SUMOylation of Arginyl tRNA Synthetase Modulates the Drosophila Innate Immune Response

Nayak, Prajna; Kejriwal, Aarti; Ratnaparkhi, Girish S.

doi:10.3389/fcell.2021.695630

Cited by 1 publication

(1 citation statement)

References 49 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Non-canonical enzyme functions are an important consideration when generating and interpreting novel ARS disease models. A number of non-canonical ARS functions have been demonstrated in vivo , including regulation of the immune response ( Nayak et al, 2021 ), myogenic differentiation ( Dai et al, 2021 ), autophagy ( Han et al, 2012 ), cell proliferation, and tumorigenesis ( Ho et al, 2021 ). Understanding secondary ARS functions can enable better anticipation of cellular dysfunction that may result from pathogenic variants, guiding identification of relevant disease biomarkers and selection of appropriate treatment strategies.…”

Section: Animal Model Systems For Studying Recessive Ars Diseasesmentioning

confidence: 99%

Recessive aminoacyl-tRNA synthetase disorders: lessons learned from in vivo disease models

et al. 2023

View full text Add to dashboard Cite

Protein synthesis is a fundamental process that underpins almost every aspect of cellular functioning. Intriguingly, despite their common function, recessive mutations in aminoacyl-tRNA synthetases (ARSs), the family of enzymes that pair tRNA molecules with amino acids prior to translation on the ribosome, cause a diverse range of multi-system disorders that affect specific groups of tissues. Neurological development is impaired in most ARS-associated disorders. In addition to central nervous system defects, diseases caused by recessive mutations in cytosolic ARSs commonly affect the liver and lungs. Patients with biallelic mutations in mitochondrial ARSs often present with encephalopathies, with variable involvement of peripheral systems. Many of these disorders cause severe disability, and as understanding of their pathogenesis is currently limited, there are no effective treatments available. To address this, accurate in vivo models for most of the recessive ARS diseases are urgently needed. Here, we discuss approaches that have been taken to model recessive ARS diseases in vivo, highlighting some of the challenges that have arisen in this process, as well as key results obtained from these models. Further development and refinement of animal models is essential to facilitate a better understanding of the pathophysiology underlying recessive ARS diseases, and ultimately to enable development and testing of effective therapies.

show abstract