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1 and congenital contractural arachnodactyly (fibrillin-2).
2 ssembly, including fibulin-4, fibulin-5, and fibrillin-2.
3 xon 24 and portions of the central region of fibrillin-2.
4 nd can differentiate between fibrillin-1 and fibrillin-2.
5 tin-associated microfibrillar protein called fibrillin-2.
6 n fibrillin-1 and the second RGD sequence in fibrillin-2.
7 the glycine-rich and proline-rich regions of fibrillin-2 and fibrillin-1, respectively, and continuin
8 It had approximately 88% homology with human fibrillin-2 and had Ca(2+) binding epidermal growth fact
9 f immunolabeled ECM components (fibronectin, fibrillin-2) and TIE1 positive endocardial progenitors i
11 everal glycoproteins, including fibrillin-1, fibrillin-2, and MAGP1/2 (microfibril-associated glycopr
12 h aneurysm formation, including fibrillin-1, fibrillin-2, and type III procollagen, and chromosome 3p
15 re, we show that the corresponding region of fibrillin-2 binds heparin very poorly, highlighting a no
18 ins was corroborated by the finding that the fibrillin-2 construct did not bind to mature elastin, wh
19 zation of the binding requirements using the fibrillin-2 construct found that a folded, secondary str
22 ull-length protein, also bound to regions of fibrillin-2 defined by exons 16 and 17, exon 20, and exo
23 Consistent with a specialized function of fibrillin-2, electron microscopy visualized ultrastructu
27 fined epitopes, demonstrated that N-terminal fibrillin-2 epitopes are masked in postnatal microfibril
29 photoaging were examined for fibrillin-1 and fibrillin-2 expression and microfibril distribution.
30 ealed an approximately 10-kb transcript, and fibrillin-2 expression was developmentally regulated, an
31 nd identified a rare variant p.Glu1144Lys in Fibrillin 2 (FBN2), a glycoprotein of the elastin-rich e
32 rk to vertebrate organogenesis, we generated fibrillin 2 (Fbn2)-null mice by gene targeting and ident
33 brils in cultured fibroblasts and suppresses fibrillin-2 (FBN2) incorporation in microfibrils, in par
34 d on these results, FBN1 and a related gene, fibrillin-2 (FBN2), were sequenced in a total of 852 AIS
37 vely revealed in these mice, suggesting that fibrillin-2 immunoreactivity can serve as a marker for m
40 ng finding implicates distinct functions for fibrillin-2 in peripheral nerves, because a unique featu
42 , these data demonstrate that involvement of fibrillin-2 in the initial assembly of the aortic matrix
43 two microfibrillar proteins, fibrillin-1 and fibrillin-2, interact with tropoelastin in solid phase b
44 o be related to the perturbed biology of the fibrillin-2 interacting protein, i.e., elastin, the latt
46 que feature in humans and in mice mutant for fibrillin-2 is joint contractures that resolve over time
48 led decreased fibrillin-1 mRNA but unchanged fibrillin-2 mRNA levels in severely photoaged forearm bi
53 normal, as evidenced by normal expression of fibrillin-2 protein (JB3 antigen) and normal formation o
55 NA, antibody reactivity in the explants, and fibrillin-2-specific radioincorporation were reduced.
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