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10.1007/s00401-016-1582-9 http://scihub22266oqcxt.onion/10.1007/s00401-016-1582-9 C5023734!5023734!27221146     free     free     free Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Acta+Neuropathol 2016 ; 132 (4): 577-92 Nephropedia Template TP {{tp|p=27221146|t=2016. Human-to-mouse prion-like propagation of mutant huntingtin protein |pdf=|usr=}}{{27221146}} gab.com Text Human-to-mouse prion-like propagation of mutant huntingtin protein JO: Acta Neuropathol http://www.kidney.de/pget.php?&tw=1&pmid=27221146 #FOAvip Huntington?s disease (HD) is an autosomal dominant neurodegenerative disorder of the central nervous system (CNS) that is defined by a CAG expansion in exon 1 of the huntingtin gene leading to the production of mutant huntingtin (mHtt). To date, the disease pathophysiology has been thought to be primarily driven by cell-autonomous mechanisms, but, here, we demonstrate that fibroblasts derived from HD patients carrying either 72, 143 and 180 CAG repeats as well as induced pluripotent stem cells (iPSCs) also characterized by 143 CAG repeats can transmit protein aggregates to genetically unrelated and healthy host tissue following implantation into the cerebral ventricles of neonatal mice in a non-cell-autonomous fashion. Transmitted mHtt aggregates gave rise to both motor and cognitive impairments, loss of striatal medium spiny neurons, increased inflammation and gliosis in associated brain regions, thereby recapitulating the behavioural and pathological phenotypes which characterizes HD. In addition, both in vitro work using co-cultures of mouse neural stem cells with 143 CAG fibroblasts and the SH-SY5Y human neuroblastoma cell line as well as in vivo experiments conducted in newborn wild-type mice suggest that exosomes can cargo mHtt between cells triggering the manifestation of HD-related behaviour and pathology. This is the first evidence of human-to-mouse prion-like propagation of mHtt in the mammalian brain; a finding which will help unravel the molecular bases of HD pathology as well as to lead to the development of a whole new range of therapies for neurodegenerative diseases of the CNS.Electronic supplementary material: The online version of this article (doi:10.1007/s00401-016-1582-9) contains supplementary material, which is available to authorized users. Twit Text FOAVip Human-to-mouse prion-like propagation of mutant huntingtin protein ????Acta Neuropathol http://www.kidney.de/pget.php?&tw=1&pmid=27221146 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=27221146 #FOAvip English Wikipedia <ref>{{Cite pmid|27221146}}</ref> Human-to-mouse prion-like propagation of mutant huntingtin protein #MMPMID27221146 Jeon I; Cicchetti F; Cisbani G; Lee S; Li E; Bae J; Lee N; Li L; Im W; Kim M; Kim HS; Oh SH; Kim TA; Ko JJ; Aubé B; Oueslati A; Kim YJ; Song J Acta Neuropathol 2016[]; 132 (4): 577-92 PMID27221146show ga Huntington?s disease (HD) is an autosomal dominant neurodegenerative disorder of the central nervous system (CNS) that is defined by a CAG expansion in exon 1 of the huntingtin gene leading to the production of mutant huntingtin (mHtt). To date, the disease pathophysiology has been thought to be primarily driven by cell-autonomous mechanisms, but, here, we demonstrate that fibroblasts derived from HD patients carrying either 72, 143 and 180 CAG repeats as well as induced pluripotent stem cells (iPSCs) also characterized by 143 CAG repeats can transmit protein aggregates to genetically unrelated and healthy host tissue following implantation into the cerebral ventricles of neonatal mice in a non-cell-autonomous fashion. Transmitted mHtt aggregates gave rise to both motor and cognitive impairments, loss of striatal medium spiny neurons, increased inflammation and gliosis in associated brain regions, thereby recapitulating the behavioural and pathological phenotypes which characterizes HD. In addition, both in vitro work using co-cultures of mouse neural stem cells with 143 CAG fibroblasts and the SH-SY5Y human neuroblastoma cell line as well as in vivo experiments conducted in newborn wild-type mice suggest that exosomes can cargo mHtt between cells triggering the manifestation of HD-related behaviour and pathology. This is the first evidence of human-to-mouse prion-like propagation of mHtt in the mammalian brain; a finding which will help unravel the molecular bases of HD pathology as well as to lead to the development of a whole new range of therapies for neurodegenerative diseases of the CNS.Electronic supplementary material: The online version of this article (doi:10.1007/s00401-016-1582-9) contains supplementary material, which is available to authorized users. äHuman-to-mouse prion-like propagation of mutant huntingtin protein (epmc).pdf DeepDyve Pubget Overpricing