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The Expression of Matrix Metalloprotease 20 is Stimulated by Wild Type but not by 4 bp- or 2 bp- Deletion Mutant DLX3

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Authors
Ryoo, Hyun-Mo; Park, Hyun-Jung; Woo, Kyung-Mi; Kim, Gwan-Shik; Baek, Jeong-Hwa
Issue Date
2009-03
Publisher
Korean Acadamy of Oral Biology
Citation
International Journal of Oral Biology, 2009;34:21-28
Keywords
DLX34bp-del DLX32bp-del DLX3Matrix metalloprotease 20Amelogenesis imperfectaTricho-dento-osseous syndrome
Abstract
Mutations in DLX3 are associated with both autosomal
dominant hypoplastic hypomaturation amelogenesis
imperfecta (ADHHAI) and tricho-dento-osseous (TDO)
syndrome. ADHHAI is caused by a c.561_562delCT (2bpdel
DLX3) mutation whereas TDO syndrome is associated
with a c.571_574delGGGG (4bp-del DLX3) mutation.
However, although the causal relationships between DLX3
and an enamel phenotype have been established, the
pathophysiological role of DLX3 mutations in enamel
development has not yet been clarified. In our current study,
we prepared expression vectors for wild type and deletion
mutant DLX3 products (4bp-del DLX3, 2bp-del DLX3) and
examined the effects of their overexpression on the
expression of the enamel matrix proteins and proteases.
Wild type DLX3 enhanced the expression of matrix
metalloprotease 20 (MMP20) mRNA and protein in murine
ameloblast-like cells. However, neither a 4bp-del nor 2bpdel
DLX3 increased MMP20 expression. Wild type DLX3,
but not the above DLX3 mutants, also increased the activity
of reporters containing 1.5 kb or 0.5 kb of the MMP20
promoter. An examination of protein stability showed that
the half-life of wild type DLX3 protein was less than 12 h
whilst that of both deletion mutants was longer than 24 h.
Endogenous Dlx3 was also found to be continuously
expressed during ameloblast differentiation. Since
inactivating mutations in the gene encoding MMP20 are
associated with amelogenesis imperfecta, the inability of
4bp-del or 2bp-del DLX3 to induce MMP20 expression
suggests a possible involvement of such mutations in the enamel phenotype associated with TDO syndrome or
ADHHAI.
ISSN
1226-7155
Language
English
URI
http://hdl.handle.net/10371/69650
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College of Dentistry/School of Dentistry (치과대학/치의학대학원)Dept. of Dentistry (치의학과)Journal Papers (저널논문_치의학과)
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