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zprogerin transgenic zebrafish embryos (∼40%) showed similar slow muscle fiber abnormalities as shown later.
We find no loss of either motor or sensory neurons at symptomatic ages; instead, our analysis identifies decreased complexity of neuromuscular junction synapses, with associated muscle fiber abnormalities.
Moreover, the slow muscle fiber abnormalities, in addition to the onset of embryonic senescence, in these transgenic embryos were significantly blocked by FTI treatment (Fig. 7C, c, f).
Experimental models of VDR null mutant mice document diffused muscle fiber abnormalities and severe alterations in muscle cell differentiation or fiber development/maturation [ 7– 9]; in humans, VDR gene polymorphisms have been associated with muscle strength defects, as recently reported [ 1, 10].
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Once we analyzed their slow skeletal muscle fibers, striking abnormalities with wavy migrations could be observed in a number of cases at 24 hpf (Fig. 7C, b).
Background: In a previous study of motor unit properties in patients with schizophrenia, muscle fiber histologic and electrophysiologic abnormalities were observed.
These include abnormalities in muscle fiber size and length, in muscle architecture (i.e. the angle and the physical properties of the fiber tendon attachment) and in the muscle fiber length ratio, fiber type and number of cross-bridges [ 2].
A muscle degenerative phenotype has also been described in morpholino-directed dystroglycan knockdown fish embryos in which there is a disruption of the DGC resulting in abnormalities in muscle fiber organization (42).
The muscle fibers showed subtle abnormalities such as slightly swollen mitochondria in focal areas of the fibers and some folding of the sarcolemma.
Activation of muscle cell death pathways was age‐dependent with most apoptotic and necrotic muscle fibers exhibiting ETC abnormalities.
We previously demonstrated that the length of ETC‐abnormal regions in an aged muscle fiber varies, with fibers containing longer ETC abnormalities being more prone to atrophy and fiber breakage (Bua et al., 2004).
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