Sentences with phrase «in dystrophic»

Inset shows higher resolution confocal images of photoreceptor cell nuclear layers (DAPI blue) and rhodopsin expression (red) in the dystrophic control (left inset) and dystrophic with iPS - RPE transplant (right inset) RCS rat.

Darabi R, Arpke RW, Irion S, et al., Human ES - and iPS - derived myogenic progenitors restore DYSTROPHIN and improve contractility upon transplantation in dystrophic mice.

In vivo gene editing in dystrophic mouse muscle and muscle stem cells.

A head - tracking response is observed in the dystrophic RCS rat during optokinetic testing of the left eye, 13 weeks after iPS - RPE cell transplantation.

The progression of retinal dystrophy in the RCS rat is such that by 13 weeks post-graft most of the ONL has disappeared [33] and the photoreceptor outer segment layer is reduced to a debris zone [40], a finding we observed in dystrophic controls.

As these factors can exert protective effects on retinal neurons [52]--[56] and neurons in other neurodegenerative disease models [49], [57], [58], it seems likely that these substances may contribute to the latent photoreceptor cell survival we observe in the dystrophic retina.

In dystrophic neurites, autophagosomes, multivesicular bodies, multilamellar bodies, and cathepsin - containing autophagolysosomes were the predominant organelles and accumulated in large numbers.

The striking accumulations of immature AV forms in dystrophic neurites suggest that the transport of AVs and their maturation to lysosomes may be impaired, thereby impeding the suspected neuroprotective functions of autophagy.

Working in dystrophic mice while searching for a cure for Duchenne muscular dystrophy (DMD), Dr. Huard's laboratory team first identified a unique population of muscle - derived stem cells with the ability to repair muscle 8 years ago.

In dystrophic RCS rats, threshold levels at P100 are greater than 3.0 log units above the background level of 0.02 log candela / m2.

«VBP15 has an additional property of addressing membrane defects in dystrophic muscle cells,» remarked Kanneboyina Nagaraju, DVM, PhD, the lead author of the study, and principal investigator in the Center for Genetic Medicine at Children's National.

The study «Elastase levels and activity are increased in dystrophic muscle and impair myoblast cell survival, proliferation and differentiation» is published in the journal Scientific Reports.

Not exact matches

In myotonic dystrophic patients, titration of MBNL protein by RNA containing expanded CUG repeats leads to expression of a fetal splicing form of the cardiac sodium channel, SCN5A, inappropriate to adult heart physiology, ultimately resulting in cardiac conduction delay and heart arrhythmias, which are two key features of myotonic dystrophIn myotonic dystrophic patients, titration of MBNL protein by RNA containing expanded CUG repeats leads to expression of a fetal splicing form of the cardiac sodium channel, SCN5A, inappropriate to adult heart physiology, ultimately resulting in cardiac conduction delay and heart arrhythmias, which are two key features of myotonic dystrophin cardiac conduction delay and heart arrhythmias, which are two key features of myotonic dystrophy.

Later, the researchers simply added the compound to the drinking water in the habitats of young dystrophic mice.

This approach has proved successful in disrupting multiple genes simultaneously in neurons and in editing the Dmdmdx mutation in muscle, thereby restoring dystrophin protein expression and partially alleviating the dystrophic muscles» functional deficiencies.

Muscle satellite cells from GRMD dystrophic dogs are not phenotypically distinguishable from wild type satellite cells in ex vivo culture.

Topics such as the mechanisms of cell injury in normal and dystrophic muscle, compensatory muscle regeneration and hypertrophy, and the effects of various therapies or voluntary exercise on muscle repair, satellite cell activation, muscle growth, bone density and age - related atrophy are examined using a large variety of cellular, molecular and whole - animal in vivo assays of function.

Gross brain pathology from infants with presumed or laboratory - confirmed ZIKV infection, primarily from neuroimaging, closely resembles neuropathology associated with congenital cytomegalovirus (CMV).48 The most notable difference is the distribution of intracranial calcifications (ie, typically subcortical in congenital ZIKV infection and periventricular in CMV).48, 49 Such calcifications are likely dystrophic and related to cell death, either by necrosis, apoptosis, or both.50

We used the head - tracking response to assess the visual function of RCS dystrophic rats 13 weeks after receiving a subretinal iPS - RPE transplantation in one eye only (Fig. 6 and Movies S2 and S3).

iPS - RPE cells were injected into the subretinal space between the host RPE and photoreceptor cells (B) A layer of iPS - RPE cells in the subretinal space of the dystrophic RCS rat 20 hours following transplantation.

In order to test the therapeutic efficacy of iPS - RPE, we transplanted cells into the subretinal space of RCS dystrophic rats (Fig. 5A).

Only occasional foci of c - Fos positive cells were detectable in the INL of the non-transplanted dystrophic retina where the ONL consisted of a single layer.

Conservation of visual acuity in the iPS - RPE transplanted eyes was associated with the preservation of photoreceptors in the host outer nuclear layer (ONL — Fig. 7A), identified by the expression of rhodopsin in the outer segments of photoreceptors (Fig. 7A inset, Dystrophic + transplant).

(A) Preservation of optokinetic head - tracking response to a rotating vertical stimulus in 16 - week - old RCS dystrophic rats following transplantation of iPS - RPE.

Citation: Carr A-J, Vugler AA, Hikita ST, Lawrence JM, Gias C, Chen LL, et al. (2009) Protective Effects of Human iPS - Derived Retinal Pigment Epithelium Cell Transplantation in the Retinal Dystrophic Rat.

(A) Extensive preservation of the nuclear photoreceptor layers in the dorsal retina of the dystrophic RCS rat 13 weeks following transplantation of iPS - RPE cells (DAPI stained nuclei).

iPS - RPE are capable of phagocytosing photoreceptor material, in vitro and in vivo following transplantation into the Royal College of Surgeons (RCS) dystrophic rat.

The distribution of occasional light - activated c - Fos cells observed in the GCL of non-transplanted dystrophic rats matches that reported for intrinsically light - responsive melanopsin ganglion cells [35].

Read the full article, «Branched fibers from old fast - twitch dystrophic muscles are the sites of terminal damage in muscular dystrophy,» published ahead of print in the American Journal of Physiology — Cell Physiology.

No head - tracking response is observed in the 16 - week old dystrophic RCS rat during optokinetic testing of the right, non-transplanted, eye.

The cause may be either dystrophic (local factors only causing damage leading to calcification) or metastatic (too high a concentration of calcium in the blood leading to deposits of calcium in the locally damaged tissues).