Porcine Model of Huntington's Disease

Rausova, Petra; Vochozková, Petra; Vidinská, Daniela; Hrnciarova, Bozena Bohuslavova Eva; Macakova, Monika; Valeková, Ivona; StefanJuhas,; Ardan, Taras; Solc, P; Motlı́k, Jan; Ellederová, Zdeňka

doi:10.5772/66353

Cited by 3 publications

(3 citation statements)

References 89 publications

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“…How much these differences affect the interpretation of the EEG and the SedLine®derived variables is not known. Precise and homogeneous information on the functional areas in the pig's brain is still missing [25][26][27][28][29][30]. Further anatomical studies on a larger sample of animals are required.…”

Section: Discussionmentioning

confidence: 99%

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Usability of the SedLine® electroencephalographic monitor of depth of anaesthesia in pigs: a pilot study

Mirra

Casoni

Barge

et al. 2022

J Clin Monit Comput

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To investigate the usability of the SedLine® monitor in anaesthetized pigs. Five juvenile healthy pigs underwent balanced isoflurane-based general anaesthesia for surgical placement of a subcutaneous jugular venous port. The SedLine® was applied to continuously monitor electroencephalographic (EEG) activity and its modulation during anaesthesia. Computer tomography and magnetic resonance were performed to investigate the relationship between electrodes’ positioning and anatomical structures. The pediatric SedLine® EEG-sensor could be easily applied and SedLine®-generated variables collected. An EEG Density Spectral Array (DS) was displayed over the whole procedure. During surgery, the EEG signal was dominated by elevated power in the delta range (0.5–4 Hz), with an underlying broadband signal (where power decreased with increasing frequency). The emergence period was marked by a decrease in delta power, and a more evenly distributed power over the 4–40 Hz frequency range. From incision to end of surgery, mean SedLine®-generated values (± standard deviation) were overall stable [23.0 (± 2.8) Patient State Index (PSI), 1.0% (± 3.8%) Suppression Ratio (SR), 8.8 Hz (± 2.5 Hz) Spectral Edge Frequency 95% (SEF) left, 7.7 Hz (± 2.4 Hz) SEF right], quickly changing during emergence [75.3 (± 11.1) PSI, 0.0 (± 0.0) SR, 12.5 (± 6.6) SEF left 10.4 (± 6.6) SEF right]. Based on the imaging performed, the sensor does not record EEG signals from the same brain areas as in humans. SedLine®-DSA and -generated variables seemed to reflect variations in depth of anaesthesia in pigs. Further studies are needed to investigate this correlation, as well as to define the species-specific brain structures monitored by the EEG-sensor.

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Section: Discussionmentioning

confidence: 99%

“…Further studies are needed to investigate the correlation between Sedline®-generated variables and actual depth of anaesthesia in pigs. Within the dorsolateral prefrontal cortex [24]/motor part of the frontal cortex [25,26], approximately covering the region of the somatosensory and insular cortex [27]/temporoparietal cortex [28] R1…”

Section: Discussionmentioning

confidence: 99%

Usability of the SedLine® electroencephalographic monitor of depth of anaesthesia in pigs: a pilot study

Mirra

Casoni

Barge

et al. 2022

J Clin Monit Comput

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“…Even though non-human primates share the greatest similarities to humans genetically, porcine models are preferable in many aspects such as availability, breeding, size, and a 80% overlap of immune parameters (Meurens et al, 2011 ). Porcine models have already been generated for neurological disorders such as Huntington's disease (Rausova et al, 2017 ), stroke (Lau et al, 2018 ), and other neurodegenerative disorders (Perleberg et al, 2018 ). Such models can provide platforms for testing of non-immunogenic cell lines for treatment prior to human testing.…”

Section: Generating Non-immunogenic Ipscmentioning

confidence: 99%

Non-immunogenic Induced Pluripotent Stem Cells, a Promising Way Forward for Allogenic Transplantations for Neurological Disorders

et al. 2021

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Neurological disorder is a general term used for diseases affecting the function of the brain and nervous system. Those include a broad range of diseases from developmental disorders (e.g., Autism) over injury related disorders (e.g., stroke and brain tumors) to age related neurodegeneration (e.g., Alzheimer's disease), affecting up to 1 billion people worldwide. For most of those disorders, no curative treatment exists leaving symptomatic treatment as the primary mean of alleviation. Human induced pluripotent stem cells (hiPSC) in combination with animal models have been instrumental to foster our understanding of underlying disease mechanisms in the brain. Of specific interest are patient derived hiPSC which allow for targeted gene editing in the cases of known mutations. Such personalized treatment would include (1) acquisition of primary cells from the patient, (2) reprogramming of those into hiPSC via non-integrative methods, (3) corrective intervention via CRISPR-Cas9 gene editing of mutations, (4) quality control to ensure successful correction and absence of off-target effects, and (5) subsequent transplantation of hiPSC or pre-differentiated precursor cells for cell replacement therapies. This would be the ideal scenario but it is time consuming and expensive. Therefore, it would be of great benefit if transplanted hiPSC could be modulated to become invisible to the recipient's immune system, avoiding graft rejection and allowing for allogenic transplantations. This review will focus on the current status of gene editing to generate non-immunogenic hiPSC and how these cells can be used to treat neurological disorders by using cell replacement therapy. By providing an overview of current limitations and challenges in stem cell replacement therapies and the treatment of neurological disorders, this review outlines how gene editing and non-immunogenic hiPSC can contribute and pave the road for new therapeutic advances. Finally, the combination of using non-immunogenic hiPSC and in vivo animal modeling will highlight the importance of models with translational value for safety efficacy testing; before embarking on human trials.

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Behavioral Assessment of Stress Compensation in Minipigs Transgenic for the Huntington Gene Using Cortisol Levels: A Proof-of-Concept Study

Schuldenzucker

Schubert

Muratori

et al. 2018

JHD

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Porcine Model of Huntington's Disease

Cited by 3 publications

References 89 publications

Usability of the SedLine® electroencephalographic monitor of depth of anaesthesia in pigs: a pilot study

Usability of the SedLine® electroencephalographic monitor of depth of anaesthesia in pigs: a pilot study

Non-immunogenic Induced Pluripotent Stem Cells, a Promising Way Forward for Allogenic Transplantations for Neurological Disorders

Behavioral Assessment of Stress Compensation in Minipigs Transgenic for the Huntington Gene Using Cortisol Levels: A Proof-of-Concept Study

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