ライフサイエンスコーパス: myopathic

1 implied that this condition is predominantly myopathic.

2 were classified as neurogenic (8 patients), myopathic (10 patients), "other" (12 patients), or unkno

3 Myopathic alterations in soleus were apparent by 12 mo,

4 ur data indicate that T32KI mice have both a myopathic and a neurogenic phenotype, very similar to th

5 We also describe potential overlap between myopathic and neurogenic findings in this family.

6 ts degradation, and results in the same mild myopathic and neurogenic phenotype as that found in Trim

7 ess characterized by the early occurrence of myopathic and neurogenic skeletal muscle pathology and b

8 Echo-guided repair rates for degenerative, myopathic, and inflammatory diseases were 99.0%, 97.1%,

9 lated Langendorff-perfused hearts from these myopathic animals showed a 73% decrease in baseline isov

10 ts in concurrence with an abnormally spliced myopathic cardiac TnT (cTnT).

11 ise in animal models of heart failure and in myopathic cardiomyocytes derived from patients.

12 Myopathic carnitine deficiency could be due to an increa

13 ions of mitochondrial DNA in the later-onset myopathic cases, were identified in all affected individ

14 tudy MR properties attributed to healthy and myopathic cells.

15 ber atrophy, regeneration, acute and chronic myopathic change, and stromal changes.

16 Extensive active myopathic changes (excluding regeneration) and central n

17 D1L2 in transgenic mice reproduced the ostes myopathic changes and, indeed, caused severe muscle atro

18 hy with loss of thick filaments in 79%, mild myopathic changes in 14%, and atrophy of type 1 and type

19 EMG revealed predominantly myopathic changes in the axial and periscapular muscles.

20 Myopathic changes include pale M-lines devoid of MURF-1,

21 plasmic inclusions in combination with focal myopathic changes may be a disease-specific morphologica

22 ous mice (ostes/+, Tg(Pkd1l2)/0) suffer from myopathic changes more profound than each heterozygote,

23 icant fat replacement of muscles on imaging, myopathic changes on muscle biopsy and loss of calpain 3

24 rs of muscle weakness and with dystrophic or myopathic changes present in muscle biopsy specimens.

25 ction, but statin therapy is associated with myopathic changes through a poorly defined mechanism.

26 myopathies were found in 5 (9%), nonspecific myopathic changes were present in 11 (20%), and a neurog

27 In addition, myopathic changes were present on muscle MRI.

28 ) of the 24-month-old transgenic mice showed myopathic changes, and approximately one-third of them h

29 Cav-3 (-/-) knock-out mice show a number of myopathic changes, consistent with a mild-to-moderate mu

30 t age 9 months revealed minimal, nonspecific myopathic changes, leading to a diagnosis of "minimal ch

31 Muscle biopsies showed myopathic changes, whereas immunohistochemistry showed n

32 muscle biopsy specimens revealed nonspecific myopathic changes.

33 ysis of T32KO skeletal muscles revealed mild myopathic changes.

34 ransmission only in the affected muscles and myopathic changes.

35 s muscle was reported to exhibit nonspecific myopathic changes.

36 these caveolin-3 null mice reveals: (i) mild myopathic changes; (ii) an exclusion of the dystrophin-g

37 Half of the muscle biopsies (6/12) showed myopathic changes; increased connective tissue elements

38 they have been associated with a variety of myopathic complaints.

39 iency of cathepsin B could contribute to the myopathic component of this illness.

40 These data define an important myopathic contribution to the Kennedy disease phenotype

41 and RNA polyA+ pools relative to normal and myopathic control muscles (P < 0.002), measured relative

42 phy patients, four normal controls, and four myopathic controls).

43 ot in non-myopathic muscle or non-vacuolated myopathic controls.

44 Mice infected with myopathic coxsackievirus B1 Tucson (CVB1(T)) develop chr

45 ines with transgenic mice overexpressing the myopathic cTnT (exon 7 deletion).

46 dditional determinant of weakness encoded by myopathic CVB1(T).

47 sure of denervation and can usually identify myopathic damage, neurogenic damage, or mixed injury.

48 ic cardiomyopathy [HCM], n = 15) and dilated-myopathic (dilated cardiomyopathy [DCM], n = 37) hearts

49 nd progression towards inherited or acquired myopathic disease.

50 lity, and suggest novel molecular aspects of myopathic disease.

51 However, in severe myopathic diseases such as Duchenne Muscular Dystrophy,

52 ll-based therapies for chronic, debilitating myopathic diseases.

53 nemaline myopathy is a distinct, heritable, myopathic disorder caused by a mutation in TNNT1.

54 These patients mimic myopathic disorders and are likely to be under-diagnosed

55 d introduce a candidate for consideration in myopathic disorders.

56 expressing various mutant genes involved in myopathic disorders.

57 rea, and pathologic fibrosis consistent with myopathic disuse atrophy.

58 ere available, for the medical management of myopathic dysfunction and transplantation in pediatric p

59 thick filaments, could be a useful index of myopathic dysfunction.

60 age of 14 years, revealed chronic and severe myopathic (dystrophic) changes, with normal staining for

61 Importantly, we found the myopathic effects of DUX4 were p53 dependent, as p53 inh

62 In this study, the long-term myopathic effects of ricin-mAb 35 on extraocular muscle

63 lease niacin do not substantially potentiate myopathic effects when given in combination with statins

64 iosis, elevated serum creatine kinase level, myopathic electrodiagnostic changes, brain MRI with cobb

65 Both had myopathic electromyogram with abnormal electrical irrita

66 der, with primary clinical manifestations of myopathic exercise intolerance and a macrocytic siderobl

67 ssing both nNOSmu and nNOSbeta were severely myopathic, exhibiting structural defects in the microtub

68 Some patients have myopathic facies.

69 AR113Q limb muscles show similar myopathic features and express decreased levels of mRNAs

70 Muscle biopsies showed myopathic features including fibre size variability, pre

71 nital myasthenic syndrome had more prominent myopathic features that were detectable on muscle biopsi

72 observed in Gne(M712T/M712T) muscle, but no myopathic features were apparent.

73 ndrome may be complicated by the presence of myopathic features, but correct diagnosis is important b

74 ellular collagen VI microfibrils and exhibit myopathic features, including decreased muscle mass and

75 shared with the dystroglycanopathies include myopathic features, raised CK levels and variable mild c

76 SBMA patients exhibit myopathic features, suggesting a role for muscle in dise

77 se (T32KO) that displays both neurogenic and myopathic features.

78 transgenic littermates and exhibited typical myopathic features.

79 hat these clinical abnormalities result from myopathic fibrosis of the extraocular muscles.

80 cle biopsy were concordant for neurogenic or myopathic findings, they were more accurate than either

81 previously been associated with an isolated myopathic form of MDS (OMIM 609560).

82 Locus for the myopathic form of SP syndrome (scapuloperoneal muscular

83 20 cM telomeric to the one described for the myopathic form of SP syndrome.

84 Both neurogenic and myopathic forms of autosomally dominantly inherited scap

85 erlies a familial syndrome of electrical and myopathic heart disease.

86 to regenerate myocardium in the ischemic or myopathic heart.

87 ed impulse propagation and arrhythmia in the myopathic heart.

88 Isolated myocytes from myopathic hearts exhibited increased volume, length, wid

89 The altered intracellular Ca handling of the myopathic hearts prolongs the decay of the L-type Ca cur

90 The myopathic hearts show an increased recycling of oxidized

91 anodine receptors at the Z-lines, whereas in myopathic hearts the degree of co-localization was marke

92 H7 loci and is repressed in various types of myopathic hearts, suggesting a conserved lncRNA mechanis

93 tance catheter in 87 patients with normal or myopathic hearts.

94 effects of mineralocorticoid antagonists in myopathic hearts.

95 e of this study is to measure abdominal wall myopathic histologic and mechanical changes during incis

96 Enhanced myopathic injury and mortality across different etiologi

97 ic Drosophila melanogaster muscle expressing myopathic lamin mutations deformed more under applied st

98 cerbated limb tissue necrosis, increased the myopathic lesion size, reduced muscle regeneration, atte

99 muscle mass and ameliorated muscle injury in myopathic mouse models.

100 es of strength and life span in the severely myopathic Mtm1delta4 mouse model of X-linked myotubular

101 M), dermatomyositis (DM), dystrophic and non-myopathic muscle as controls, and cultured human myotube

102 bers of sIBM muscle biopsies, but not in non-myopathic muscle or non-vacuolated myopathic controls.

103 in the damage and dysfunction that occur in myopathic muscle tissue.

104 Free Mg(2+) levels were normal in DM and JDM myopathic muscles at rest, but were significantly lower

105 Mg-ATP levels in DM and JDM myopathic muscles were at least 37% lower than those in

106 the visceral organs is heterogeneous with a myopathic origin.

107 Evidence for managing myopathic patients with coenzyme Q10 is not conclusive.

108 Electro myography revealed a myopathic pattern associated with abnormal electrical ir

109 im32-/- mice) and showed that they display a myopathic phenotype accompanied by neurogenic features.

110 We show that Pabpn1+/A17 mice have a mild myopathic phenotype in adult and aged animals.

111 The myopathic phenotype of these mutant mice resembled that

112 r muscle development in zebrafish, causing a myopathic phenotype when knocked down.

113 were associated with a milder predominantly myopathic phenotype with a later mean age of disease ons

114 at lack dystrophin and exhibit only a subtle myopathic phenotype.

115 om the absence of TRIM32, which underlie the myopathic phenotype.

116 Our work demonstrates the myopathic potential of DUX4 in animal muscle.

117 study, our objective was to test the in vivo myopathic potential of DUX4.

118 ultimately increase expression of genes with myopathic potential.

119 induced cardiomyopathy demonstrates that the myopathic process associated with rapid heart rates is l

120 Electrophysiology suggested a myopathic process, without myotonia.

121 ral studies supported a drug-induced primary myopathic process.

122 ay of hemodynamic, ventilatory, and skeletal myopathic processes in this common, chronic condition.

123 l myopathy (FVM) is a rare inherited form of myopathic pseudo-obstruction; little is known about the

124 uated surgical approach for degenerative and myopathic repairs by quantifying segmental prolapse, ant

125 both slow and fast isoforms was increased in myopathic skeletal muscles.

126 Both neurogenic and myopathic SP syndromes have been described.

127 to disease pathogenesis in various inherited myopathic states in vivo remains unknown.

128 Clinical response of enteropathic and myopathic symptoms and serum albumin level.

129 ta on the effect of CoQ10 supplementation on myopathic symptoms are scarce and contradictory.

130 Enteropathic and myopathic symptoms resolved after infliximab therapy, an

131 rame internal deletions show relatively mild myopathic symptoms, this may in the future offer a thera

132 in the parents may account for their lack of myopathic symptoms.

133 tRNA genes of mtDNA can cause predominantly myopathic syndromes and - contrary to conventional wisdo

134 These diseases can cause a variety of myopathic syndromes and can become clinically manifest a

135 tochondrial DNA can also cause predominantly myopathic syndromes and--contrary to conventional wisdom

136 Histopathology ranged from mild myopathic to severe dystrophic changes including vacuole

137 to sustain functional effects as that in the myopathic turkey cTnT.

138 determinants that were strictly conserved in myopathic viruses with one located in the 5' untranslate

139 A muscle biopsy specimen was myopathic with a lack of myophosphorylase and multiple l