PMID- 30718999
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 1662-5102 (Print)
IS  - 1662-5102 (Electronic)
IS  - 1662-5102 (Linking)
VI  - 12
DP  - 2018
TI  - Brain Derived Neurotrophic Factor (BDNF) Delays Onset of Pathogenesis in 
      Transgenic Mouse Model of Spinocerebellar Ataxia Type 1 (SCA1).
PG  - 509
LID - 10.3389/fncel.2018.00509 [doi]
LID - 509
AB  - Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused 
      by an abnormal expansion of CAG repeats in the Ataxin-1 (ATXN1) gene and 
      characterized by motor deficits and cerebellar neurodegeneration. Even though 
      mutant ATXN1 is expressed from an early age, disease onset usually occurs in 
      patient's mid-thirties, indicating the presence of compensatory factors that 
      limit the toxic effects of mutant ATXN1 early in disease. Brain derived 
      neurotrophic factor (BDNF) is a growth factor known to be important for the 
      survival and function of cerebellar neurons. Using gene expression analysis, we 
      observed altered BDNF expression in the cerebella of Purkinje neuron specific 
      transgenic mouse model of SCA1, ATXN1[82Q] mice, with increased expression during 
      the early stage and decreased expression in the late stage of disease. We 
      therefore investigated the potentially protective role of BDNF in early stage 
      SCA1 through intraventricular delivery of BDNF via ALZET osmotic pumps. Extrinsic 
      BDNF delivery delayed onset of motor deficits and Purkinje neuron pathology in 
      ATXN1[82Q] mice supporting its use as a novel therapeutic for SCA1.
FAU - Mellesmoen, Aaron
AU  - Mellesmoen A
AD  - Department of Neuroscience, University of Minnesota, Minneapolis, MN, United 
      States.
FAU - Sheeler, Carrie
AU  - Sheeler C
AD  - Department of Neuroscience, University of Minnesota, Minneapolis, MN, United 
      States.
FAU - Ferro, Austin
AU  - Ferro A
AD  - Department of Neuroscience, University of Minnesota, Minneapolis, MN, United 
      States.
FAU - Rainwater, Orion
AU  - Rainwater O
AD  - Department of Lab Medicine and Pathology, University of Minnesota, Minneapolis, 
      MN, United States.
FAU - Cvetanovic, Marija
AU  - Cvetanovic M
AD  - Department of Neuroscience, University of Minnesota, Minneapolis, MN, United 
      States.
AD  - Institute for Translational Neuroscience, University of Minnesota, Minneapolis, 
      MN, United States.
LA  - eng
GR  - R01 NS107387/NS/NINDS NIH HHS/United States
PT  - Journal Article
DEP - 20190121
PL  - Switzerland
TA  - Front Cell Neurosci
JT  - Frontiers in cellular neuroscience
JID - 101477935
PMC - PMC6348256
OTO - NOTNLM
OT  - ATAXIN-1
OT  - BDNF
OT  - astroglia
OT  - cerebellum
OT  - neurodegeneration
OT  - neuroprotective
OT  - nuclear factor kappab
EDAT- 2019/02/06 06:00
MHDA- 2019/02/06 06:01
PMCR- 2018/01/01
CRDT- 2019/02/06 06:00
PHST- 2018/09/11 00:00 [received]
PHST- 2018/12/10 00:00 [accepted]
PHST- 2019/02/06 06:00 [entrez]
PHST- 2019/02/06 06:00 [pubmed]
PHST- 2019/02/06 06:01 [medline]
PHST- 2018/01/01 00:00 [pmc-release]
AID - 10.3389/fncel.2018.00509 [doi]
PST - epublish
SO  - Front Cell Neurosci. 2019 Jan 21;12:509. doi: 10.3389/fncel.2018.00509. 
      eCollection 2018.