SLC25A42 encodes a mitochondrial carrier protein that mediates the transport of coenzyme A (CoA) across the inner mitochondrial membrane in exchange for intramitochondrial adenine nucleotides and adenosine 3',5'-diphosphate 1. The protein exhibits high transport affinity for CoA, dephospho-CoA, ADP, and adenosine 3',5'-diphosphate through counter-exchange transport mechanisms 1. SLC25A42 is critical for maintaining mitochondrial CoA pools and supporting CoA-dependent pathways including the TCA cycle and fatty acid oxidation 2. The transporter enables spatial regulation of anabolic and catabolic processes by compartmentalizing acyl-CoA metabolism 2. Biallelic pathogenic variants in SLC25A42 cause a mitochondrial disorder characterized by encephalomyopathy with variable severity, including myopathy, developmental delay, lactic acidosis, and metabolic decompensations 34. The disorder shows extremely variable clinical presentation, with some patients experiencing mild symptoms while others suffer metabolic crises with neurological regression 3. Most reported cases involve individuals of Arab descent carrying a founder variant (c.871A>G, p.Asn291Asp) 3. Pantothenic acid administration has shown promise as a therapeutic intervention, leading to clinical stabilization and increased CoA levels in patient fibroblasts 4.