AASDH (aminoadipate-semialdehyde dehydrogenase) is an acyl-CoA synthetase family member involved in fatty acid and amino acid metabolism. The protein contains an AMP-binding domain and phosphopantetheine-binding domain 1, enabling covalent binding of beta-alanine in an ATP-dependent manner to form a thioester bond, with potential roles in post-translational protein modification or RNA modification. AASDH participates in lipid metabolic processes 2 and is ubiquitously expressed across tissues 1. Clinically, AASDH variants have been identified as novel genetic candidates associated with childhood-onset cardiomyopathy in consanguineous populations, with homozygous variants detected in affected individuals 3. Additionally, AASDH has been identified as a potential microsatellite instability (MSI) target gene in colorectal cancer 4, suggesting involvement in cancer-associated pathways. Notably, AASDH variants were enriched in glioblastoma cells showing reversible temozolomide chemotherapy response 5, implicating dysregulation in chemotherapy resistance. While AASDH is distinct from ALDH7A1 (which causes pyridoxine-dependent epilepsy) 6, the gene's role in metabolic pathways affecting cancer development and cardiomyopathy warrants further investigation as a potential therapeutic target.