V. 
References 
1. Brown MS and Goldstein JL. A receptor-mediated pathway for cholesterol homeostasis. 
Science, Apr 4 1 986;232(4746):34-47. 
2. Goldstein JL and Brown MS. Familial hypercholesterolemia. Lipoprotein and lipid 
Metabolism Disorders. Metabolic Basis of Inherited Disease II 1989;1:1215-1250. 
3. Bilheimer DW. Portacaval shunt and liver transplantation in treatment of familial 
hypercholesterolemia. Arteriosclerosis, Jan-Feb 1989;9(1 Suppl) :I1 58-63. 
4. Thompson GR, Barbir M, Okabayashi K, Trayner I and Larkin S. Plasmapheresis in 
familial hypercholesterolemia. Arteriosclerosis, Jan-Feb 1 989 ;9(1 Suppl):l1 52-57. 
5. Starzl TE, Bilheimer DW, Bahnson HT, Shaw BW Jr, Hardesty RL, Griffity BP, Iwatsuki 
S, Zitelli BJ, Gartner JC Jr, Malatack, JJ and et al. Heart-liver transplantation in a 
patient with familial hypercholesterolemia. Lancet, Jun 23 1 984 ;1 (8391 ):1 382-83. 
6. Bilheimer DW, Goldstein JL, Grundy SM, Starzl TE and Brown MS. Liver 
transplantation to provide low density lipoprotein receptors and lower plasma 
cholesterol in a child with homozygous familial hypercholesterolemia. N Engl J Med, 
Dec 27 1 9 84 ;3 1 1 (26) :1 658-64. 
7. Shaw BW Jr, Bahnson HT, Hardesty RL, Griffity BP and Starzl TE. Combined 
transplantation of the heart and liver. Ann Surg, Dec 1985;202(6):667-72. 
8. Hoeg JM, Starzl TE and Brewer HB Jr. Liver transplantation for treatment of 
cardiovascular disease: comparison with medication and plasma exchange in homozygous 
familial hypercholesterolemia. Am J Cardio, Mar 1 1987;59(6):705-07. 
9. Valdivielso P, Escolar JL, Cuervas-Mons V, Pulpon LA, Chaparro MA and Gonzalez- 
Santos P. Lipids and lipoprotein changes after heart and liver transplantation in a 
patient with homozygous familial hypercholesterolemia. Ann Intern Med, Feb 
1 9 8 8 ; 1 08(2) :204-06. 
10. Watanabe Y. Serial inbreeding of rabbits with hereditary hyperlipidemia (WHHL 
rabbit). Atherosclerosis, Jun 1980;36(2):261-8. 
1 1 . Goldstein JL, Kita T and Brown MS. Defective lipoprotein receptors and atherosclerosis. 
Lessons from an animal counterpart of familial hypercholesterolemia. N Engl J Med, 
Aug 4 1 983;309(5):288-96. 
12. Yamamoto T, Bishop RW, Brown MS, Goldstein JL and Russell DW. Deletion in cysteine- 
rich region of LDL receptor impedes transport to cell surface in WHHL rabbit. Science, 
Jun 6 1 986;232(4755):1 230-37. 
13. Havel RJ, Yamada N and Shames DM. Watanabe heritable hyperlipidemic rabbit. Animal 
model for familial hypercholesterolemia. Arteriosclerosis, Jan-Feb 1989;9(1 
Suppl) :l33-38. 
1 4. Wilson JM, Johnston DE, Jefferson DM and Mulligan RC. Correction of the genetic defect 
in hepatocytes from the Watanabe heritable hyperlipidemic rabbit. Proc Natl Acad Sci 
USA, Jun 1 988;85(1 2):4421 -25. 
15. Wilson, JM and Chowdhury JR. Prospects for gene therapy of familial 
hypercholesterolemia. Mol Biol Med, Jun 1990;7(3):223-32. 
16. Wilson JM, Chowdhury NR, Grossman M, Wajsman R, Epstein A, Mulligan RC and 
Chowdhury JR. Temporary amelioration of hyperlipidemia in low density lipoprotein 
receptor-deficient rabbits transplanted with genetically modified hepatocytes. Proc 
Natl Acad Sci USA, Nov 1987;87(21):8437-41 . 
17. Wilson JM, Chowdhury NR, Grossman M, Gupta SJ, Jeffers J, Huang TJ and Chowdhury 
JR. Transplantation of allogeneic hepatocytes into LDL receptor deficient rabbits leads 
to transient improvement in hypercholesterolemia. Clin Biotechnology, Spring 1991. 
(In Press). 
[196] 
Recombinant DNA Research, Volume 15 
