ライフサイエンスコーパス: progeroid

1 rtic vascular smooth muscle cells from these progeroid animals had an impaired capacity to inhibit va

2 patients who had severe MAD associated with progeroid appearance and generalized lipodystrophy.

3 poatrophy and cutis laxa are the basis for a progeroid appearance.

4 We also found that these progeroid cells exhibited nuclear foci of xeroderma pigm

5 camptothecin were efficiently removed in the progeroid cells.

6 A foci colocalized with the DSB sites in the progeroid cells.

7 ients with Werner syndrome (WS), a segmental progeroid condition with a high incidence of cancer, and

8 expression and alleviates the severity of a progeroid disease by increasing RNR activity.

9 reveal abnormal TP63 RT as an early event in progeroid disease progression, and suggest TP63 gene reg

10 ly of DNA helicases, have been linked to the progeroid disease Rothmund-Thomson Syndrome.

11 min A accumulates in cells, causing a severe progeroid disease, restrictive dermopathy (RD).

12 gy of a protein at the center of devastating progeroid diseases.

13 a osteodysplastica is a hereditary segmental progeroid disorder affecting skin, connective tissues, a

14 Werner syndrome (WS) is a rare progeroid disorder characterized by genomic instability,

15 Werner Syndrome (WS) is a human progeroid disorder characterized by genomic instability.

16 tations instead cause the neurodevelopmental progeroid disorder Cockayne syndrome, but little is know

17 fective in the autosomal recessive segmental progeroid disorder Cockayne syndrome.

18 ow that mouse models of Cockayne syndrome, a progeroid disorder resulting from a defect in the transc

19 helicase WRN protein causes the cancer-prone progeroid disorder Werner syndrome (WS).

20 The segmental, progeroid disorder Werner syndrome results from loss of

21 A more severe progeroid disorder, restrictive dermopathy (RD), is caus

22 syndrome (WS) is a rare autosomal recessive progeroid disorder.

23 athway mutations cause neurodegenerative and progeroid disorders (xeroderma pigmentosum (XP), Cockayn

24 Several progeroid disorders are caused by mutations that lead to

25 Progeroid disorders overlapping with De Barsy syndrome (

26 stematically compared liver from 5-month-old progeroid Ercc1(-/Delta) mice to old (24-36-month-old) w

27 Two of the girls had pronounced neonatal progeroid features and were initially diagnosed with Wie

28 ncommon autosomal recessive disease in which progeroid features are associated with genetic instabili

29 mice with reduced life span and accelerated progeroid features are indistinguishable from age-matche

30 ycle is perturbed and this might explain the progeroid features of the POLG mutator mouse.

31 ogressive aneuploidy along with a variety of progeroid features, including short lifespan, cachectic

32 n autosomal-dominant form of cutis laxa with progeroid features.

33 n inherited neurodevelopmental disorder with progeroid features.

34 e dietary-restriction-like therapy for human progeroid genome instability syndromes and possibly neur

35 nd genetic clearance of senescent cells from progeroid INK-ATTAC mice prevents lipodystrophy.

36 uffer from partial embryonic lethality and a progeroid-like phenotype.

37 Werner syndrome (WS) is a progeroid-like syndrome caused by WRN gene mutations.

38 The Lmna null (Lmna(-/-)) and progeroid LmnaDelta9 mutant mice are models for AD-EDMD

39 As both WT and progeroid mice aged, NF-kappaB was activated stochastica

40 Zmpste24(-/-) progeroid mice also developed severe fibrosis-unrelated

41 Here we show that MDSPCs from old and progeroid mice are defective in proliferation and multil

42 SPCs, isolated from young wild-type mice, to progeroid mice confer significant lifespan and healthspa

43 The similarities between HGPS patients and progeroid mice reported here strongly suggest that defec

44 ian and maximal remaining lifespans of these progeroid mice, strongly retarding numerous aspects of a

45 90 inhibitor reduces age-related symptoms in progeroid mice.

46 hosphate inhibited vascular calcification in progeroid mice.

47 the age-related symptoms and pathologies of progeroid mice.

48 asured the activation of NF-kappaB in WT and progeroid model mice.

49 Here we show that in the BubR1 progeroid mouse background, INK-ATTAC removes p16(Ink4a)

50 Clearance of SCs in a progeroid mouse model using a transgenic approach delays

51 Moreover, the RT abnormalities in progeroid patients were associated with altered isoform

52 f mitochondrial DNA (mtDNA) deletions in the progeroid phenotype of exonuclease-deficient DNA polymer

53 ncreased mtDNA mutation levels contribute to progeroid phenotypes came from the mtDNA mutator mouse.

54 egulation of proteases potentially linked to progeroid phenotypes in CS, and our results suggest resc

55 thologies induced by DSBs, and the segmental progeroid phenotypes in humans and mice with genetic def

56 ough Sprtn hypomorphic mice recapitulate key progeroid phenotypes of RJALS, whether this model expres

57 vels and possibly link mechanisms leading to progeroid phenotypes to those of cell immortalization.

58 ce results in changes in animal size but not progeroid phenotypes.

59 r syndrome (WS) is the canonical adult human progeroid ('premature aging') syndrome.

60 Our results identified a progeroid-specific RT signature that is common to cells

61 Ercc1(-/Delta) mice model a rare human progeroid syndrome caused by inherited defects in DNA re

62 Werner's syndrome is a progeroid syndrome caused by mutations at the WRN helica

63 ome (WS) is an autosomal recessive segmental progeroid syndrome caused by mutations in a novel member

64 ree patients are affected by a new segmental progeroid syndrome characterized by genomic instability

65 Werner syndrome (WS) is a human progeroid syndrome characterized by the early onset of a

66 tchinson-Gilford progeria syndrome (HGPS), a progeroid syndrome in children, is caused by mutations i

67 yndrome (WS) is a prototypic adult Mendelian progeroid syndrome in which signs of premature aging are

68 XFE progeroid syndrome is a disease of accelerated aging cau

69 lar to that seen in cells from patients with progeroid syndrome resulting from a point mutation in BA

70 Ruijs-Aalfs syndrome is a segmental progeroid syndrome resulting from mutations in the SPRTN

71 Similarly, XFE progeroid syndrome results from defects in the ERCC1-XPF

72 e WRN helicase gene cause Werner syndrome- a progeroid syndrome with an elevated risk of cancer and o

73 ford progeria syndrome (HGPS) is a segmental progeroid syndrome with multiple features suggestive of

74 search on Werner syndrome, a heritable adult progeroid syndrome with prominent dermatologic features,

75 sitive to oxygen and displays hallmarks of a progeroid syndrome, including early-onset mortality and

76 Loss of WRN causes the genomic instability progeroid syndrome, Werner syndrome.

77 of Ercc1 (-/) mice, a mouse model of a human progeroid syndrome, with the HSP90 inhibitor 17-DMAG ext

78 s rare mutations in SPRTN that cause a novel progeroid syndrome.

79 g in Werner and Bloom syndromes, but not XFE progeroid syndrome.

80 A, misshapen nuclei, and a lethal perinatal progeroid syndrome: restrictive dermopathy (RD).

81 deled after A-lamin (LMNA) mutations causing progeroid syndromes (PSs) in humans.

82 obvious cellular pathology in lamin-related progeroid syndromes and suggest a potential strategy for

83 Progeroid syndromes are rare genetic disorders that phen

84 mes might be involved in the pathogenesis of progeroid syndromes caused by defective RecQ helicases.

85 of prelamin A suggested that lamin A-related progeroid syndromes might be treated with impunity by re

86 er syndrome and a surprising number of other progeroid syndromes support the importance of the mainte

87 iety of congenital cancer susceptibility and progeroid syndromes that are caused by defects in genome

88 seases is illustrated by numerous congenital progeroid syndromes that are caused by mutations in geno

89 upon multiple organs and tissues (segmental progeroid syndromes) and those that have their major imp

90 acts upon a single organ or tissue (unimodal progeroid syndromes).

91 Studies of progeroid syndromes, particularly HGPS, the most dramati

92 otent stem cells as a model for the onset of progeroid syndromes, we tracked the progression of RT ab

93 Single-gene mutations can produce human progeroid syndromes--phenotypes that mimic usual or "nor

94 islocalization of XPA in laminopathy-related progeroid syndromes.

95 , cardiomyopathy, partial lipodystrophy, and progeroid syndromes.

96 protein p63 gene (TP63) as a gene marker for progeroid syndromes.

97 , cardiomyopathy, partial lipodystrophy, and progeroid syndromes.

98 elope, cause misshapen nuclei, and result in progeroid syndromes.

99 eir rarity, diseases of premature aging, or "progeroid" syndromes, have provided important insights i

100 ctivity of WRN, which is absent in the human progeroid Werner syndrome, is thought to counteract this

101 Other DNA-repair-deficient, progeroid Xpg(-/-) (also known as Ercc5(-/-)) mice, a mo