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Kinetic bases of the primar hperlipidaemias: studies of apolipoprotein B turnover in geneticall defined subjects
Author(s) -
JANUS E. D.,
NICOLL A. M.,
TURNER P. R.,
MAGILL P.,
LEWIS B.
Publication year - 1980
Publication title -
european journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.164
H-Index - 107
eISSN - 1365-2362
pISSN - 0014-2972
DOI - 10.1111/j.1365-2362.1980.tb02076.x
Subject(s) - apolipoprotein b , medicine , cholesterol
. Autologous 131 I‐labelled ver low densit lipoprotein (VLDL) and 125 I‐labelled low densit lipoprotein (LDL) were injected into seven normal subjects and into fort‐three hperlipidaemic patients, classified into groups on the basis of famil studies and clinical findings, to quantitate VLDL and LDL apolipoprotein B kinetics. In normal subjects, mean VLDL‐B peptide snthetic rate was 151 mg kg ‐1 da ‐1 , mean LDL‐B peptide snthetic rate 7‐7 mg kg ‐1 da ‐1 and mean LDL‐B fractional catabolic rate (FCR) 0–31 da ‐1 . In heterozgous familial hpercholesterolaemia ( n = 14) VLDL‐B peptide production was normal in patients with normal triglceride levels; in those with high triglceride levels there was either VLDL overproduction or a catabolic defect. LDL‐B peptide snthetic rates ranged from high normal to increased (8‐5‐180 mg kg ‐1 da ‐1 ) and LDL‐B peptide FCR values were markedl reduced (0–14‐0‐28 da ‐1 ) confirming the presence of a defect in LDL catabolism but indicating over‐production as well. In familial combined hperlipidaemia ( n = 11) VLDL‐B peptide production ranged from normal to elevated (13‐9‐44‐4 mg kg ‐1 da ‐1 , mean 23‐8 mg kg ‐1 da ‐1 ) correlating with the VLDL triglceride level (i.e. with the phenotpic expression of the disorder). LDL‐B peptide production ranged from high normal to markedl increased (8–9‐19‐5 mg kg ‐1 da ‐1 , mean 12‐2 mg kg ‐1 da ‐1 ) and correlated with LDL cholesterol levels (i.e. the phenotpe), ( r =+ 0–66, P < 005). Three patients with unclassified hpercholesterolaemia had increased LDL‐B peptide snthetic rates. One patient with remnant hperlipoproteinaemia (tpe III) had a high normal VLDL‐B peptide snthetic rate, 17‐3 mg kg ‐1 da ‐1 , and a strikingl low FCR of VLDL‐B. In familial hpertriglceridaemia (three patients)