Case Reports
doi: 10.1016/0026-0495(77)90124-x.
Turnover of apolipoprotein-B in two subjects with familial hypobetalipoproteinemia
- PMID: 189158
- DOI: 10.1016/0026-0495(77)90124-x
Item in Clipboard
Case Reports
Turnover of apolipoprotein-B in two subjects with familial hypobetalipoproteinemia
Metabolism.
1977 Jan.
Abstract
The metabolism of the apoprotein of low-density apoliporprotein-B was studied in a father and son with familial hypobetalipoproteinemia. The synthetic rate of apolipoprotein-B was below the normal range in both subjects, while the fractional removal rate was normal. It is concluded that the subnormal concentration of low-density lipoprotein (LDL) inthese two subjects is due to a low synthetic rate. The synthetic rate of apolipoprotein-B of very low-density lipoprotein (BLDL-apoB) measured in the father was subnormal. The fraction of the VLDL-apoB pool which was oncverted into LDL-apoB was within the normal range. It is suggested that a low synthetic rate of VLDL may underlie the disorder.
Similar articles
-
Integrated regulation of very low density lipoprotein triglyceride and apolipoprotein-B kinetics in man: normolipemic subjects, familial hypertriglyceridemia and familial combined hyperlipidemia.Metabolism. 1981 Sep;30(9):856-68. doi: 10.1016/0026-0495(81)90064-0. Metabolism. 1981. PMID: 7266376
-
Effects of dietary saturated and polyunsaturated fat on the metabolism of apolipoproteins A-I and B. Study of a patient with type IIb hyperlipoproteinaemia.Clin Chim Acta. 1978 Aug 1;87(3):395-404. doi: 10.1016/0009-8981(78)90184-5. Clin Chim Acta. 1978. PMID: 209911
-
Effects of short- and long-term growth hormone replacement on lipoprotein composition and on very-low-density lipoprotein and low-density lipoprotein apolipoprotein B100 kinetics in growth hormone-deficient hypopituitary subjects.Metabolism. 2003 Jan;52(1):50-9. doi: 10.1053/meta.2003.50061. Metabolism. 2003. PMID: 12524662 Clinical Trial.
-
The metabolism of very low density and intermediate density lipoproteins in patients with familial hypercholesterolaemia.Atherosclerosis. 1982 Jun;43(2-3):217-31. doi: 10.1016/0021-9150(82)90024-7. Atherosclerosis. 1982. PMID: 7115461
-
Lipoprotein Metabolism in APOB L343V Familial Hypobetalipoproteinemia.J Clin Endocrinol Metab. 2015 Nov;100(11):E1484-90. doi: 10.1210/jc.2015-2731. Epub 2015 Aug 31. J Clin Endocrinol Metab. 2015. PMID: 26323024
Cited by
-
Apolipoprotein B detected in the plasma of a patient with homozygous hypobetalipoproteinaemia: implications for aetiology.J Med Genet. 1983 Jun;20(3):189-95. doi: 10.1136/jmg.20.3.189. J Med Genet. 1983. PMID: 6876109 Free PMC article.
-
Unusual presentation of three siblings with familial heterozygous hypobetalipoproteinaemia.Eur J Pediatr. 2003 Mar;162(3):129-131. doi: 10.1007/s00431-002-1123-5. Epub 2003 Jan 11. Eur J Pediatr. 2003. PMID: 12655413
-
A truncated species of apolipoprotein B (B67) in a kindred with familial hypobetalipoproteinemia.J Clin Invest. 1991 May;87(5):1748-54. doi: 10.1172/JCI115193. J Clin Invest. 1991. PMID: 2022744 Free PMC article.
-
Development of an integrated model for analysis of the kinetics of apolipoprotein B in plasma very low density lipoproteins, intermediate density lipoproteins, and low density lipoproteins.J Clin Invest. 1985 Aug;76(2):575-85. doi: 10.1172/JCI112009. J Clin Invest. 1985. PMID: 4031063 Free PMC article.
-
Hypobetalipoproteinaemia--a variant of the Bassen-Kornzweig syndrome.Gut. 1979 Feb;20(2):163-8. doi: 10.1136/gut.20.2.163. Gut. 1979. PMID: 428829 Free PMC article.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Miscellaneous
