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Lymphoblastoid Cell Lines as Models to Study Mitochondrial Function in Neurological Disorders

Journal

Publisher

MDPI
DOI: 10.3390/ijms22094536

Keywords

lymphoblastoid cell lines; mitochondria; neurodegenerative disease; neurological disorder; cell models

Funding

  1. MJ Fox Foundation [10862.01]
  2. Mason Foundation National Medical Program [MASONMECFS051, MAS2018F00026]

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The study of mitochondrial function in neurological disorders is challenging due to the inaccessibility of brain tissue, but the use of lymphoblastoid cell lines (LCLs) is an effective approach. These cell models can help understand mitochondrial defects, signaling pathways, and biomarkers in neurological disorders, and are valuable for the development of diagnostic tests and identification of drug targets.
Neurological disorders, including neurodegenerative diseases, are collectively a major cause of death and disability worldwide. Whilst the underlying disease mechanisms remain elusive, altered mitochondrial function has been clearly implicated and is a key area of study in these disorders. Studying mitochondrial function in these disorders is difficult due to the inaccessibility of brain tissue, which is the key tissue affected in these diseases. To overcome this issue, numerous cell models have been used, each providing unique benefits and limitations. Here, we focussed on the use of lymphoblastoid cell lines (LCLs) to study mitochondrial function in neurological disorders. LCLs have long been used as tools for genomic analyses, but here we described their use in functional studies specifically in regard to mitochondrial function. These models have enabled characterisation of the underlying mitochondrial defect, identification of altered signalling pathways and proteins, differences in mitochondrial function between subsets of particular disorders and identification of biomarkers of the disease. The examples provided here suggest that these cells will be useful for development of diagnostic tests (which in most cases do not exist), identification of drug targets and testing of pharmacological agents, and are a worthwhile model for studying mitochondrial function in neurological disorders.

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