Induced pluripotent stem cell line derived from a sporadic amyotrophic lateral sclerosis patient.

Stem Cell Res

Hebei Medical University-National University of Ireland Galway Stem Cell Research Center, Hebei Medical University, Hebei Province 050017, China; Hebei Research Center for Stem Cell Medical Translational Engineering, Hebei Province 050017, China; Human Anatomy Department, Hebei Medical University, Hebei Province 050017, China. Electronic address:

Published: May 2020

Induced pluripotent stem cells (iPSCs) were generated from peripheral blood mononuclear cells (PBMCs) obtained from a 60-year-old female diagnosed with sporadic amyotrophic lateral sclerosis (sALS). The iPSCs shared the same karyotype with the parent PBMCs, expressed pluripotency stem cell markers, and demonstrated trilineage differentiation potential. This cell line could serve as an ideal model to investigate the mechanisms underlying amyotrophic lateral sclerosis (ALS).

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.scr.2020.101841DOI Listing

Publication Analysis

Top Keywords

amyotrophic lateral
12
lateral sclerosis
12
induced pluripotent
8
pluripotent stem
8
stem cell
8
sporadic amyotrophic
8
cell derived
4
derived sporadic
4
sclerosis patient
4
patient induced
4

Similar Publications

Neurodegenerative diseases are significant health concerns that have a profound impact on the quality and duration of life for millions of individuals. These diseases are characterized by pathological changes in various brain regions, specific genetic mutations associated with the disease, deposits of abnormal proteins, and the degeneration of neurological cells. As neurodegenerative disorders vary in their epidemiological characteristics and vulnerability of neurons, treatment of these diseases is usually aimed at slowing disease progression.

View Article and Find Full Text PDF

Artificial enforcement of the unfolded protein response (UPR) reduces disease features in multiple preclinical models of ALS/FTD.

Mol Ther

January 2025

Program of Cellular and Molecular Biology, Biomedical Sciences Institute (ICBM), Universidad de Chile, Santiago, Chile; Biomedical Neuroscience, Faculty of Medicine, Universidad de Chile, Santiago, Chile; FONDAP Center for Geroscience, Brain Health and Metabolism, Santiago, Chile; Buck Institute for Research on Aging, Novato, CA, USA. Electronic address:

Amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (FTD) are part of a spectrum of diseases that share several causative genes, resulting in a combinatory of motor and cognitive symptoms and abnormal protein aggregation. Multiple unbiased studies have revealed that proteostasis impairment at the level of the endoplasmic reticulum (ER) is a transversal pathogenic feature of ALS/FTD. The transcription factor XBP1s is a master regulator of the unfolded protein response (UPR), the main adaptive pathway to cope with ER stress.

View Article and Find Full Text PDF
Article Synopsis
  • Sarcoid-like reaction (SLR) is an immune response affecting lymph nodes and organs, which doesn't fulfill the criteria for systemic sarcoidosis, and can be associated with certain diseases like Chronic lymphocytic leukaemia (CLL) and Amyotrophic lateral sclerosis (ALS).
  • A 60-year-old male patient with treated CLL developed ALS symptoms following exposure to Venetoclax and Rituximab, presenting with rashes and weakness that progressed over a year.
  • Diagnosis complications included atypical signs and symptoms, leading to misdiagnosis of neurosarcoidosis and challenges in treatment, despite initial interventions with prednisolone and infliximab.
View Article and Find Full Text PDF

Loss of Insight in Syndromes Associated with Frontotemporal Lobar Degeneration: Clinical and Imaging Features.

Am J Geriatr Psychiatry

December 2024

Department of Clinical and Experimental Sciences (DA, BB), University of Brescia, Brescia, Italy; Molecular Markers Laboratory (BB), IRCCS Istituto Centro San Giovanni di Dio Fatebenefratelli, Brescia, Italy. Electronic address:

Objectives: The present study aims to assess the prevalence, associated clinical symptoms, longitudinal changes, and imaging correlates of Loss of Insight (LOI), which is still unexplored in syndromes associated with Frontotemporal Lobar Degeneration (FTLD).

Design: Retrospective longitudinal cohort study, from Oct 2009 to Feb 2023.

Setting: Tertiary Frontotemporal Dementia research clinic.

View Article and Find Full Text PDF

The role of mitochondrial remodeling in neurodegenerative diseases.

Neurochem Int

January 2025

Department of Environmental and Occupational Health, School of Public Health, Guangdong Medical University, Dongguan, 523808, PR China; Dongguan Key Laboratory of Environmental Medicine, School of Public Health, Guangdong Medical University, Dongguan, 523808, PR China. Electronic address:

Neurodegenerative diseases are a group of diseases that pose a serious threat to human health, such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD) and Amyotrophic Lateral Sclerosis (ALS). In recent years, it has been found that mitochondrial remodeling plays an important role in the onset and progression of neurodegenerative diseases. Mitochondrial remodeling refers to the dynamic regulatory process of mitochondrial morphology, number and function, which can affect neuronal cell function and survival by regulating mechanisms such as mitochondrial fusion, division, clearance and biosynthesis.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!