scholarly journals Gorlin Syndrome: Recent Advances in Genetic Testing and Molecular and Cellular Biological Research

2020 ◽  
Vol 21 (20) ◽  
pp. 7559
Author(s):  
Shoko Onodera ◽  
Yuriko Nakamura ◽  
Toshifumi Azuma
Keyword(s):  
Stem Cells ◽  
Pluripotent Stem Cells ◽  
Biological Research ◽  
Driver Gene ◽  
Gorlin Syndrome ◽  
Recent Advances ◽  
Genetic Level ◽  
Skeletal Disorder ◽  
Hh Signaling ◽  
Induced Pluripotent

Gorlin syndrome is a skeletal disorder caused by a gain of function mutation in Hedgehog (Hh) signaling. The Hh family comprises of many signaling mediators, which, through complex mechanisms, play several important roles in various stages of development. The Hh information pathway is essential for bone tissue development. It is also the major driver gene in the development of basal cell carcinoma and medulloblastoma. In this review, we first present the recent advances in Gorlin syndrome research, in particular, the signaling mediators of the Hh pathway and their functions at the genetic level. Then, we discuss the phenotypes of mutant mice and Hh signaling-related molecules in humans revealed by studies using induced pluripotent stem cells.

scholarly journals Recent Advances in Disease Modeling and Drug Discovery for Diabetes Mellitus Using Induced Pluripotent Stem Cells

2016 ◽  
Vol 17 (2) ◽  
pp. 256 ◽  
Author(s):  
Mohammed Kawser Hossain ◽  
Ahmed Abdal Dayem ◽  
Jihae Han ◽  
Subbroto Kumar Saha ◽  
Gwang-Mo Yang ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Stem Cells ◽  
Drug Discovery ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Disease Modeling ◽  
Recent Advances ◽  
Induced Pluripotent

Keratinocytes from Gorlin Syndrome-induced pluripotent stem cells are resistant against UV radiation

2020 ◽  
Author(s):  
Nana Morita ◽  
Shoko Onodera ◽  
Yuriko Nakamura ◽  
Takashi Nakamura ◽  
Shin-ichi Takahashi ◽  
...  
Keyword(s):  
Stem Cells ◽  
Induced Pluripotent Stem Cells ◽  
Uv Radiation ◽  
Pluripotent Stem Cells ◽  
Gorlin Syndrome ◽  
Induced Pluripotent

scholarly journals Overcoming barriers to reprogramming and differentiation in nonhuman primate induced pluripotent stem cells

2017 ◽  
Vol 4 (2) ◽  
pp. 153-162 ◽  
Author(s):  
Jacob J. Hemmi ◽  
Anuja Mishra ◽  
Peter J. Hornsby
Keyword(s):  
Stem Cells ◽  
Induced Pluripotent Stem Cells ◽  
Dimethyl Sulfoxide ◽  
Pluripotent Stem Cells ◽  
Ips Cells ◽  
Cellular Reprogramming ◽  
Biological Research ◽  
Differentiation Potential ◽  
Wide Range ◽  
Induced Pluripotent

Abstract. Induced pluripotent stem cells (iPS cells) generated by cellular reprogramming from nonhuman primates (NHPs) are of great significance for regenerative medicine and for comparative biology. Autologously derived stem cells would theoretically avoid any risk of rejection due to host–donor mismatch and may bypass the need for immune suppression post-transplant. In order for these possibilities to be realized, reprogramming methodologies that were initially developed mainly for human cells must be translated to NHPs. NHP studies have typically used pluripotent cells generated from young animals and thus risk overlooking complications that may arise from generating iPS cells from donors of other ages. When reprogramming is extended to a wide range of NHP species, available donors may be middle- or old-aged. Here we have pursued these questions by generating iPS cells from donors across the life span of the common marmoset (Callithrix jacchus) and then subjecting them to a directed neural differentiation protocol. The differentiation potential of different clonal cell lines was assessed using the quantitative polymerase chain reaction. The results show that cells derived from older donors often showed less neural marker induction. These deficits were rescued by a 24 h pretreatment of the cells with 0.5 % dimethyl sulfoxide. Another NHP that plays a key role in biological research is the chimpanzee (Pan troglodytes). iPS cells generated from the chimpanzee can be of great interest in comparative in vitro studies. We investigated if similar deficits in differentiation potential might arise in chimpanzee iPS cells reprogrammed using various technologies. The results show that, while some deficits were observed in iPS cell clones generated using three different technologies, there was no clear association with the vector used. These deficits in differentiation were also prevented by a 24 h pretreatment with 0.5 % dimethyl sulfoxide.

scholarly journals Induced Pluripotent Stem Cells (iPSCs)—Roles in Regenerative Therapies, Disease Modelling and Drug Screening

Cells ◽  
2021 ◽  
Vol 10 (9) ◽  
pp. 2319
Author(s):  
Mourad A. M. Aboul-Soud ◽  
Alhusain J. Alzahrani ◽  
Amer Mahmoud
Keyword(s):  
Stem Cells ◽  
Induced Pluripotent Stem Cells ◽  
Drug Screening ◽  
Pluripotent Stem Cells ◽  
Embryonic Stem ◽  
Disease Modelling ◽  
Biological Research ◽  
Cord Injury ◽  
Induced Pluripotent ◽  
Regenerative Therapies

The discovery of induced pluripotent stem cells (iPSCs) has made an invaluable contribution to the field of regenerative medicine, paving way for identifying the true potential of human embryonic stem cells (ESCs). Since the controversy around ethicality of ESCs continue to be debated, iPSCs have been used to circumvent the process around destruction of the human embryo. The use of iPSCs have transformed biological research, wherein increasing number of studies are documenting nuclear reprogramming strategies to make them beneficial models for drug screening as well as disease modelling. The flexibility around the use of iPSCs include compatibility to non-invasive harvesting, and ability to source from patients with rare diseases. iPSCs have been widely used in cardiac disease modelling, studying inherited arrhythmias, neural disorders including Alzheimer’s disease, liver disease, and spinal cord injury. Extensive research around identifying factors that are involved in maintaining the identity of ESCs during induction of pluripotency in somatic cells is undertaken. The focus of the current review is to detail all the clinical translation research around iPSCs and the strength of its ever-growing potential in the clinical space.

scholarly journals Gorlin syndrome-derived induced pluripotent stem cells are hypersensitive to hedgehog-mediated osteogenic induction

PLoS ONE ◽  
2017 ◽  
Vol 12 (10) ◽  
pp. e0186879 ◽  
Author(s):  
Daigo Hasegawa ◽  
Hiromi Ochiai-Shino ◽  
Shoko Onodera ◽  
Takashi Nakamura ◽  
Akiko Saito ◽  
...  
Keyword(s):  
Stem Cells ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Gorlin Syndrome ◽  
Osteogenic Induction ◽  
Induced Pluripotent

scholarly journals Identification of an epigenetic signature in human induced pluripotent stem cells using a linear machine learning model

Human Cell ◽  
2020 ◽  
Vol 34 (1) ◽  
pp. 99-110
Author(s):  
Koichiro Nishino ◽  
Ken Takasawa ◽  
Kohji Okamura ◽  
Yoshikazu Arai ◽  
Asato Sekiya ◽  
...  
Keyword(s):  
Stem Cells ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Ethical Issues ◽  
Embryonic Stem ◽  
Learning Model ◽  
Carcinoma Cells ◽  
Biological Research ◽  
Machine Learning Model ◽  
Induced Pluripotent

AbstractThe use of human induced pluripotent stem cells (iPSCs), used as an alternative to human embryonic stem cells (ESCs), is a potential solution to challenges, such as immune rejection, and does not involve the ethical issues concerning the use of ESCs in regenerative medicine, thereby enabling developments in biological research. However, comparative analyses from previous studies have not indicated any specific feature that distinguishes iPSCs from ESCs. Therefore, in this study, we established a linear classification-based learning model to distinguish among ESCs, iPSCs, embryonal carcinoma cells (ECCs), and somatic cells on the basis of their DNA methylation profiles. The highest accuracy achieved by the learned models in identifying the cell type was 94.23%. In addition, the epigenetic signature of iPSCs, which is distinct from that of ESCs, was identified by component analysis of the learned models. The iPSC-specific regions with methylation fluctuations were abundant on chromosomes 7, 8, 12, and 22. The method developed in this study can be utilized with comprehensive data and widely applied to many aspects of molecular biology research.

Recent Advances in Therapeutic Applications of Induced Pluripotent Stem Cells

2017 ◽  
Vol 19 (2) ◽  
pp. 65-74 ◽  
Author(s):  
Farzaneh Rami ◽  
Shamsi Naderi Beni ◽  
Mahboobeh Mojaver Kahnamooi ◽  
Ilnaz Rahimmanesh ◽  
Ahmad Reza Salehi ◽  
...  
Keyword(s):  
Stem Cells ◽  
Induced Pluripotent Stem Cells ◽  
Pluripotent Stem Cells ◽  
Therapeutic Applications ◽  
Recent Advances ◽  
Induced Pluripotent
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