The glycine N-acyltransferases, GLYAT and GLYATL1, contribute to the detoxification of isovaleryl-CoA - an in-silico and in vitro validation
Isovaleric acidemia (IVA), due to isovaleryl-CoA dehydrogenase (IVD) deficiency, results in the accumulation of isovaleryl-CoA, isovaleric acid and secondary metabolites. The increase in these metabolites decreases mitochondrial energy production and increases oxidative stress. This contributes to t...
Main Authors: | Stefan Kühn, Monray E. Williams, Marli Dercksen, Jörn Oliver Sass, Rencia van der Sluis |
---|---|
Format: | Article |
Language: | English |
Published: |
Elsevier
2023-01-01
|
Series: | Computational and Structural Biotechnology Journal |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S2001037023000430 |
Similar Items
-
Functional Characterisation of Three Glycine <i>N</i>-Acyltransferase Variants and the Effect on Glycine Conjugation to Benzoyl–CoA
by: Johann M. Rohwer, et al.
Published: (2021-03-01) -
Decreased Expression of GLYATL1 Predicts Poor Prognosis in Patients with Clear Cell Renal Cell Carcinoma
by: Deng L, et al.
Published: (2023-08-01) -
Downregulation of Glycine N-Acyltransferase in Kidney Renal Clear Cell Carcinoma: A Bioinformatic-Based Screening
by: Juan P. Muñoz, et al.
Published: (2023-11-01) -
Retrospective Review of Positive Newborn Screening Results for Isovaleric Acidemia and Development of a Strategy to Improve the Efficacy of Newborn Screening in the UK
by: Rachel S. Carling, et al.
Published: (2024-03-01) -
A Simple Flow Injection Analysis–Tandem Mass Spectrometry Method to Reduce False Positives of C5-Acylcarnitines Due to Pivaloylcarnitine Using Reference Ions
by: Takanari Hattori, et al.
Published: (2022-05-01)