Familial hypercholesterolemia as a homozygous form in the rate of 1:1 000 000 inhabitants leads to the first clinical manifestation in the first decade of the life.Patients with both defected allelas for apolipoproteins B100 and E receptors do not reach the age of 20.Liver transplantation (LT)is only one successful therapy. The possibilities to study the influence of LT to FH are very limited.Prague hereditary hypercholesterolemic (PHHC)rat is an imbread line with high basal cholesterolemia.After stimulation of hypercholesterolemia by cholesterol diet the lipoproteine composition there is very similar to human patology.The cause of hypercho- lesterolemia is the feedback disorder of cholesterol synthesis in liver.There is a severe displacement to higher plasmatic cholesterol levels in hepatocytes and vessels resulting to a significant rise of LDL and IDL concen- trations after the high volume lipid and cholesterol diet.There were 15 liver transplantations in PHHC rats provided by authors.The plasmatic cholesterol level was significantly decreased from 4.46 mmol/1 ±1.24 to 2.24 mmol/l ±0.98.Liver transplantation is a very effective therapeutic method in this indication.

        Key words: familial hypercholesterolemia –experimental liver transplantation –Prague hereditary hyper- cholesterolemic rat