In our previous report [17], CBDL rats had significantly higher portal pressure, elevated total bilirubin (TB), aspartate aminotransferase (AST), alanine aminotransferase (ALT), decreased partial pressure of oxygen (PaO2) and increased alveolar-arterial oxygen gradient (AaPO2) compared to the sham-operated rats, indicating the typical presentation of liver cirrhosis and HPS (see Supplementary Table S1). The gene discussed is GPT; the disease is Hermansky-Pudlak syndrome.