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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 owth differentiation factor-11/8], and RGMC [hemojuvelin]).
11 support a key role for BMP6 as a ligand for hemojuvelin and an endogenous regulator of hepcidin expr
13 we report expression of soluble versions of hemojuvelin and neogenin for biochemical characterizatio
14 est that MT2 suppresses hepcidin by cleaving hemojuvelin and other components of the bone morphogenet
16 s of hepcidin mRNA, it did not interact with hemojuvelin, and interference with its signaling pathway
17 y of a new gene involved in iron metabolism, hemojuvelin, and new data on the role of HFE mutations i
18 endent pathway requires the presence of Hfe, hemojuvelin, and probably transferrin receptor 2 (tfr-2)
20 y known iron metabolic proteins such as HFE, hemojuvelin, and transferrin receptor 2 are expanding th
23 hanism of this process is not clear, several hemojuvelin-binding proteins, including the cell surface
25 coordination of multiple proteins, including hemojuvelin, bone morphogenetic protein 6 (BMP6), heredi
27 which HFE2 mutations cause hemochromatosis: hemojuvelin dysfunction decreases BMP signaling, thereby
28 alytic cleavage, and as in vivo, recombinant hemojuvelin exists as a mixture of cleaved and uncleaved
29 ding HFE, transferrin receptor 2 (TfR2), and hemojuvelin, function at least in part, by modulating th
30 s caused by mutations in the recently cloned hemojuvelin gene (HJV; also called HFE2), whereas Type 2
32 ee hemochromatosis-associated proteins: HJV (hemojuvelin), HFE (hemochromatosis protein), and TfR2 (t
33 BMP2/4 and hemochromatosis-related proteins hemojuvelin, HFE and transferrin receptor 2, also regula
40 ic proteins (BMPs), apparently by binding to hemojuvelin (Hjv) as a coreceptor and signaling through
43 netic protein 6 (BMP6) or the BMP coreceptor hemojuvelin (HJV) in mice leads to a similar phenotype w
50 ontrast, mice deficient for both Tmprss6 and hemojuvelin (Hjv), a BMP coreceptor that augments hepcid
52 to regulate hepcidin production by cleaving Hemojuvelin (Hjv), a key modulator of hepcidin expressio
55 rrence of compound heterozygous mutations in hemojuvelin (HJV), including a termination codon, in a p
58 in (HAMP), homeostatic iron regulator (HFE), hemojuvelin (HJV), nuclear factor erythroid 2-related fa
59 tions in HFE, transferrin receptor 2 (Tfr2), hemojuvelin (HJV), or bone morphogenetic protein 6 (BMP6
60 sis type 2 (HFE2), which encodes the protein hemojuvelin (HJV), result in the absence of hepcidin and
70 idin expression were not affected by loss of hemojuvelin in skeletal muscle regardless of dietary iro
79 ave any known function outside of the liver, hemojuvelin is expressed at very high levels in cardiac
82 inhibitors pacritinib and momelotinib, anti-hemojuvelin monoclonal antibody DISC-0974C) are currentl
83 we used hemojuvelin-specific siRNAs to vary hemojuvelin mRNA concentration and showed that cellular
84 hat hemojuvelin is a BMP coreceptor and that hemojuvelin mutants associated with hemochromatosis have
85 mochromatosis is associated with hepcidin or hemojuvelin mutations, and these patients have low or ab
87 rarer instances, transferrin-receptor 2 and hemojuvelin, or make its receptor ferroportin, resistant
88 mRNA concentration and showed that cellular hemojuvelin positively regulated hepcidin mRNA expressio
89 te with low hepcidin levels, suggesting that hemojuvelin positively regulates hepcidin expression.
90 at BMP-2 and neogenin bind simultaneously to hemojuvelin raises the possibility that neogenin is part
91 We propose that soluble and cell-associated hemojuvelin reciprocally regulate hepcidin expression in
92 lthough our previous studies have shown that hemojuvelin regulates hepcidin expression and iron metab
93 s engineered to express hemojuvelin, soluble hemojuvelin release was progressively inhibited by incre
100 present study, we show in mice that loss of hemojuvelin specifically in the liver leads to decreased
101 wed that recombinant soluble hemojuvelin (rs-hemojuvelin) suppressed hepcidin mRNA expression in prim
102 EC BMP6 has paracrine actions on hepatocyte hemojuvelin to regulate hepcidin transcription and maint
107 ) family members RGMa, RGMb/Dragon, and RGMc/hemojuvelin were found recently to act as bone morphogen
108 evels of soluble and membrane-bound forms of hemojuvelin, which in turn would influence the amount of