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Literature DB >> 33692757

Modeling Maturity Onset Diabetes of the Young in Pluripotent Stem Cells: Challenges and Achievements.

Carmel Braverman-Gross¹, Nissim Benvenisty¹.

Abstract

Maturity onset diabetes of the young (MODY), is a group of monogenic diabetes disorders. Rodent models for MODY do not fully recapitulate the human phenotypes, calling for models generated in human cells. Human pluripotent stem cells (hPSCs), capable of differentiation towards pancreatic cells, possess a great opportunity to model MODY disorders in vitro. Here, we review the models for MODY diseases in hPSCs to date and the molecular lessons learnt from them. We also discuss the limitations and challenges that these types of models are still facing.

Entities: Chemical

Keywords: disease modeling; genetic and epigenetic aberrations; human embryonic stem cells; induced pluripotent stem cells; maturity onset diabetes of the young

Mesh：

Year: 2021 PMID： 33692757 PMCID： PMC7937923 DOI： 10.3389/fendo.2021.622940

Source DB: PubMed Journal: Front Endocrinol (Lausanne) ISSN： 1664-2392 Impact factor: 5.555

67 in total

1. Controlled induction of human pancreatic progenitors produces functional beta-like cells in vitro.

Authors: Holger A Russ; Audrey V Parent; Jennifer J Ringler; Thomas G Hennings; Gopika G Nair; Mayya Shveygert; Tingxia Guo; Sapna Puri; Leena Haataja; Vincenzo Cirulli; Robert Blelloch; Greg L Szot; Peter Arvan; Matthias Hebrok
Journal: EMBO J Date: 2015-04-23 Impact factor: 11.598

Review 2. Genetic and epigenetic stability of human pluripotent stem cells.

Authors: Riikka J Lund; Elisa Närvä; Riitta Lahesmaa
Journal: Nat Rev Genet Date: 2012-09-11 Impact factor: 53.242

3. Recapitulating endocrine cell clustering in culture promotes maturation of human stem-cell-derived β cells.

Authors: Gopika G Nair; Jennifer S Liu; Holger A Russ; Stella Tran; Michael S Saxton; Richard Chen; Charity Juang; Mei-Lan Li; Vinh Q Nguyen; Simone Giacometti; Sapna Puri; Yuan Xing; Yong Wang; Gregory L Szot; Jose Oberholzer; Anil Bhushan; Matthias Hebrok
Journal: Nat Cell Biol Date: 2019-02-01 Impact factor: 28.824

4. The maturity-onset diabetes of the young (MODY1) transcription factor HNF4alpha regulates expression of genes required for glucose transport and metabolism.

Authors: M Stoffel; S A Duncan
Journal: Proc Natl Acad Sci U S A Date: 1997-11-25 Impact factor: 11.205

Modeling Maturity Onset Diabetes of the Young in Pluripotent Stem Cells: Challenges and Achievements.

1. Controlled induction of human pancreatic progenitors produces functional beta-like cells in vitro.

Review 2. Genetic and epigenetic stability of human pluripotent stem cells.

3. Recapitulating endocrine cell clustering in culture promotes maturation of human stem-cell-derived β cells.

4. The maturity-onset diabetes of the young (MODY1) transcription factor HNF4alpha regulates expression of genes required for glucose transport and metabolism.

Review 5. CRISPR technologies for stem cell engineering and regenerative medicine.

Review 6. Causes and effects of haploinsufficiency.

Review 7. From Genetic Association to Molecular Mechanisms for Islet-cell Dysfunction in Type 2 Diabetes.

Review 8. Quality control: Genome maintenance in pluripotent stem cells.

Review 9. Concise Review: Human Pluripotent Stem Cells for the Modeling of Pancreatic β-Cell Pathology.

Review 10. Genetics of Monogenic Diabetes: Present Clinical Challenges.