Abstract |
Marfan's syndrome (MFS) is a systemic disorder of the connective tissues caused by insufficient fibrillin-1 microfibril formation and can cause cardiac complications, emphysema, ocular lens dislocation and severe periodontal disease. ADAMTSL6β, a microfibril-associated extracellular matrix protein that has been implicated in fibrillin-1 microfibril assembly is able to improve microfibril insufficiency in MFS mice model. These findings suggest a new therapeutic strategy for the treatment of MFS through ADAMTSL6β-mediated fibrillin-1 microfibril assembly. We here review effect on ADAMTSL6β to the improvement of microfibril insufficiency in periodontal tissue as a model.
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Authors | Masahiro Saito, Takashi Tsuji |
Journal | Clinical calcium
(Clin Calcium)
Vol. 22
Issue 1
Pg. 35-42
(Jan 2012)
ISSN: 0917-5857 [Print] Japan |
PMID | 22201097
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
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Chemical References |
- Extracellular Matrix Proteins
- FBN1 protein, human
- Fbn1 protein, mouse
- Fibrillin-1
- Fibrillins
- Microfilament Proteins
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Topics |
- Animals
- Extracellular Matrix Proteins
(physiology)
- Fibrillin-1
- Fibrillins
- Humans
- Marfan Syndrome
(genetics, physiopathology, therapy)
- Mice
- Microfibrils
(metabolism, physiology)
- Microfilament Proteins
(physiology)
- Molecular Targeted Therapy
- Periodontal Ligament
(physiopathology)
- Protein Multimerization
- Regeneration
- Wound Healing
(genetics)
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