PMID- 19259393
OWN - NLM
STAT- MEDLINE
DCOM- 20090826
LR  - 20231213
IS  - 1754-8411 (Electronic)
IS  - 1754-8403 (Print)
IS  - 1754-8403 (Linking)
VI  - 2
IP  - 3-4
DP  - 2009 Mar-Apr
TI  - miR-23 regulation of lamin B1 is crucial for oligodendrocyte development and 
      myelination.
PG  - 178-88
LID - 10.1242/dmm.001065 [doi]
AB  - Duplication of the gene encoding lamin B1 (LMNB1) with increased mRNA and protein 
      levels has been shown to cause severe myelin loss in the brains of adult-onset 
      autosomal dominant leukodystrophy patients. Similar to many neurodegenerative 
      disorders, patients with adult-onset autosomal dominant leukodystrophy are 
      phenotypically normal until adulthood and the defect is specific to the central 
      nervous system despite the ubiquitous expression pattern of lamin B1. We set out 
      to dissect the molecular mechanisms underlying this demyelinating phenotype. 
      Increased lamin B1 expression results in disturbances of inner nuclear membrane 
      proteins, chromatin organization and nuclear pore transport in vitro. It also 
      leads to premature arrest of oligodendrocyte differentiation, which might be 
      caused by reduced transcription of myelin genes and by mislocalization of myelin 
      proteins. We identified the microRNA miR-23 as a negative regulator of lamin B1 
      that can ameliorate the consequences of excessive lamin B1 at the cellular level. 
      Our results indicate that regulation of lamin B1 is important for myelin 
      maintenance and that miR-23 contributes to this process, at least in part, by 
      downregulating lamin B1, therefore establishing novel functions of lamin B1 and 
      miR-23 in the regulation of oligodendroglia development and myelin formation in 
      vitro.
FAU - Lin, Shu-Ting
AU  - Lin ST
AD  - Department of Neurology, University of California San Francisco, 94158, USA.
FAU - Fu, Ying-Hui
AU  - Fu YH
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20090202
PL  - England
TA  - Dis Model Mech
JT  - Disease models & mechanisms
JID - 101483332
RN  - 0 (Chromatin)
RN  - 0 (Lamin Type B)
RN  - 0 (MicroRNAs)
RN  - 0 (Mirn23b microRNA, mouse)
SB  - IM
MH  - Animals
MH  - Cell Line
MH  - Cell Membrane/metabolism
MH  - Cell Nucleus/metabolism
MH  - Chromatin/metabolism
MH  - Gene Duplication
MH  - Gene Expression Regulation, Developmental
MH  - Humans
MH  - Lamin Type B/*genetics/*physiology
MH  - Mice
MH  - MicroRNAs/*genetics
MH  - Models, Biological
MH  - Myelin Sheath/metabolism/*physiology
MH  - Oligodendroglia/*physiology
MH  - Phenotype
PMC - PMC2650193
EDAT- 2009/03/05 09:00
MHDA- 2009/08/27 09:00
PMCR- 2009/03/01
CRDT- 2009/03/05 09:00
PHST- 2008/07/02 00:00 [received]
PHST- 2008/11/17 00:00 [accepted]
PHST- 2009/03/05 09:00 [entrez]
PHST- 2009/03/05 09:00 [pubmed]
PHST- 2009/08/27 09:00 [medline]
PHST- 2009/03/01 00:00 [pmc-release]
AID - 0020178 [pii]
AID - 10.1242/dmm.001065 [doi]
PST - ppublish
SO  - Dis Model Mech. 2009 Mar-Apr;2(3-4):178-88. doi: 10.1242/dmm.001065. Epub 2009 
      Feb 2.