Oral cholic acid therapy is indicated for 3β-Hydroxy-Δ(5)-C27-steroid oxidoreductase (HSD3B7) deficiency, Δ (4)-3-oxosteroid 5β-reductase (SRD5B1, AKR1D1) deficiency, and Zellweger spectrum disorders [106]. This evidence concerns the gene AKR1D1 and Zellweger syndrome.