ACADVL is overexpressed in human AML. (A) Schematic diagram of the FAO pathway highlighting enzymes associated with the carnitine shuttle; long, medium, and short chain FAO; and ACAD electron transfer with the protein VLCAD, coded by the gene ACADVL (in red). Enzyme names are in roman type; corresponding gene names (beneath the protein name) are italicized. Each enzyme’s full name, short form name, and encoding gene are shown in supplemental Figure 1A. UQ, I, II, III, and IV are abbreviations for ubiquinone and complexes 1, 2, 3, and 4 of the ETC. Created using BioRender. (B) Volcano plots comparing the fold change in the expression of various intramitochondrial FAO genes between AML patient and healthy BM populations in the GSE9476, GSE13204, and GSE1159 data sets. (C) ACADVL gene overexpression between AML patient and healthy BM populations in GSE9476, GSE13204, and GSE1159. AML populations were stratified into quartiles, labeled Q1 through Q4. In (B) and (C), comparisons between genes are presented as a scatter plot with the x-axis as log2 of the fold change and the y-axis as a negative log10 of the P value for AML vs healthy BM populations. (D) Immunoblot showing VLCAD protein levels across patient-derived AML cells (#1-#8, where the number after # denotes the patient ID), isolated from peripheral blood, and normal MNCs. Patient cytogenetics are in supplemental Figure 10. **P ≤ .002, ***P ≤ .001, 2-tailed unpaired Student t test. n.s., not significant.