ライフサイエンスコーパス: fibrillin-2

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

10 Concentrations of fibrillin-1, fibrillin-2, and fibulin-4 were measured with novel immu

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

13 In postnatal tissues, fibrillin-2 antibodies demonstrated exuberant staining i

14 Treatment with fibrillin-2 antisense oligodeoxynucleotide induced dysmo

15 Although ADAMTS6 cleaves fibrillin-1 and fibrillin-2 as well as fibronectin, which provides the i

16 re, we show that the corresponding region of fibrillin-2 binds heparin very poorly, highlighting a no

17 ADAMTS17 binds recombinant fibrillin-2 but not fibrillin-1 and does not cleave eith

18 Also, rat fibrillin-2 cDNA was isolated and sequenced and its spat

19 ins was corroborated by the finding that the fibrillin-2 construct did not bind to mature elastin, wh

20 zation of the binding requirements using the fibrillin-2 construct found that a folded, secondary str

21 We recently found that fibrillin-1 and fibrillin-2 control bone formation by regulating osteobl

22 ated that the comparable cysteine residue in fibrillin-2 (Cys(233)) also occurs as a free thiol.

23 ull-length protein, also bound to regions of fibrillin-2 defined by exons 16 and 17, exon 20, and exo

24 Consistent with a specialized function of fibrillin-2, electron microscopy visualized ultrastructu

25 GP-1) interacts with the 8-cysteine motif of fibrillin-2 encoded by exon 24.

26 Fibrillin-1 and fibrillin-2, encoded by FBN1 on chromosome 15q21.1 and F

27 Fibrillin-2 epitopes are also progressively revealed in

28 fined epitopes, demonstrated that N-terminal fibrillin-2 epitopes are masked in postnatal microfibril

29 Both TGFbeta2 and 3 increased fibrillin 2 expression.

30 photoaging were examined for fibrillin-1 and fibrillin-2 expression and microfibril distribution.

31 ealed an approximately 10-kb transcript, and fibrillin-2 expression was developmentally regulated, an

32 nd identified a rare variant p.Glu1144Lys in Fibrillin 2 (FBN2), a glycoprotein of the elastin-rich e

33 rk to vertebrate organogenesis, we generated fibrillin 2 (Fbn2)-null mice by gene targeting and ident

34 brils in cultured fibroblasts and suppresses fibrillin-2 (FBN2) incorporation in microfibrils, in par

35 modify over half of the EGF repeats on FBN1, fibrillin-2 (FBN2), and LTBP1.

36 among others the tumor endothelial antigens fibrillin-2 (Fbn2), elastin microfibril interface-locate

37 d on these results, FBN1 and a related gene, fibrillin-2 (FBN2), were sequenced in a total of 852 AIS

38 Fibrillin 2 filaments were tracked for 12 h throughout t

39 Genetic-linkage analysis has implicated the fibrillin-2 gene (FBN2) as the CCA locus.

40 vely revealed in these mice, suggesting that fibrillin-2 immunoreactivity can serve as a marker for m

41 nding epidermal growth factor-like repeat in fibrillin-2 in a mother and daughter with CCA.

42 In this study, the role of fibrillin-2 in lung development was investigated.

43 d in combination with ADAMTS10 led to excess fibrillin-2 in perichondrium, with impaired skeletal dev

44 ng finding implicates distinct functions for fibrillin-2 in peripheral nerves, because a unique featu

45 ity of microfibrils to determine the role of fibrillin-2 in postnatal microfibril structure.

46 , these data demonstrate that involvement of fibrillin-2 in the initial assembly of the aortic matrix

47 two microfibrillar proteins, fibrillin-1 and fibrillin-2, interact with tropoelastin in solid phase b

48 o be related to the perturbed biology of the fibrillin-2 interacting protein, i.e., elastin, the latt

49 Fibrillin-2 is an extracellular matrix protein.

50 que feature in humans and in mice mutant for fibrillin-2 is joint contractures that resolve over time

51 These data indicate that fibrillin-2 modulates organogenesis of the lung in the c

52 led decreased fibrillin-1 mRNA but unchanged fibrillin-2 mRNA levels in severely photoaged forearm bi

53 Concomitantly, fibrillin-2 mRNA, antibody reactivity in the explants, a

54 Fibrillin-2-null (Fbn2(-/-)) mice display a low bone mas

55 ither TAA nor dissection was associated with fibrillin-2 or fibulin-4.

56 Fibrillin 2 particles initially deposited in the segment

57 normal, as evidenced by normal expression of fibrillin-2 protein (JB3 antigen) and normal formation o

58 e 43 calcium-binding EGF-like domains of the fibrillin-2 protein.

59 eostatic mechanism contributing to postnatal fibrillin-2 reduction and fibrillin-1 dominance.

60 rtilage, and increased GDF5 sequestration in fibrillin-2-rich tissue.

61 NA, antibody reactivity in the explants, and fibrillin-2-specific radioincorporation were reduced.

62 Mutations in fibrillin-1 or fibrillin-2, the major structural components of extracel

63 A spatially analogous fibrillin-2 truncated protein did not coprecipitate the

64 At day 13 of gestation, fibrillin-2 was expressed in the mesenchyme and at the e

65 e-substrate relationship between ADAMTS6 and fibrillin-2 was unequivocally established by reversal of