Because acetyl‐carnitine was increased in ivd‐1(tm6784); bcat‐1(hh58) (Figure 3d) and has been reported as a marker of adequate carnitine supplementation in the clinical treatment of isovaleric acidemia (Itoh et al., 1996), we wondered whether increased isovaleric acid underline lower UPS functionality in ivd‐1(tm6784) mutants. Here, BCAT1 is linked to isovaleric acidemia.