Recent molecular biological progress in Marfan syndrome and Marfan-associated disorders
β Scribed by Ling-Gen Gao; Fang Luo; Ru-Tai Hui; Xian-Liang Zhou
- Book ID
- 116255287
- Publisher
- Elsevier Science
- Year
- 2010
- Tongue
- English
- Weight
- 160 KB
- Volume
- 9
- Category
- Article
- ISSN
- 1872-9649
No coin nor oath required. For personal study only.
π SIMILAR VOLUMES
## Abstract Elastic system fibers are composed of two distinct elements, elastin, which is an amorphous component crosslinked in the core, and microfibril, localized in the periphery of elastin. As microfibrillar proteins, fibrillins, microfibrilβassociated glycoproteins (MAGPs), latent TGFβΞ²βbindi
Marfan syndrome (MFS), a multisystem autosomal-dominant disorder, is characterized by mutations of the fibrillin-1 (FBNI) gene and by abnormal patterns of synthesis, secretion, and matrix deposition of the fibrillin protein. To determine the sensitivity and specificity of fibrillin protein abnormali