ライフサイエンスコーパス: cystic disease

1 ysema and cystic bronchiectasis can simulate cystic disease.

2 ptive immune cells during injury-accelerated cystic disease.

3 d found no evidence of systemic infective or cystic disease.

4 renal cyst formation in two mouse models of cystic disease.

5 treatment did not significantly reduce renal cystic disease.

6 nt removal of Tulp3 surprisingly ameliorates cystic disease.

7 x1Cre(+) allele causes a late onset of focal cystic disease.

8 ney and that acute kidney injury exacerbates cystic disease.

9 e cell cycle and proliferation, resulting in cystic disease.

10 is associated with significantly more severe cystic disease.

11 patients may be because of extensive hepatic cystic disease.

12 d patients with tuberous sclerosis and renal cystic disease.

13 ate of PAH secretion despite the presence of cystic disease.

14 romising strategy for the treatment of renal cystic diseases.

15 a contributing factor to the homeostasis in cystic diseases.

16 d progression in models orthologous to human cystic diseases.

17 have been implicated in the pathogenesis of cystic diseases.

18 out contiguous deletions had relatively mild cystic disease, 3 of whom had gross rearrangements of TS

19 rticoid metabolism result in recessive renal cystic disease, a developmental disorder of the kidney.

20 Mosaicism and mild cystic disease also were demonstrated in parents of 3 of

21 rare, some patients develop massive hepatic cystic disease and become clinically symptomatic.

22 the administration of GME did not lessen the cystic disease and did not reverse the effects of BSO.

23 during embryogenesis develop profound renal cystic disease and die from renal failure within 3 weeks

24 1(-/-) or Pkd2(-/-) mice develop rapid renal cystic disease and exhibit embryonic lethality; this sup

25 ic knockdown of ILK strikingly reduced renal cystic disease and fibrosis and extended the life of pcy

26 that loss of Ift140 causes pronounced renal cystic disease and suggest that abnormalities in several

27 l polarity underlie TSC and ADPKD-associated cystic disease and targeting of this pathway may be of k

28 pk/cpk pups expressed both the typical renal cystic disease and the DPM.

29 Renal cystic diseases are a leading cause of renal failure.

30 chanisms that link cilia function with renal cystic diseases are not well understood, and the mechani

31 e, but more aggressive, renal and pancreatic cystic disease as del34/del34.

32 undertaken to further characterize the renal cystic disease as quantitative trait in this F2 cohort a

33 Cystic disease as the underlying cause of renal failure

34 mpanied by a marked aggravation of the renal cystic disease, as reflected by kidney weights, histolog

35 aneous deletion of Gli2 attenuated the renal cystic disease associated with deletion of Thm1.

36 re associated with significant reductions in cystic disease, BUN and serum creatinine levels.

37 lls significantly reduced injury-accelerated cystic disease but had no effect on cyst growth in non-i

38 ppressor gene syndrome in which severe renal cystic disease can occur.

39 pplies only to patients with typical diffuse cystic disease (class 1).

40 other protein products of genes involved in cystic disease: Cystin, the product of the mouse cpk loc

41 neys results in rapid or slow progression of cystic disease depending on whether the animals are juve

42 cystic dysplasia), or manifest as bilateral cystic disease (eg, autosomal recessive polycystic kidne

43 ) is a lethal disorder associated with renal cystic disease, encephalocele, ductal plate malformation

44 Cystic diseases, especially autosomal dominant polycysti

45 marked variability was observed in the renal cystic disease expressed in F2 bpk/bpk homozygotes of a

46 Gross cystic disease fluid protein (GCDFP-15), also known as p

47 The presence of gross cystic disease fluid protein 15 is also suggestive of me

48 for epithelial membrane antigen (EMA), gross cystic disease fluid protein-15 (GCDFP-15), cytokeratin

49 lacement or replacement of cytoplasmic gross cystic disease fluid protein-15 and CK 7-positive tonofi

50 rotein-3 (Tulp3) as a key regulator of renal cystic disease from a forward genetic screen in the mous

51 These studies identify a link between two cystic disease genes, HNF1beta (MODY5) and PKHD1 (ARPKD)

52 hat HNF-1beta regulates the transcription of cystic disease genes, including Pkd2 and Pkhd1.

53 polarity and decreased expression of several cystic disease genes, some of which we identified as nov

54 1beta regulates the transcription of several cystic disease genes.

55 Children with severe renal cystic disease (> 10 cysts; n = 54) had greater protein

56 Recently, renal cystic diseases have been associated with dysfunctional

57 le proteins whose functions are disrupted in cystic diseases have now been localized to the cilium or

58 ic approaches to slow the development of the cystic disease; however, little is known about the role

59 n the major loci responsible for human renal cystic disease in a common PKD pathway.

60 and XBP1 overexpression in vivo ameliorated cystic disease in a murine model with reduced PC1 functi

61 RAS inhibitors in slowing the progression of cystic disease in ADPKD are inconclusive, and we hypothe

62 ropic phenotype with hydrocephalus and renal cystic disease in both humans and rodent models.

63 By one-month cystic disease in both the BC and 129 Pkd1(RC/RC) mice w

64 VEGFC administration also improved cystic disease in Cys1(cpk/cpk) mice, a model of autosom

65 etween the severity of the DPM and the renal cystic disease in either F(2) cohort.

66 Renal cystic disease in homo- and heterozygotes of a Pkd2 mous

67 disease (ARPKD) is a common hereditary renal cystic disease in infants and children.

68 vitine does indeed yield effective arrest of cystic disease in jck and cpk mouse models of PKD.

69 d, although more than half developed hepatic cystic disease in later life, similar to the phenotype o

70 reatment with a proteasome inhibitor reduces cystic disease in orthologous gene models of human autos

71 cyst formation in normal mice and accelerate cystic disease in PKD mice.

72 deficiency in FPC increases the severity of cystic disease in Pkd2 mutants and down-regulates PC2 in

73 utation of the Cys1 gene underlies the renal cystic disease in the Cys1(cpk/cpk) (cpk) mouse that phe

74 In addition, they cause an endothelial-lined cystic disease in the liver known as bacillary peliosis.

75 The severity of renal cystic disease in the major form of autosomal dominant p

76 e we show that renal injury leads to massive cystic disease in the same mouse line.

77 Cystic disease in trans-heterozygous Pkd1(+/-) : Pkd2 (+

78 Significant renal cystic disease in tuberous sclerosis usually reflects mu

79 ing a role for PKD1 in the etiology of renal cystic disease in tuberous sclerosis.

80 ml, respectively) on mTOR activity and renal cystic disease in two Pkd1-mutant mouse models at differ

81 The pathology of pancreatic cystic disease in VHL patients has not been well charact

82 of RIalpha upregulates PKA activity, induces cystic disease in wild-type mice, and aggravates it in P

83 e is one of the most common hereditary renal cystic diseases in children.

84 s an excellent candidate for as yet unmapped cystic diseases in man and animals.

85 tants offer a new vertebrate model for renal cystic diseases, in which cilia morphology can be analyz

86 Many renal cystic diseases, including autosomal dominant polycystic

87 The renal cystic disease is fully expressed in homozygotes and is

88 least in some patients, the severity of the cystic disease is inversely correlated with the level of

89 ing approaches, earlier diagnosis of hepatic cystic disease is possible, and measurement of kidney an

90 o therapies are currently available to treat cystic diseases, making it imperative to dissect molecul

91 Recent studies suggest that the cystic disease might result from defects in planar cell

92 t Xpl mutant, in which polydactyly and renal cystic disease occurs, maps to the homologous region of

93 type of PCK AVP(-/-) rats and aggravated the cystic disease of PCK AVP(+/+) rats but did not induce c

94 renin-angiotensin-aldosterone signaling, and cystic diseases of the kidney illustrated the applicatio

95 have intermediate survival in the absence of cystic disease or renal failure, providing the first ind

96 concluded that the severity of the bpk renal cystic disease phenotype is modulated by multiple loci a

97 In kat/kat mice, the renal cystic disease progresses more slowly but is morphologic

98 dk5 in the jck mice significantly attenuates cystic disease progression and is associated with shorte

99 Mutations associated with renal cystic diseases reside in genes encoding proteins that l

100 a-analysis of published animal-model data of cystic disease reveals mRNA upregulation of several comp

101 stal Chr 6, near D6Mit14, that affects renal cystic disease severity.

102 this drug leads to amelioration of the renal cystic disease similar to genetic STAT6 inactivation.

103 zygotes; these mutants developed less severe cystic disease than jck homozygotes and provided genetic

104 l depletion are molecular features of kidney cystic disease that may contribute to its pathogenesis.

105 dered were the extent and pattern of hepatic cystic disease, the degree of hepatic and renal dysfunct

106 ild and maintain the cilium also cause renal cystic disease through unknown pathways.

107 orphogenesis in 3D renal cultures link renal cystic disease to apical organization defects, whereas c

108 one pair of sisters who were discordant for cystic disease, two mother- daughter pairs who were disc

109 were somatic mosaics; the severity of their cystic disease varied considerably.

110 tion of polycystins and that the severity of cystic disease was directly related to the length of tim

111 After induction of cilia loss, cystic disease was not more pronounced in adult mice wit

112 n 17 patients with constitutional deletions, cystic disease was severe, with early renal insufficienc

113 e the role of the TWEAK signaling pathway in cystic disease, we evaluated Fn14 expression in human an

114 irm that a defect in the Nek8 gene can cause cystic disease, we performed a cross-species analysis: i

115 in adult mice results in slowly progressive cystic disease, which can be greatly accelerated by rena

116 omas, and 22 (88%) had no or mild pancreatic cystic disease, which is substantially more than the gen

117 t88 gene leads to delayed, adult-onset renal cystic disease, which provides a window of opportunity t

118 tion and the development of severe postnatal cystic disease, which resulted in premature death.