ライフサイエンスコーパス: hemojuvelin

1 ease TMPRSS6 that cleaves the BMP coreceptor hemojuvelin.

2 d conditional knockout mice that lack muscle hemojuvelin.

3 e membrane involving BMP, its receptors, and hemojuvelin.

4 rified by its binding to an uncleaved mutant hemojuvelin.

5 errin receptor 2, ferroportin, hepcidin, and hemojuvelin.

6 petition between soluble and cell-associated hemojuvelin.

7 Repulsive guidance molecule c (RGMc or hemojuvelin), a glycosylphosphatidylinositol-linked glyc

8 Hemojuvelin, a glycophosphatidyl inositol-linked membran

9 Hemojuvelin, a protein critical for maintaining appropri

10 support a key role for BMP6 as a ligand for hemojuvelin and an endogenous regulator of hepcidin expr

11 e hepcidin expression, a process enhanced by hemojuvelin and blunted in Hfe2-/- hepatocytes.

12 we report expression of soluble versions of hemojuvelin and neogenin for biochemical characterizatio

13 hepatocytes in vitro; and measured hepcidin, hemojuvelin, and ferroportin-1 gene expression.

14 s of hepcidin mRNA, it did not interact with hemojuvelin, and interference with its signaling pathway

15 y of a new gene involved in iron metabolism, hemojuvelin, and new data on the role of HFE mutations i

16 endent pathway requires the presence of Hfe, hemojuvelin, and probably transferrin receptor 2 (tfr-2)

17 We show that a robust BMP, hemojuvelin, and SMAD1 response by murine Hamp1 is depen

18 y known iron metabolic proteins such as HFE, hemojuvelin, and transferrin receptor 2 are expanding th

19 Neogenin binds to cleaved and noncleaved hemojuvelin, as verified by its binding to an uncleaved

20 We localized the hemojuvelin binding site on neogenin to the membrane-pro

21 hanism of this process is not clear, several hemojuvelin-binding proteins, including the cell surface

22 ulate hepcidin mRNA concentrations through a hemojuvelin/BMP2/4-dependent pathway.

23 coordination of multiple proteins, including hemojuvelin, bone morphogenetic protein 6 (BMP6), heredi

24 The protein product of HJV, hemojuvelin, contains a C-terminal glycosylphosphatidyli

25 which HFE2 mutations cause hemochromatosis: hemojuvelin dysfunction decreases BMP signaling, thereby

26 alytic cleavage, and as in vivo, recombinant hemojuvelin exists as a mixture of cleaved and uncleaved

27 ding HFE, transferrin receptor 2 (TfR2), and hemojuvelin, function at least in part, by modulating th

28 s caused by mutations in the recently cloned hemojuvelin gene (HJV; also called HFE2), whereas Type 2

29 rotein (HFE), transferrin-receptor 2 (TfR2), hemojuvelin, hepcidin, or ferroportin genes.

30 ee hemochromatosis-associated proteins: HJV (hemojuvelin), HFE (hemochromatosis protein), and TfR2 (t

31 BMP2/4 and hemochromatosis-related proteins hemojuvelin, HFE and transferrin receptor 2, also regula

32 iron regulatory hormone hepcidin (HAMP) and hemojuvelin (HFE2).

33 Mutations in the gene encoding hemojuvelin (HFE2, also known as HJV) cause severe iron

34 Both hemojuvelin (HJV) and bone morphogenic protein-6 (BMP6)

35 Cells co-transfected with hemojuvelin (HJV) and matriptase-2 (MT-2) were used as a

36 Hemojuvelin (HJV) and matriptase-2 (MT2) are co-expresse

37 (BMPs) through a receptor complex requiring hemojuvelin (HJV) as a co-receptor.

38 ic proteins (BMPs), apparently by binding to hemojuvelin (Hjv) as a coreceptor and signaling through

39 Mutations in hemojuvelin (HJV) cause severe, early-onset juvenile hem

40 the HFE, transferrin receptor-2 (TfR2), and hemojuvelin (HJV) genes.

41 netic protein 6 (BMP6) or the BMP coreceptor hemojuvelin (HJV) in mice leads to a similar phenotype w

42 Hemojuvelin (HJV) is a coreceptor for bone morphogenetic

43 Hemojuvelin (HJV) is a glycosylphosphatidylinositol-link

44 Hemojuvelin (HJV) is an important regulator of iron meta

45 severe form, usually caused by mutations in hemojuvelin (HJV) or hepcidin (HAMP).

46 Hemojuvelin (HJV) regulates iron homeostasis by direct i

47 Hemojuvelin (HJV) was recently identified as a critical

48 ontrast, mice deficient for both Tmprss6 and hemojuvelin (Hjv), a BMP coreceptor that augments hepcid

49 to regulate hepcidin production by cleaving Hemojuvelin (Hjv), a key modulator of hepcidin expressio

50 Hemojuvelin (HJV), encoded by the gene HFE2, is a critic

51 Hemojuvelin (HJV), HFE, and transferrin receptor-2 (TfR2

52 rrence of compound heterozygous mutations in hemojuvelin (HJV), including a termination codon, in a p

53 Its encoded protein, hemojuvelin (HJV), is a co-receptor for the bone morphog

54 RGMc, also called hemojuvelin (HJV), is essential for iron homeostasis.

55 tions in HFE, transferrin receptor 2 (Tfr2), hemojuvelin (HJV), or bone morphogenetic protein 6 (BMP6

56 sis type 2 (HFE2), which encodes the protein hemojuvelin (HJV), result in the absence of hepcidin and

57 Similarly, RPE cells from hemojuvelin (Hjv)-knockout mice, another model of hemoch

58 is strongly modulated by the BMP coreceptor hemojuvelin (HJV).

59 main is greatly attenuated in the absence of hemojuvelin (Hjv).

60 y iron loading in hh are hepcidin (HAMP) and hemojuvelin (HJV).

61 We also show that soluble hemojuvelin (HJV.Fc) selectively inhibits BMP induction

62 Here we show that compared with soluble hemojuvelin (HJV.Fc), the homologous DRAGON.Fc is a more

63 Hemojuvelin (HJV; also called repulsive guidance molecul

64 However, neither the specific role of hemojuvelin in maintaining iron homeostasis nor its rela

65 idin expression were not affected by loss of hemojuvelin in skeletal muscle regardless of dietary iro

66 To explore possible roles for hemojuvelin in skeletal muscle, we analyzed conditional

67 ron regulatory hormone, by cleaving membrane hemojuvelin into an inactive form.

68 Here, we report that hemojuvelin is a BMP coreceptor and that hemojuvelin mut

69 Hemojuvelin is a bone morphogenetic protein (BMP) co-rec

70 We have previously shown that hemojuvelin is a co-receptor for bone morphogenetic prot

71 We have recently shown that hemojuvelin is a coreceptor for bone morphogenetic prote

72 Hemojuvelin is a member of the repulsive guidance molecu

73 Hemojuvelin is a recently identified iron-regulatory pro

74 ave any known function outside of the liver, hemojuvelin is expressed at very high levels in cardiac

75 de that, in spite of its expression pattern, hemojuvelin is primarily important in the liver.

76 We used hemojuvelin-knockout (Hjv(-/-)) mice to examine whether

77 we used hemojuvelin-specific siRNAs to vary hemojuvelin mRNA concentration and showed that cellular

78 hat hemojuvelin is a BMP coreceptor and that hemojuvelin mutants associated with hemochromatosis have

79 mochromatosis is associated with hepcidin or hemojuvelin mutations, and these patients have low or ab

80 Hemojuvelin normally undergoes an autocatalytic cleavage

81 rarer instances, transferrin-receptor 2 and hemojuvelin, or make its receptor ferroportin, resistant

82 mRNA concentration and showed that cellular hemojuvelin positively regulated hepcidin mRNA expressio

83 te with low hepcidin levels, suggesting that hemojuvelin positively regulates hepcidin expression.

84 at BMP-2 and neogenin bind simultaneously to hemojuvelin raises the possibility that neogenin is part

85 We propose that soluble and cell-associated hemojuvelin reciprocally regulate hepcidin expression in

86 lthough our previous studies have shown that hemojuvelin regulates hepcidin expression and iron metab

87 s engineered to express hemojuvelin, soluble hemojuvelin release was progressively inhibited by incre

88 Inactivating mutations in hemojuvelin/repulsive guidance molecule c (HJV/RGMc) cau

89 guidance molecules RGMa, DRAGON (RGMb), and hemojuvelin (RGMc) as coreceptors for BMP signaling.

90 ption-level changes of the murine homolog of Hemojuvelin (Rgmc).

91 We also showed that recombinant soluble hemojuvelin (rs-hemojuvelin) suppressed hepcidin mRNA ex

92 In cells engineered to express hemojuvelin, soluble hemojuvelin release was progressive

93 In this study we used hemojuvelin-specific siRNAs to vary hemojuvelin mRNA con

94 present study, we show in mice that loss of hemojuvelin specifically in the liver leads to decreased

95 wed that recombinant soluble hemojuvelin (rs-hemojuvelin) suppressed hepcidin mRNA expression in prim

96 EC BMP6 has paracrine actions on hepatocyte hemojuvelin to regulate hepcidin transcription and maint

97 ne (LOC148738) encoding a protein designated hemojuvelin was cloned.

98 Soluble hemojuvelin was found in human sera at concentrations si

99 Hemojuvelin was localized on the hepatocyte sinusoidal m

100 Localization of the BMP co-receptor hemojuvelin was visualized by immunofluorescence microsc

101 ) family members RGMa, RGMb/Dragon, and RGMc/hemojuvelin were found recently to act as bone morphogen

102 evels of soluble and membrane-bound forms of hemojuvelin, which in turn would influence the amount of