ライフサイエンスコーパス: HNF-1beta

1 HNF-1beta and the structurally related HNF-1alpha bind s

2 HNF-1beta binds to the SOCS3 promoter and represses SOCS

3 HNF-1beta binds to two evolutionarily conserved sites lo

4 HNF-1beta directly regulates the transcription of Pkhd1,

5 HNF-1beta is a tissue-specific transcription factor that

6 HNF-1beta mutant cells also expressed lower levels of ch

7 HNF-1beta mutant kidneys showed increased expression of

8 63% HNF-1alpha, 2% HNF-4alpha, 0% IPF-1, 1% HNF-1beta, 0% NeuroD1/ BETA2, and 20% glucokinase.

9 HNF-4alpha, insulin promoter factor (IPF)-1, HNF-1beta, and NeuroD1/BETA2, all resulting in early-ons

10 gated to the coding sequence for HNF-1alpha, HNF-1beta, HNF-3, or HNF-4 completely restored the PKA r

11 ial role of hepatocyte nuclear factor-1beta (HNF-1beta) in regulating PPARGC1A expression in AKI.

12 Hepatocyte nuclear factor-1beta (HNF-1beta) is a homeodomain-containing transcription fac

13 Hepatocyte nuclear factor-1beta (HNF-1beta) is a Pit-1, Oct-1/2, Unc-86 (POU) homeodomain

14 Hepatocyte nuclear factor-1beta (HNF-1beta) is a Pit-1, Oct-1/2, UNC-86 (POU)/homeodomain

15 Hepatocyte nuclear factor-1beta (HNF-1beta) is a transcription factor that regulates gene

16 tion factor hepatocyte nuclear factor-1beta (HNF-1beta) is essential for normal kidney development an

17 tion factor hepatocyte nuclear factor-1beta (HNF-1beta) regulates tissue-specific gene expression in

18 tion factor hepatocyte nuclear factor-1beta (HNF-1beta), mutations of which produce kidney cysts.

19 al similarity between HNF-1alpha and -1beta, HNF-1beta mutation carriers have hyperinsulinemia, where

20 An HNF-1beta deletion mutant lacking the C-terminal domain

21 Expression of an HNF-1beta C-terminal deletion mutant in transgenic mice

22 gene expression profiling, we found that an HNF-1beta target gene in the kidney is kinesin family me

23 and -1alpha, insulin promoter factor-1, and HNF-1beta are the causes of four forms of maturity-onset

24 nal heterodimerization of the HNF-1alpha and HNF-1beta proteins.

25 tions in HNF-1beta develop kidney cysts, and HNF-1beta regulates the transcription of several cystic

26 he proteins encoded by Srebf2 and Hmgcr, and HNF-1beta directly controlled the renal epithelial expre

27 y increasing DRA expression via the RAR-beta/HNF-1beta-dependent pathway.

28 and RBP-J results in down-regulation of both HNF-1beta and Sox9 (sex determining region Y-related HMG

29 s and transcriptional programs controlled by HNF-1beta are poorly understood.

30 eutic approach for human diseases induced by HNF-1beta mutations.

31 -coding genes that are directly regulated by HNF-1beta in murine kidney epithelial cells.

32 RNAs (miRNAs) that are directly regulated by HNF-1beta in renal epithelial cells.

33 This may be mediated through regulation by HNF-1beta of the key gluconeogenic enzymes glucose-6-pho

34 cipitation assays and PCR analysis confirmed HNF-1beta binding to the Ppargc1a promoter in mouse kidn

35 Here, we used Pkhd1/Cre mice to delete HNF-1beta specifically in renal collecting ducts (CDs).

36 NA and miR-200 was decreased in kidneys from HNF-1beta knock-out mice and renal epithelial cells expr

37 revealed downregulated expression of FXR in HNF-1beta mutant kidneys.

38 atment resulted in a significant increase in HNF-1beta mRNA levels.

39 200 targets, Zeb2 and Pkd1, was increased in HNF-1beta knock-out kidneys and in cells expressing muta

40 The expression of SOCS3 is increased in HNF-1beta knockout mice and in renal epithelial cells ex

41 n-regulated and cAMP levels are increased in HNF-1beta mutant kidney cells and mice.

42 te was not suppressed by low-dose insulin in HNF-1beta subjects but was suppressed by 89% in HNF-1alp

43 Humans with mutations in HNF-1beta develop kidney cysts, and HNF-1beta regulates

44 decreased the expression of Zeb2 and Pkd1 in HNF-1beta mutant cells.

45 Functional studies of R137-K161del HNF-1beta revealed that it could not bind an HNF-1 targe

46 Compared with wild-type littermates, HNF-1beta mutant mice exhibited polyuria and polydipsia.

47 k-out kidneys and in cells expressing mutant HNF-1beta.

48 Expression of dominant negative mutant HNF-1beta or kidney-specific inactivation of HNF-1beta d

49 al cells expressing dominant-negative mutant HNF-1beta rescues the defect in HGF-induced tubulogenesi

50 al cells expressing dominant-negative mutant HNF-1beta.

51 al cells expressing dominant-negative mutant HNF-1beta.

52 m mitigated the inhibitory effects of mutant HNF-1beta on the proteins encoded by Srebf2 and Hmgcr, a

53 -1 site or expression of a dominant-negative HNF-1beta mutant inhibit Pkhd1 promoter activity in tran

54 ansgenic mice expressing a dominant-negative HNF-1beta mutant under the control of a kidney-specific

55 This is likely to reflect reduced action of HNF-1beta in the liver and possibly the kidney.

56 of HNF1B leading to a superimposed defect of HNF-1beta transcriptional activity.

57 We conclude that the C-terminal domain of HNF-1beta is required for the activation of the Pkhd1 pr

58 arget genes responsible for the functions of HNF-1beta, however, is incompletely defined.

59 as a previously unrecognized target gene of HNF-1beta in the kidney.

60 HNF-1beta or kidney-specific inactivation of HNF-1beta decreased the expression of genes that are ess

61 Inactivation of HNF-1beta in mouse kidney tubules leads to early-onset c

62 to mice with kidney-specific inactivation of HNF-1beta.

63 Moreover, inhibition of HNF-1beta significantly reduced PPARGC1A expression and

64 IFN-gamma and TNF-alpha led to inhibition of HNF-1beta transcriptional activity.

65 Knockout of HNF-1beta in the mouse kidney results in cyst formation.

66 Mutations of HNF-1beta cause maturity-onset diabetes of the young, ty

67 Humans with autosomal dominant mutations of HNF-1beta develop maturity-onset diabetes of the young t

68 Mutations of HNF-1beta inhibited Kif12 transcription in both cultured

69 Mutations of HNF-1beta lead to a syndrome of inherited renal cysts an

70 Mutations of HNF-1beta produce congenital cystic abnormalities of the

71 fy multiple mechanisms, whereby mutations of HNF-1beta produce defects in urinary concentration.

72 1 expression, which argues that mutations of HNF-1beta produce kidney cysts by down-regulating the AR

73 Mutations of HNF-1beta produce kidney cysts, and previous studies hav

74 expression was inhibited in the presence of HNF-1beta siRNA indicative of its involvement in ATRA-in

75 Here we show that the C-terminal region of HNF-1beta contains an activation domain that is function

76 etion is located in the pseudo-POU region of HNF-1beta, a region implicated in the specificity of DNA

77 These findings reveal a novel role of HNF-1beta in a transcriptional network that regulates in

78 These findings reveal a novel role of HNF-1beta in osmoregulation and identify multiple mechan

79 However, the complete spectrum of HNF-1beta-regulated genes and pathways is not known.

80 00a/429) as novel transcriptional targets of HNF-1beta.

81 Treatment of HNF-1beta mutant mIMCD3 cells with hypertonic NaCl inhib

82 of the homologous transcriptional regulator HNF-1beta rationalize the functional heterodimerization

83 pitation and sequencing experiments revealed HNF-1beta binding to the Nr1h4 promoter in wild-type kid

84 CD-specific HNF-1beta mutant mice survived long term and developed s

85 Pathway analysis predicted that HNF-1beta regulates cholesterol metabolism.

86 Thus, we propose that HNF-1beta links extracellular inflammatory signals to mi

87 the kidney, and previous studies showed that HNF-1beta regulates the expression of the autosomal rece

88 cysts, and previous studies have shown that HNF-1beta regulates the transcription of cystic disease

89 y suggest that heterozygous mutations in the HNF-1beta gene are associated with a syndrome characteri

90 Mutations of the HNF-1beta binding sites abolished promoter activity.

91 The sequence of the HNF-1beta gene ( TCF2 ) revealed a 75 bp deletion in exo

92 which overlapped with downregulation of the HNF-1beta transcriptional network.

93 Luciferase reporter assays showed that the HNF-1beta binding sites were located within a promoter t

94 Thus, HNF-1beta regulates tubulogenesis by controlling the lev

95 ctivation of the Pkhd1 promoter by wild-type HNF-1beta is stimulated by sodium butyrate or coactivato

96 e C-terminal domain interacts with wild-type HNF-1beta, binds DNA, and functions as a dominant-negati

97 These studies reveal a novel pathway whereby HNF-1beta directly contributes to the control of miRNAs

98 We examined whether HNF-1beta mutation carriers are insulin resistant.

99 esent in complexes throughout the day, while HNF-1beta binding exhibited circadian periodicity.

100 nal clinical features may be associated with HNF-1beta mutations.

101 Subjects with HNF-1beta mutations have reduced insulin sensitivity of

102 with HNF-1alpha mutations, six subjects with HNF-1beta mutations, and six control subjects, matched f

103 Subjects with HNF-1beta mutations, in contrast to the other transcript

104 s (42.7 years) than HNF-1alpha (20.4 years), HNF-1beta (24.2 years), or HNF-4alpha (26.3 years) gene