PMID- 9303017
OWN - NLM
STAT- MEDLINE
DCOM- 19971009
LR  - 20190727
IS  - 0039-2499 (Print)
IS  - 0039-2499 (Linking)
VI  - 28
IP  - 9
DP  - 1997 Sep
TI  - Increase in elastin gene expression and protein synthesis in arterial smooth 
      muscle cells derived from patients with Moyamoya disease.
PG  - 1733-8
AB  - BACKGROUND AND PURPOSE: Moyamoya disease is a progressive cerebrovascular 
      occlusive disease that is rare in all ages but frequently presents in children. 
      The etiology of the disease is unknown. We examined elastin gene transcripts and 
      elastin synthesis in cultured arterial smooth muscle cells (SMCs) derived from 
      moyamoya patients and compared them with those in SMCs from age-matched control 
      subjects. METHODS: We used six cell strains from moyamoya patients and four from 
      controls. The expression of elastin protein was observed by Western blot analysis 
      and metabolic labeling with 3H-valine. Elastin gene transcripts were identified 
      by Northern blot analysis. RESULTS: Elastin mRNA and protein levels were elevated 
      in all SMCs from moyamoya patients compared with control SMCs. Although 
      transforming growth factor-beta 1 (TGF-beta 1), a potent enhancer of the 
      expression of elastin in arterial SMCs, upregulated elastin mRNA and protein 
      levels in SMCs from both moyamoya patients and control subjects, the maximum 
      levels of elastin synthesis and elastin gene transcripts in response to exogenous 
      TGF-beta 1 were significantly greater in moyamoya SMCs than control SMCs. In 
      addition, quiescent moyamoya SMCs secreted significantly more TGF-beta 1 into the 
      culture medium than quiescent control SMCs (P < .01). CONCLUSIONS: Our findings 
      suggest that moyamoya disease may result, at least in part, from an abnormal 
      regulation of extracellular matrix metabolism that leads to increased steady 
      state levels of elastin mRNA and elastin accumulation in the intimal thickening 
      and that increased elastin accumulation is a stable marker of SMCs from patients 
      with moyamoya disease.
FAU - Yamamoto, M
AU  - Yamamoto M
AD  - Department of Cell Biology, Tokyo Metropolitan Institute of Gerontology, Japan.
FAU - Aoyagi, M
AU  - Aoyagi M
FAU - Tajima, S
AU  - Tajima S
FAU - Wachi, H
AU  - Wachi H
FAU - Fukai, N
AU  - Fukai N
FAU - Matsushima, Y
AU  - Matsushima Y
FAU - Yamamoto, K
AU  - Yamamoto K
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Stroke
JT  - Stroke
JID - 0235266
RN  - 0 (Transforming Growth Factor beta)
RN  - 9007-58-3 (Elastin)
SB  - IM
MH  - Adolescent
MH  - Arteries/pathology/*physiopathology
MH  - Blotting, Northern
MH  - Blotting, Western
MH  - Cells, Cultured
MH  - Child
MH  - Child, Preschool
MH  - Elastin/*genetics/*metabolism
MH  - Electrophoresis, Polyacrylamide Gel
MH  - Female
MH  - *Gene Expression
MH  - Humans
MH  - Male
MH  - Moyamoya Disease/*genetics/*metabolism/pathology
MH  - Muscle, Smooth/pathology/*physiopathology
MH  - Transforming Growth Factor beta/metabolism/pharmacology
EDAT- 1997/09/26 00:00
MHDA- 1997/09/26 00:01
CRDT- 1997/09/26 00:00
PHST- 1997/09/26 00:00 [pubmed]
PHST- 1997/09/26 00:01 [medline]
PHST- 1997/09/26 00:00 [entrez]
AID - 10.1161/01.str.28.9.1733 [doi]
PST - ppublish
SO  - Stroke. 1997 Sep;28(9):1733-8. doi: 10.1161/01.str.28.9.1733.