Mouse models of genetic diseases resulting from mutations in elastic fiber proteins

Matrix Biology

Volumn 19, Issue 6, 2000, Pages 481-488

  Dietz, Harry C ^a   Mecham, Robert P ^b  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ELASTIN; FIBRILLIN; MUTANT PROTEIN;

ANIMAL MODEL; AORTA SUPRAVALVULAR STENOSIS; ELASTIC FIBER; ELASTOLYSIS; GENETIC DISORDER; MARFAN SYNDROME; MORPHOGENESIS; MOUSE; MUTATION; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN EXPRESSION; REVIEW;

EID: 0033787772     PISSN: 0945053X     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0945-053X(00)00101-3     Document Type: Short Survey

References (42)

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3. The elastin gene is disrupted by a translocation associated with supravalvular aortic-stenosis
4. TGF-beta1 binding protein-like modules of fibrillin-1 and -2 mediate integrin-dependent cell adhesion
5. Immunolocalization of microfibril and microfibril-associated proteins in the subendothelial matrix of the developing mouse aorta
6. Endothelial cell connecting filaments anchor endothelial cells to the subjacent elastic lamina in the developing aortic intima of the mouse
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14. The fine structure of elastic fibers
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28. Targetting of the gene encoding fibrillin-1 recapitulates the vascular aspect of Marfan syndrome
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36. A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation
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