binding protein 43 kDa(TDP-43)and fusedin-sarcoma protein in neurons and glial cells)into three main categories:(1)microtubule-associated protein tau(FTLD-Tau);(2)TAR DNA-binding protein-43(FTLD-TDP);and(3)fused in sarcoma protein(FTLD-FUS).There are five main genes mutations leading clinical and pathological variants in FTLD that identified by molecular genetic studies,which are chromosome 9 open reading frame 72(C9ORF72)gene,granulin(GRN)gene,microtubule associated protein tau gene(MAPT),the gene encoding valosin-containing protein(VCP)and the charged multivesicular body protein 2B(CHMP2B).In this review,recent advances on the different clinic variants,neuroimaging,genetics,pathological subtypes and clinicopathological associations of FTD will be discussed.展开更多
基金supported by Ateneo Sapienza Funds 2017(RM11715C7F959CA4)“Avvio Giovani”Project 2018(AR11816435A0F81D)from Ateneo Sapienza to RP.RP fellowship was also supported by CIB 2018+1 种基金AF and AJR are supported by the Hargreaves and Ball Trust,the Academy of Medical Sciences(AMS-SGCL7)by Seed Corn Funding from the Rosetrees Trust and the Stoneygate Trust(M746).
文摘Schwann-like adipose-derived stem cells and nerve injury:Peripheral nerve injuries(PNIs)are a common clinical problem usually as a consequence of trauma.Despite optimal surgical management,PNI has a lifelong impact on function and wellbeing of the patient.The peripheral nervous system(PNS)has regenerative capability,in contrast to the central nervous system(CNS),and is dependent on the plasticity of the peripheral glia,Schwann cells(SCs).Despite this regenerative capability,PNI recovery of sensorial and motor function is always incomplete causing pain,cold intolerance,paralysis and impairment of activities of daily living.
基金This work was supported by the grants from the National Natural Science Foundation of China(81200991)Outstanding Young Persons’Research Program for Higher Education of Fujian Province,China(JA10123)Major Project of Fujian Science and Technology Bureau(2009D061).
文摘Frontotemporal lobar degeneration(FTLD)represents a group of clinically,neuropathologically and genetically heterogeneous disorders with plenty of overlaps between the neurodegenerative mechanism and the clinical phenotype.FTLD is pathologically characterized by the frontal and temporal lobar atrophy.Frontotemporal dementia(FTD)clinically presents with abnormalities of behavior and personality and language impairments variants.The clinical spectrum of FTD encompasses distinct canonical syndromes:behavioural variant of FTD(bvFTD)and primary progressive aphasia.The later includes nonfluent/agrammatic variant PPA(nfvPPA or PNFA),semantic variant PPA(svPPA or SD)and logopenic variant PPA(lvPPA).In addition,there is also overlap of FTD with motor neuron disease(FTD-MND or FTD-ALS),as well as the parkinsonian syndromes,progressive supranuclear palsy(PSP)and corticobasal syndrome(CBS).The FTLD spectrum disorders are based upon the predominant neuropathological proteins(containing inclusions of hyperphosphorylated tau or ubiquitin protein,e.g transactive response(TAR)DNA-binding protein 43 kDa(TDP-43)and fusedin-sarcoma protein in neurons and glial cells)into three main categories:(1)microtubule-associated protein tau(FTLD-Tau);(2)TAR DNA-binding protein-43(FTLD-TDP);and(3)fused in sarcoma protein(FTLD-FUS).There are five main genes mutations leading clinical and pathological variants in FTLD that identified by molecular genetic studies,which are chromosome 9 open reading frame 72(C9ORF72)gene,granulin(GRN)gene,microtubule associated protein tau gene(MAPT),the gene encoding valosin-containing protein(VCP)and the charged multivesicular body protein 2B(CHMP2B).In this review,recent advances on the different clinic variants,neuroimaging,genetics,pathological subtypes and clinicopathological associations of FTD will be discussed.
