Moreover, the impact of HADHA and HADHB mutations found in patients with TFP/LCHAD deficiency on CL content or structure has only been cursorily investigated in isolated LCHAD deficiency due to the HADHA c.1528G>C (p.E510Q) mutation (32). Here, TRIM39 is linked to long chain 3-hydroxyacyl-CoA dehydrogenase deficiency.