Liver cell transplantation for Crigler-Najjar syndrome type I: Update and perspectives

Table 1 Representative liver cell transplantation experiments in the Gunn rat model

Donor cells	Injection site	Hepatic injury	Outcome	Cell tracking	References
50 × 106 free or encapsulated congeneic Hc	Peritoneum	None	34.8% serum bilirubin reduction with encapsulated Hc vs 13.5% with free Hc at 1 mo	Light and electron microscopy	22
10 × 106 syngeneic Hc	Liver	Hepatectomy	Significant reduction of serum bilirubin up to 4 wk	ND	24
			Apparition of conjugates in bile
10 × 106 congeneic Hc	Spleen	None	Significant reduction of serum bilirubin up to 12 mo	ND	27
			Apparition of bile conjugates at 4 mo
2-20 × 106 congeneic Hc	Portal vein	Right portal vein ligation	Significant reduction of serum bilirubin when injury with 2 × 106 Hc or with 20 × 106 without injury up to 30 d	UGT1A1 activity, WB, PCR for ugt1 gene	28
			Conjugates in bile after 10 d
5 × 106 congeneic Hc	Spleen	Hepatic irradiation ± Hepatectomy	Normalization of serum bilirubin only with combined injury	UGT1A1 activity, WB, IHC	29
			Conjugates in bile detected up to 5 mo
10 × 106 congeneic Hc	Spleen	Hepatic irradiation ± FasL-induced apoptosis	Normalization of serum bilirubin up to 160 d	UGT1A1 activity, WB, IHC	31
			Conjugates in bile at 150 d
			Estimation of repopulation at 52 ± 15% when combined injury
40 × 106 fetal or adult syngeneic Hc	Spleen	Retrorsine + Triiodothyronine	Significant reduction of serum bilirubin (+ conjugates in bile) up to 90 d (no difference between fetal and adult cells)	PCNA	32

Hc: Hepatocyte; IHC: Immunohistochemistry; PCNA: Proliferating cell nuclear antigen; WB: Western blot.