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hybrid
·인용수 195
·2016
Human-to-mouse prion-like propagation of mutant huntingtin protein
Iksoo Jeon, Francesca Cicchetti, Giulia Cisbani, Suji Lee, Endan Li, Jiwoo Bae, Nayeon Lee, Ling Li, Wooseok Im, Manho Kim, Hyun Sook Kim, Seung-Hun Oh, Tae-Aug Kim, Jung Jae Ko, Benoît Aubé, Abid Oueslati, Yun Joong Kim, Jihwan Song
IF 9.3Acta Neuropathologica
초록

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.

키워드
HuntingtinBiologyInduced pluripotent stem cellHuntingtin ProteinGliosisHuntington's diseaseCell biologyTrinucleotide repeat expansionMutantNeuroscience
타입
article
IF / 인용수
9.3 / 195
게재 연도
2016