Generation of four patient-specific pluripotent induced stem cell lines from two Brazilian patients with amyotrophic lateral sclerosis and two healthy subjects

Gubert, Fernanda; Vasques, Juliana Ferreira; Cozendey, Tatiane D.; Domizi, Pablo; Toledo, María Fernanda; Kasai-Brunswick, Taís Hanae; Loureiro, Marli P.S.; Lima, J.A. De; Gress, Claudio H.; Cabello, Giselda M. K.; Cabello, Pedro Hernán; Borgonovo, Tamara; Vaz, Isadora May; Silva, Rosane; Mendez-Otero, Rosália

doi:10.1016/j.scr.2019.101448

Cited by 5 publications

(2 citation statements)

References 3 publications

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“…Expression of pluripotency markers was determined by RT-PCR and flow cytometry (BD Accuri C6, BD FACSAria II and FlowJo software version 1). The reprogramming method used has already been validated ( Kasai-Brunswick et al, 2018 ; Mesquita et al, 2019 ; Gubert et al, 2019a ; Gubert et al ., 2019b ).…”

Section: Methodsmentioning

confidence: 99%

Chromosomal aberrations after induced pluripotent stem cells reprogramming

et al. 2021

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Induced pluripotent stem cells (iPSCs) are generated from adult cells that have been reprogrammed to pluripotency. However, in vitro cultivation and genetic reprogramming increase genetic instability, which could result in chromosomal abnormalities. Maintenance of genetic stability after reprogramming is required for possible experimental and clinical applications. The aim of this study was to analyze chromosomal alterations by using the G-banding karyotyping method applied to 97 samples from 38 iPSC cell lines generated from peripheral blood or Wharton’s jelly. Samples from patients with long QT syndrome, Jervell and Lange-Nielsen syndrome and amyotrophic lateral sclerosis and from normal individuals revealed the following chromosomal alterations: acentric fragments, chromosomal fusions, premature centromere divisions, double minutes, radial figures, ring chromosomes, polyploidies, inversions and trisomies. An analysis of two samples generated from Wharton’s jelly before and after reprogramming showed that abnormal clones can emerge or be selected and generate an altered lineage. IPSC lines may show clonal and nonclonal chromosomal aberrations in several passages (from P6 to P34), but these aberrations are more common in later passages. Many important chromosomal aberrations were detected, showing that G-banding is very useful for evaluating genetic instability with important repercussions for the application of iPSC lines.

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Section: Methodsmentioning

confidence: 99%

Chromosomal aberrations after induced pluripotent stem cells reprogramming

et al. 2021

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“…Therefore, it is urgent to establish a representative model of ALS with less well-understood genetic backgrounds, such as sporadic ALS, since it affects many more patients. Currently the establishment of sporadic patient-specific iPSCs lines is flourishing [ 75 ]. Researchers reprogram sALS patients’ fibroblasts into iPSCs, which then differentiate into neurons with a disease phenotype.…”

Section: Modeling Of Alsmentioning

confidence: 99%

Induced Pluripotent Stem Cells and Their Applications in Amyotrophic Lateral Sclerosis

Huo

Chen

et al. 2023

Cells

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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that results in the loss of motor function in the central nervous system (CNS) and ultimately death. The mechanisms underlying ALS pathogenesis have not yet been fully elucidated, and ALS cannot be treated effectively. Most studies have applied animal or single-gene intervention cell lines as ALS disease models, but they cannot accurately reflect the pathological characteristics of ALS. Induced pluripotent stem cells (iPSCs) can be reprogrammed from somatic cells, possessing the ability to self-renew and differentiate into a variety of cells. iPSCs can be obtained from ALS patients with different genotypes and phenotypes, and the genetic background of the donor cells remains unchanged during reprogramming. iPSCs can differentiate into neurons and glial cells related to ALS. Therefore, iPSCs provide an excellent method to evaluate the impact of diseases on ALS patients. Moreover, patient-derived iPSCs are obtained from their own somatic cells, avoiding ethical concerns and posing only a low risk of immune rejection. The iPSC technology creates new hope for ALS treatment. Here, we review recent studies on iPSCs and their applications in disease modeling, drug screening and cell therapy in ALS, with a particular focus on the potential for ALS treatment.

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Induced pluripotent stem cells (iPSCs) as game‐changing tools in the treatment of neurodegenerative disease: Mirage or reality?

Yousefi

Abdollahii

Kouhbanani

et al. 2020

Journal Cellular Physiology

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Based on investigations, there exist tight correlations between neurodegenerative diseases' incidence and progression and aberrant protein aggregreferates in nervous tissue. However, the pathology of these diseases is not well known, leading to an inability to find an appropriate therapeutic approach to delay occurrence or slow many neurodegenerative diseases' development. The accessibility of induced pluripotent stem cells (iPSCs) in mimicking the phenotypes of various late‐onset neurodegenerative diseases presents a novel strategy for in vitro disease modeling. The iPSCs provide a valuable and well‐identified resource to clarify neurodegenerative disease mechanisms, as well as prepare a promising human stem cell platform for drug screening. Undoubtedly, neurodegenerative disease modeling using iPSCs has established innovative opportunities for both mechanistic types of research and recognition of novel disease treatments. Most important, the iPSCs have been considered as a novel autologous cell origin for cell‐based therapy of neurodegenerative diseases following differentiation to varied types of neural lineage cells (e.g. GABAergic neurons, dopamine neurons, cortical neurons, and motor neurons). In this review, we summarize iPSC‐based disease modeling in neurodegenerative diseases including Alzheimer's disease, amyotrophic lateral sclerosis, Parkinson's disease, and Huntington's disease. Moreover, we discuss the efficacy of cell‐replacement therapies for neurodegenerative disease.

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Generation of four patient-specific pluripotent induced stem cell lines from two Brazilian patients with amyotrophic lateral sclerosis and two healthy subjects

Cited by 5 publications

References 3 publications

Chromosomal aberrations after induced pluripotent stem cells reprogramming

Chromosomal aberrations after induced pluripotent stem cells reprogramming

Induced Pluripotent Stem Cells and Their Applications in Amyotrophic Lateral Sclerosis

Induced pluripotent stem cells (iPSCs) as game‐changing tools in the treatment of neurodegenerative disease: Mirage or reality?

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