ライフサイエンスコーパス: NPD

1 SM plasma activities in NPD patients (N=19), NPD carriers (N=11), and normal subjects (N=15) were 2.5

2 ere neurodegeneration associated with Type A NPD.

3 el shifts and enhanced conductivity in alpha-NPD:1% Mo(tfd)(3).

4 of a standard hole transport material (alpha-NPD) is demonstrated via measurements of Fermi level shi

5 An NPD model captures the essential factors for the evoluti

6 ssues and fluids from normal individuals and NPD patients.

7 ssues and fluids from normal individuals and NPD patients.

8 tissues and plasma of normal individuals and NPD patients.

9 g and diagnosing pathological narcissism and NPD, in addition to specific traits.

10 plasma and tissues obtained from normal and NPD mice and humans.

11 reover, SpO2 correlated with ENaC-associated NPD positively in patients only, but apparently related

12 of Niemann-Pick disease (i.e. Types A and B NPD).

13 that, unlike the type A form of NPD, type B NPD does not occur frequently within this population.

14 omyelin levels in plasma from several Type B NPD patients also was significantly elevated compared to

15 omyelin levels in plasma from several type B NPD patients also were significantly elevated compared t

16 an effective therapeutic approach for Type B NPD, but is unlikely to prevent the severe neurodegenera

17 consistent with a less severe form of type B NPD, whereas the H421Y and K576N mutations led to an ear

18 Basal NPD was significantly associated with amiloride-resistan

19 nfirmed by the opposite associations between NPD values and altitude, which had a negative regression

20 of maternal normal protein diet (18% casein; NPD) or isocaloric low protein diet (9% casein; LPD) res

21 iency enhances SINV replication, we compared NPD-A fibroblasts (NPAF) to normal human fibroblasts (NH

22 , but apparently related to CFTR-contributed NPD level inversely.

23 The Nuclear Protein Database (NPD) is a curated database that contains information on

24 utant alleles in patients with ASM-deficient NPD and in carriers influences the disease phenotype.

25 ivity and clinical features of ASM-deficient NPD.

26 ication on the presentation of ASM-deficient NPD.

27 y method to detect ASM activity and diagnose NPD using the fluorescent substrate BODIPY C12-SPM and r

28 rth weights compared to normal-protein diet (NPD) offspring.

29 Nasal potential difference (NPD) response patterns and sweat chloride concentrations

30 Nasal potential difference (NPD), a well-established in vivo clinical test for cysti

31 measurement and neutron powder diffraction (NPD) experiment.

32 e show that the n-player prisoner's dilemma (NPD) is also central to two other prominent theories of

33 Type A and B forms of Niemann-Pick disease (NPD) are lipid storage disorders due to the deficient ac

34 type A and B forms of Niemann-Pick disease (NPD) are lipid storage disorders due to the deficient ac

35 Types A and B Niemann-Pick disease (NPD) are lysosomal storage disorders resulting from loss

36 Types A and B Niemann-Pick disease (NPD) are lysosomal storage disorders resulting from the

37 Niemann-Pick disease (NPD) is a lysosomal storage disease caused by the loss o

38 Niemann-Pick disease (NPD) is caused by the loss of acid sphingomyelinase (ASM

39 Types A and B Niemann-Pick disease (NPD) result from the deficient activity of the lysosomal

40 somal storage disorder Niemann-Pick disease (NPD).

41 cient in types A and B Niemann-Pick disease (NPD).

42 sults in Types A and B Niemann-Pick disease (NPD).

43 4 patients with type B Niemann-Pick disease (NPD).

44 Type A and B forms of Niemann-Pick disease (NPD).

45 storage disease type A Niemann-Pick disease (NPD-A), mimicked in mice by interruption of the ASMase g

46 posal for narcissistic personality disorder (NPD), and highlight some of the advantages of introducin

47 ic hypoxia is characterized by dysfunctional NPD.

48 re and nitrogen phosphorus detection (GC-ECD/NPD) has been developed and validated for the screening

49 ese findings have important implications for NPD enzyme replacement therapy, particularly in the lung

50 e Tc-Tc bond was found to be 2.622(1) A from NPD profile analysis and 2.59 A from first-principles DF

51 and tissues from normal individuals and from NPD mice and patients.

52 it was refined in the C2/m space group from NPD data collected at room temperature.

53 , the mean ASM activity in human plasma from NPD carriers (258.3 pmol/ml/h) also was significantly re

54 The mean ASM activity in human plasma from NPD patients (36 pmol/ml/h) was only 2.7% of that in nor

55 ingomyelin in plasma, urine, or tissues from NPD patients and mice.

56 ed gas chromatographic method (GC-ECD and GC-NPD) was used to determine the fungicides residues, the

57 matography-nitrogen phosphorus detection (GC-NPD).

58 content in adult homozygous or heterozygous NPD mouse plasma and urine was significantly elevated co

59 adult homozygous (-/-) or heterozygous (+/-) NPD mouse plasma was significantly elevated compared to

60 The ranges of ASM plasma activities in NPD patients (N=19), NPD carriers (N=11), and normal sub

61 for similar studies in sexually mature, male NPD patients.

62 Because ASMase-knock-out mice models NPD and our previous findings revealed that ASMase activ

63 ing the ASM-deficient mouse model (ASMKO) of NPD, we evaluated the efficacy of enzyme replacement the

64 Purkinje cell loss that is characteristic of NPD, although the protective effect declined with distan

65 could be a useful tool for the diagnosis of NPD and the evaluation of NPD treatment protocols, as we

66 simple source for the accurate diagnosis of NPD patients and carriers based on ASM activity.

67 r the diagnosis of NPD and the evaluation of NPD treatment protocols, as well as for the study of cer

68 h, revealing that, unlike the type A form of NPD, type B NPD does not occur frequently within this po

69 We have used a knockout mouse model of NPD (ASMKO mice) to evaluate the effects of direct intra

70 issue and sperm in a knockout mouse model of NPD and demonstrate the importance of ASM for normal spe

71 ncoding human ASM (hASM) in a mouse model of NPD.

72 ession of CtsB may underlie the phenotype of NPD.

73 accumulated sphingomyelin in the tissues of NPD mice was 4 to 15 times higher than that in normal mi

74 accumulated sphingomyelin in the tissues of NPD mice was 4 to 40 times higher than that in normal mi

75 Treatment of NPD cell lines with 5-aza-2'-deoxycytidine enhanced the

76 ass spectrometry (MS), nitrogen phosphorous (NPD), flame photometric (FPD) detectors, as well as gas

77 After mating, all females received NPD for the remainder of gestation and all offspring wer

78 ns or alterations in the amiloride-sensitive NPD.

79 The NPD analysis also establishes a new neutron scattering l

80 The NPD results supported by the DFT calculations suggest th

81 nd demonstrate the potential of altering the NPD phenotype by these therapeutic strategies.

82 The results of the NPD along with the saturation magnetic moment (MS) and m

83 tatistically significant improvements of the NPD response to perfusion of an isoproterenol/amiloride/

84 An integrative approach based on the NPD helps unify different perspectives on the evolution

85 oietic stem cell gene therapy (HSCGT) on the NPD phenotype.

86 ure determined by Rietveld analysis with the NPD data is of a distorted rutile type, similar to that

87 The "NPD ratio," widely used by yeast geneticists, is of limi

88 Thus, NPD provides a gateway through which the nuclear proteom

89 The new diagnostic approach to NPD can encourage a better integration of the clinicians

90 promising therapeutic modality for treating NPD and suggest a potential strategy for treating diseas

91 To analyze whether NPD can be applied to diagnose hypoxic lung injury, we s

92 from studies using cells from patients with NPD or from ASM knockout (ASMKO) mice, where the genetic

93 eaction sequencing for several patients with NPD revealed preferential expression of one mutant allel

94 treatment of liver fibrosis in patients with NPD.

95 ave specifically highlighted how people with NPD behave and are observed by others, and the negative