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1 implied that this condition is predominantly myopathic.
2  were classified as neurogenic (8 patients), myopathic (10 patients), "other" (12 patients), or unkno
3 ermore, transgenic expression of a subset of myopathic alleles (R394H, D487N, and 520fs) induce myofi
4                                              Myopathic alterations in soleus were apparent by 12 mo,
5 yering defects, progressive neuron loss, and myopathic alterations.
6 ur data indicate that T32KI mice have both a myopathic and a neurogenic phenotype, very similar to th
7   We also describe potential overlap between myopathic and neurogenic findings in this family.
8 ts degradation, and results in the same mild myopathic and neurogenic phenotype as that found in Trim
9 ess characterized by the early occurrence of myopathic and neurogenic skeletal muscle pathology and b
10   Echo-guided repair rates for degenerative, myopathic, and inflammatory diseases were 99.0%, 97.1%,
11 lated Langendorff-perfused hearts from these myopathic animals showed a 73% decrease in baseline isov
12 tial medical therapy for obstruction-induced myopathic bladder dysfunction (from benign prostatic hyp
13 ts in concurrence with an abnormally spliced myopathic cardiac TnT (cTnT).
14 ise in animal models of heart failure and in myopathic cardiomyocytes derived from patients.
15                                              Myopathic carnitine deficiency could be due to an increa
16 ions of mitochondrial DNA in the later-onset myopathic cases, were identified in all affected individ
17 tudy MR properties attributed to healthy and myopathic cells.
18 ber atrophy, regeneration, acute and chronic myopathic change, and stromal changes.
19                             Extensive active myopathic changes (excluding regeneration) and central n
20 D1L2 in transgenic mice reproduced the ostes myopathic changes and, indeed, caused severe muscle atro
21                                     Ischemic myopathic changes associated with inflammatory infiltrat
22 hy with loss of thick filaments in 79%, mild myopathic changes in 14%, and atrophy of type 1 and type
23                   EMG revealed predominantly myopathic changes in the axial and periscapular muscles.
24                                              Myopathic changes include pale M-lines devoid of MURF-1,
25 plasmic inclusions in combination with focal myopathic changes may be a disease-specific morphologica
26          We speculate that a few findings of myopathic changes might be secondary to chronic denervat
27 ous mice (ostes/+, Tg(Pkd1l2)/0) suffer from myopathic changes more profound than each heterozygote,
28 icant fat replacement of muscles on imaging, myopathic changes on muscle biopsy and loss of calpain 3
29 rs of muscle weakness and with dystrophic or myopathic changes present in muscle biopsy specimens.
30 ction, but statin therapy is associated with myopathic changes through a poorly defined mechanism.
31 myopathies were found in 5 (9%), nonspecific myopathic changes were present in 11 (20%), and a neurog
32                                 In addition, myopathic changes were present on muscle MRI.
33                  Muscle biopsy revealed mild myopathic changes with fibre type uniformity, core-like
34 ldly elevated, and muscle biopsy showed mild myopathic changes with selective type II atrophy.
35 ) of the 24-month-old transgenic mice showed myopathic changes, and approximately one-third of them h
36  Cav-3 (-/-) knock-out mice show a number of myopathic changes, consistent with a mild-to-moderate mu
37 t age 9 months revealed minimal, nonspecific myopathic changes, leading to a diagnosis of "minimal ch
38                       Muscle biopsies showed myopathic changes, whereas immunohistochemistry showed n
39 ransmission only in the affected muscles and myopathic changes.
40 month to 3 years revealed mild, non-specific myopathic changes.
41 ate dehydrogenase (SDH) is a hallmark of its myopathic changes.
42 muscle biopsy specimens revealed nonspecific myopathic changes.
43 ysis of T32KO skeletal muscles revealed mild myopathic changes.
44 s muscle was reported to exhibit nonspecific myopathic changes.
45 these caveolin-3 null mice reveals: (i) mild myopathic changes; (ii) an exclusion of the dystrophin-g
46    Half of the muscle biopsies (6/12) showed myopathic changes; increased connective tissue elements
47  they have been associated with a variety of myopathic complaints.
48 iency of cathepsin B could contribute to the myopathic component of this illness.
49  skeletal muscle biology in both healthy and myopathic contexts.
50               These data define an important myopathic contribution to the Kennedy disease phenotype
51  and RNA polyA+ pools relative to normal and myopathic control muscles (P < 0.002), measured relative
52 phy patients, four normal controls, and four myopathic controls).
53 ot in non-myopathic muscle or non-vacuolated myopathic controls.
54                           Mice infected with myopathic coxsackievirus B1 Tucson (CVB1(T)) develop chr
55 ines with transgenic mice overexpressing the myopathic cTnT (exon 7 deletion).
56 dditional determinant of weakness encoded by myopathic CVB1(T).
57 that is interrupted by a key proinflammatory myopathic cytokine.IMPORTANCE Skeletal muscle regenerati
58 sure of denervation and can usually identify myopathic damage, neurogenic damage, or mixed injury.
59 ic cardiomyopathy [HCM], n = 15) and dilated-myopathic (dilated cardiomyopathy [DCM], n = 37) hearts
60 rvation rather than indicating an additional myopathic disease process.
61 of an affected individual did not show clear myopathic disease, it showed significantly reduced abund
62 nd progression towards inherited or acquired myopathic disease.
63 lity, and suggest novel molecular aspects of myopathic disease.
64                           However, in severe myopathic diseases such as Duchenne Muscular Dystrophy,
65 ll-based therapies for chronic, debilitating myopathic diseases.
66  nemaline myopathy is a distinct, heritable, myopathic disorder caused by a mutation in TNNT1.
67                         These patients mimic myopathic disorders and are likely to be under-diagnosed
68  expressing various mutant genes involved in myopathic disorders.
69 d introduce a candidate for consideration in myopathic disorders.
70 rea, and pathologic fibrosis consistent with myopathic disuse atrophy.
71 ere available, for the medical management of myopathic dysfunction and transplantation in pediatric p
72  thick filaments, could be a useful index of myopathic dysfunction.
73 age of 14 years, revealed chronic and severe myopathic (dystrophic) changes, with normal staining for
74                    Importantly, we found the myopathic effects of DUX4 were p53 dependent, as p53 inh
75                 In this study, the long-term myopathic effects of ricin-mAb 35 on extraocular muscle
76 lease niacin do not substantially potentiate myopathic effects when given in combination with statins
77 iosis, elevated serum creatine kinase level, myopathic electrodiagnostic changes, brain MRI with cobb
78                                     Both had myopathic electromyogram with abnormal electrical irrita
79 ve sustained ischemia followed by a necrotic myopathic endpoint.
80 essive muscle atrophy of both neurogenic and myopathic etiologies.
81 der, with primary clinical manifestations of myopathic exercise intolerance and a macrocytic siderobl
82 ssing both nNOSmu and nNOSbeta were severely myopathic, exhibiting structural defects in the microtub
83 lateral ptosis (non-fatigable in adulthood), myopathic facies and marked axial weakness, especially o
84                           Some patients have myopathic facies.
85             AR113Q limb muscles show similar myopathic features and express decreased levels of mRNAs
86                       Muscle biopsies showed myopathic features including fibre size variability, pre
87 nital myasthenic syndrome had more prominent myopathic features that were detectable on muscle biopsi
88  observed in Gne(M712T/M712T) muscle, but no myopathic features were apparent.
89 ndrome may be complicated by the presence of myopathic features, but correct diagnosis is important b
90 ellular collagen VI microfibrils and exhibit myopathic features, including decreased muscle mass and
91 shared with the dystroglycanopathies include myopathic features, raised CK levels and variable mild c
92                        SBMA patients exhibit myopathic features, suggesting a role for muscle in dise
93 se (T32KO) that displays both neurogenic and myopathic features.
94 transgenic littermates and exhibited typical myopathic features.
95 hat these clinical abnormalities result from myopathic fibrosis of the extraocular muscles.
96 cle biopsy were concordant for neurogenic or myopathic findings, they were more accurate than either
97 teric nervous system, it likely results in a myopathic form of CIPO, bringing new insights to disease
98  previously been associated with an isolated myopathic form of MDS (OMIM 609560).
99                                Locus for the myopathic form of SP syndrome (scapuloperoneal muscular
100 20 cM telomeric to the one described for the myopathic form of SP syndrome.
101                          Both neurogenic and myopathic forms of autosomally dominantly inherited scap
102 rlap in genetic etiologies of structural and myopathic heart disease.
103 erlies a familial syndrome of electrical and myopathic heart disease.
104  to regenerate myocardium in the ischemic or myopathic heart.
105 ed impulse propagation and arrhythmia in the myopathic heart.
106                       Isolated myocytes from myopathic hearts exhibited increased volume, length, wid
107 The altered intracellular Ca handling of the myopathic hearts prolongs the decay of the L-type Ca cur
108                                          The myopathic hearts show an increased recycling of oxidized
109 anodine receptors at the Z-lines, whereas in myopathic hearts the degree of co-localization was marke
110 H7 loci and is repressed in various types of myopathic hearts, suggesting a conserved lncRNA mechanis
111 tance catheter in 87 patients with normal or myopathic hearts.
112  effects of mineralocorticoid antagonists in myopathic hearts.
113 e of this study is to measure abdominal wall myopathic histologic and mechanical changes during incis
114                                     Enhanced myopathic injury and mortality across different etiologi
115 /L and electromyographic study showed active myopathic involvement.
116 ic Drosophila melanogaster muscle expressing myopathic lamin mutations deformed more under applied st
117 cerbated limb tissue necrosis, increased the myopathic lesion size, reduced muscle regeneration, atte
118 termediates and compromised the life span of myopathic mice.
119 muscle mass and ameliorated muscle injury in myopathic mouse models.
120 es of strength and life span in the severely myopathic Mtm1delta4 mouse model of X-linked myotubular
121 M), dermatomyositis (DM), dystrophic and non-myopathic muscle as controls, and cultured human myotube
122 bers of sIBM muscle biopsies, but not in non-myopathic muscle or non-vacuolated myopathic controls.
123 o isotope tracing experiments indicated that myopathic muscle relies on lactate import to supply cent
124  in the damage and dysfunction that occur in myopathic muscle tissue.
125  exercise and injury, as muscle ages, and in myopathic muscle; however, how these changes in stiffnes
126 Free Mg(2+) levels were normal in DM and JDM myopathic muscles at rest, but were significantly lower
127                  Mg-ATP levels in DM and JDM myopathic muscles were at least 37% lower than those in
128 onformational preferences of HcTnI-C27 and a myopathic mutant, Arg192His, (HcTnI-C27-H).
129 mal structural architecture of desmin in the myopathic myofibers after revascularization.
130  the visceral organs is heterogeneous with a myopathic origin.
131                        Evidence for managing myopathic patients with coenzyme Q10 is not conclusive.
132                 Electro myography revealed a myopathic pattern associated with abnormal electrical ir
133 im32-/- mice) and showed that they display a myopathic phenotype accompanied by neurogenic features.
134 fish (srpk3(-/-); ttn.1(+/-)) replicated the myopathic phenotype and showed myofibrillar disorganizat
135    We show that Pabpn1+/A17 mice have a mild myopathic phenotype in adult and aged animals.
136                                          The myopathic phenotype of these mutant mice resembled that
137 r muscle development in zebrafish, causing a myopathic phenotype when knocked down.
138  were associated with a milder predominantly myopathic phenotype with a later mean age of disease ons
139 at lack dystrophin and exhibit only a subtle myopathic phenotype.
140 om the absence of TRIM32, which underlie the myopathic phenotype.
141                    Our work demonstrates the myopathic potential of DUX4 in animal muscle.
142 study, our objective was to test the in vivo myopathic potential of DUX4.
143 ultimately increase expression of genes with myopathic potential.
144 o been implicated in apparently rare, milder myopathic presentations termed fetal acetylcholine recep
145 induced cardiomyopathy demonstrates that the myopathic process associated with rapid heart rates is l
146 tes on muscle biopsy, emphasizing the active myopathic process underpinning the potential for severe
147                Electrophysiology suggested a myopathic process, without myotonia.
148 ral studies supported a drug-induced primary myopathic process.
149  single isolated cases displaying additional myopathic process.
150 ay of hemodynamic, ventilatory, and skeletal myopathic processes in this common, chronic condition.
151 l myopathy (FVM) is a rare inherited form of myopathic pseudo-obstruction; little is known about the
152            The clinical phenotype was purely myopathic, ranging from lethal neonatal to mild ambulato
153 uated surgical approach for degenerative and myopathic repairs by quantifying segmental prolapse, ant
154 both slow and fast isoforms was increased in myopathic skeletal muscles.
155                          Both neurogenic and myopathic SP syndromes have been described.
156 to disease pathogenesis in various inherited myopathic states in vivo remains unknown.
157        Clinical response of enteropathic and myopathic symptoms and serum albumin level.
158 ta on the effect of CoQ10 supplementation on myopathic symptoms are scarce and contradictory.
159                             Enteropathic and myopathic symptoms resolved after infliximab therapy, an
160 rame internal deletions show relatively mild myopathic symptoms, this may in the future offer a thera
161 in the parents may account for their lack of myopathic symptoms.
162  tRNA genes of mtDNA can cause predominantly myopathic syndromes and - contrary to conventional wisdo
163        These diseases can cause a variety of myopathic syndromes and can become clinically manifest a
164 tochondrial DNA can also cause predominantly myopathic syndromes and--contrary to conventional wisdom
165              Histopathology ranged from mild myopathic to severe dystrophic changes including vacuole
166 to sustain functional effects as that in the myopathic turkey cTnT.
167 determinants that were strictly conserved in myopathic viruses with one located in the 5' untranslate
168                 A muscle biopsy specimen was myopathic with a lack of myophosphorylase and multiple l

 
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