ライフサイエンスコーパス: matrilin

1 h matrilin 1, and that the total ablation of matrilin 1 expression has no impact on disease severity

2 investigate the hypothesis that deletion of matrilin 1 would abolish the formation of matrilin 1/mat

3 not dependent on hetero-oligomerization with matrilin 1, and that the total ablation of matrilin 1 ex

4 that were homozygous for V194D and null for matrilin 1.

5 ed aggregates and caused the co-retention of matrilin 1.

6 nt on the formation of hetero-oligomers with matrilin 1.

7 of matrilin 1 would abolish the formation of matrilin 1/matrilin 3 hetero-oligomers, eliminate the se

8 us angiogenesis inhibitors, we have purified matrilin-1 (MATN-1) and have demonstrated, for the first

9 Matrilin-1 and epiphycan were specific for rib and trach

10 ss of this domain occurs as a consequence of matrilin-1 deficiency.

11 Matrilin-1 displays a multiphasic expression during zebr

12 region, matrilin-2 will form a homotrimer as matrilin-1 does.

13 on zone, in addition to its co-assembly with matrilin-1 during endochondral ossification.

14 n of early chondrocytes that do not activate matrilin-1 expression rather than by redifferentiation f

15 c molecule with a noncollagenous A domain of matrilin-1 fused to the N terminus of NC1.

16 nase gene has been targeted to exon 1 of the matrilin-1 gene (Matn1) to investigate the origins of ar

17 e chondrocytes did not turn on expression of matrilin-1 in contrast to the other chondrocytes of the

18 However, gene targeting of matrilin-1 in mouse did not lead to pronounced phenotype

19 appears to function in the matrix linked to matrilin-1 in the form of disulfide-bonded heteromeric m

20 Later, when the skeleton develops, matrilin-1 is expressed mainly in cartilage.

21 The results show that matrilin-1 is indispensible for zebrafish cartilage form

22 expression at about 15 h post-fertilization, matrilin-1 is present throughout the zebrafish embryo wi

23 Matrilin-1 is the prototypical member of the matrilin pr

24 During the early expression phase the matrilin-1 knockdown had no effects on cell morphology,

25 with that of the mature chondrocyte-abundant matrilin-1 mRNA.

26 Morpholino knockdown of matrilin-1 results both in overall growth defects and in

27 derived from cells that have never expressed matrilin-1 whereas the remainder of the chondrocytes in

28 that, in primary chondrocyte cultures, CMP (matrilin-1) forms a filamentous network, which is made u

29 Matrilin-3 and CMP (matrilin-1) were prominent in equimolar amounts in fetal

30 sults indicate a molecular stoichiometry of (matrilin-1)2(matrilin-3)2.

31 One potential antigen is matrilin-1, a cartilage matrix protein found uniquely in

32 ain organization to cartilage matrix protein/matrilin-1, but information on the protein structure is

33 Matrilin-1, matrilin-4, epiphycan, and thrombospondin-4

34 ndrocytes in the cartilage anlagen expresses matrilin-1.

35 directed toward the cartilage matrix protein matrilin-1.

36 de corresponding to the C-terminal domain of matrilin-1.

37 rmined that the extracellular matrix protein matrilin-2 (MATN2) is an endogenous neuronal molecule th

38 Matrilin-2 is a member of von Willebrand factor A contai

39 he low sequence identity within this region, matrilin-2 will form a homotrimer as matrilin-1 does.

40 f laminin gamma3 chain, nidogen-2, netrin-4, matrilin-2, and matrilin-4 is described in the cornea fo

41 sponding to the C-terminal sequence of mouse matrilin-2.

42 Mutations in matrilin 3 can result in multiple epiphyseal dysplasia (

43 Retained mutant matrilin 3 formed disulfide-bonded aggregates and caused

44 1 would abolish the formation of matrilin 1/matrilin 3 hetero-oligomers, eliminate the secretion of

45 us, it was proposed that secretion of mutant matrilin 3 may be dependent on the formation of hetero-o

46 Mutant matrilin 3 oligomers form non-native disulfide-bonded ag

47 We showed that secretion of matrilin 3 V194D mutant protein is not dependent on hete

48 ar matrix, and the amount of retained mutant matrilin 3 was not noticeably increased.

49 A similar proportion of mutant matrilin 3 was present in the extracellular matrix, and

50 cellular retention of the majority of mutant matrilin 3 was previously observed in a murine model of

51 oligomers, eliminate the secretion of mutant matrilin 3, and influence disease severity.

52 Type IX collagen and matrilin-3 (MATN3), also accumulate in the rER cisternae

53 n result from mutations in the gene encoding matrilin-3 (MATN3).

54 Matrilin-3 and CMP (matrilin-1) were prominent in equimo

55 ts of the ADAMTS-4 cleavage motif identified matrilin-3 as a new substrate for ADAMTS-4.

56 ombinant ADAMTS-4 effectively cleaved intact matrilin-3 at the predicted motif at Glu435/Ala436 gener

57 hanism of polymeric assembly is unknown, the matrilin-3 chain appears to function in the matrix linke

58 in family of proteins and confirm a role for matrilin-3 in the development and homeostasis of cartila

59 the von Willebrand factor A (vWFA) domain of matrilin-3 in two unrelated families with MED (EDM5).

60 Matrilin-3 is an oligomeric protein that is present in t

61 Mutant matrilin-3 is retained within the rough endoplasmic reti

62 Matrilin-3 knock-out (Matn3 KO) mice exhibit these featu

63 Thus matrilin-3 may assemble into both homotypic and heteroty

64 This suggests that matrilin-3 may self-assemble in the proliferation zone,

65 e a molecular stoichiometry of (matrilin-1)2(matrilin-3)2.

66 A data base search identified the latter as matrilin-3, a molecule recently predicted from human and

67 To understand the molecular properties of matrilin-3, a newly discovered member of the novel extra

68 ork, the interacting complex of collagen IX, matrilin-3, and cartilage oligomeric matrix protein (COM

69 ssociated with dramatically reduced COMP and matrilin-3, consistent with known interactions.

70 can-3 (GPC3), phospholipid transfer protein, matrilin-3, tissue factor pathway inhibitor, fibrinogen-

71 r candidate genes identified MATN3, encoding matrilin-3, within the critical region.

72 e extracellular matrix (ECM) adaptor protein Matrilin-4 (Matn4) as an important negative regulator of

73 chain, nidogen-2, netrin-4, matrilin-2, and matrilin-4 is described in the cornea for the first time

74 Matrilin-4 largely disappeared after the age of 3 years.

75 ssion was reduced at the mRNA level, whereas matrilin-4 was verified as a novel collagen IX-binding p

76 Matrilin-4, collagen XII, thrombospondin-4, fibronectin,

77 Matrilin-1, matrilin-4, epiphycan, and thrombospondin-4 levels were

78 h as cartilage intermediate layer protein 1, matrilin-4, extracellular adipocyte enhancer binding pro

79 mtDNA have shown reciprocal monophyly of the matrilines among seven of the nine assumed subspecies [3

80 stimate the age of North American indigenous matrilines and patrilines.

81 taining VWA-like domains related to those in matrilins and collagens (AMACO), encoded by the VWA2 gen

82 sed from F1 and F2 families from each of the matrilines, and were found to differ significantly betwe

83 ellular matrix components such as collagens, matrilins, and proteoglycans.

84 MAT-3 and MAT-1 cDNAs results in three major matrilins as follows: (MAT-1)(3), (MAT-3)(4), and (MAT-1

85 ated adhesion mechanism may be applicable to matrilin assembly.

86 ent lead to the formation and persistence of matrilines at habitat sites.

87 d other extracellular matrix proteins (e.g., matrilins, cochlin/vitrin, and von Willebrand factor).

88 identified in any of the genes encoding the matrilin family of proteins and confirm a role for matri

89 MP) is the prototype of the newly discovered matrilin family, all of which contain von Willebrand fac

90 Thirty-six matrilines from a single Muscovite sample of Adalia bipu

91 We established two matrilines from the same population of the linyphiid spi

92 e zebrafish as an alternative model to study matrilin function in vivo.

93 ed in (i) infection of previously uninfected matrilines, (ii) a double infection in a matriline alrea

94 suggest that the varying synthetic levels of matrilins in different zones of a growth plate may resul

95 uencies of PPC were highly consistent within matrilines, indicating that individuals maintained lifel

96 Decreased fitness in matrilines of four or five was associated with agonistic

97 of a growth plate may result in a change of matrilin oligomeric forms during endochondral ossificati

98 Matrilin-1 is the prototypical member of the matrilin protein family and is highly expressed in carti

99 ncreased when mortality and fission of large matrilines reduced group size and the surviving females

100 Although the function of matrilins remain unclear, we have shown that, in primary

101 Both size and number of matrilines varied among sites and survivorship and net r