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Biomedical subjects

J C Fruchart

Publications and source records attributed to J C Fruchart.

At least 199 records · Page 11Linked to original sources
PubMed1996

Alterations in plasma lipoproteins and apolipoproteins before the age of 40 in heterozygotes for lipoprotein lipase deficiency.

We have assessed the expression of heterozygosity for lipoprotein lipase (LPL) deficiency by studying a single large French Canadian family comprising 92 persons including 21 carriers of the catalytically defective P207L mutation. Phenotypic changes distinguishing heterozygotes from controls were seen early, before the age of 40 and often before 20 years of age. In the total cohort these changes included an elevation in the mean very low density (VLDL) and intermediate density lipoprotein (IDL) triglyceride (+69%; P = 0.01 and +40%; P = 0.001) and cholesterol (+51%; P = 0.03 and +67%; P = 0.007) and apoB levels but decreased HDL2 and HDL3 cholesterol, (-32%; P = 0.01 and -15%; P = 0.002 respectively). While the lipid compositions of VLDL and IDL were similar between heterozygotes and controls, the low density (LDL) and high density lipoproteins (HDL) of carriers were triglyceride enriched. Heterozygotes also had a markedly lower apoC-III ratio (apoC-III in supernatant/apoC-III in heparin precipitate) (1.46 vs. 3.86 P = 1 x 10(-4)) indicating a substantial enrichment of VLDL and IDL with apoC-III and depletion of HDL apoC-III supporting this ratio as an effective index for efficiency of lipolysis. LpA-I was markedly reduced (0.34 vs. 0.43 P = 1 x 10(-5)) showing that levels of this particle are partly dependent on LPL catalytic activity. Heterozygotes manifest from an early age with a markedly reduced HDL, LpA-I, apoC-III ratio and an increased TC/HDLc ratio which would predict a relatively increased risk of premature coronary artery disease, compared to their normal siblings.

Adult↗
PubMed1996

Lipoprotein(a) as a marker for coronary heart disease.

Lipoprotein(a) (Lp(a)) resembles a low-density lipoprotein (LDL) particle, but with the addition of apolipoprotein(a). Apolipoprotein(a) (apo(a)) is homologous to plasminogen, and its similarity to plasminogen indicates a prothrombogenic role for Lp(a), whereas the similarity of Lp(a) to LDL suggests a proatherogenic role. Thus, Lp(a) is a link between atherosclerosis and thrombosis. The apo(a) gene is highly polymorphic, and a relationship exists between the different apo(a) alleles and plasma Lp(a) levels. There is also evidence to suggest an association between serum Lp(a) concentration and the frequency of cardiovascular atherosclerosis, particularly myocardial infarction in men, although some of the recently published epidemiological studies have been controversial. The heightened awareness of increased Lp(a) levels being a risk factor for coronary artery disease has led to the evaluation of the effects of lipid-lowering agents on Lp(a) levels. The data that have emerged, however, are conflicting, and are derived mostly from short-term studies involving the use of statins. Our group has studied the short- and long-term effect of fluvastatin on Lp(a) in hypercholesterolaemic patients; fluvastatin was found to have no effect in such patients as observed in the short-term studies, but significant reductions in Lp(a) levels were noted during long-term treatment. Although our findings are in agreement with those of a previously published report, there is still a great need to confirm the data that have been reported in separate studies.

Biomarkers↗
PubMed1996

Effects of gender and menopausal status on plasma lipoprotein subspecies and particle sizes.

The risk of coronary heart disease (CHD) is lower in women than in men, but increases in women after menopause. Some of the gender, age, and menopausal-related differences in CHD risk may relate to differences in lipoprotein subspecies. We therefore examined these subspecies in three groups of healthy subjects: premenopausal women (W, n = 72, mean age 41.2 +/- 6.5), postmenopausal women (PMW, n = 74, 55.8 +/- 7.4), and men (M, n = 139, 48.8 +/- 10.7). We measured plasma levels of lipids, lipoprotein cholesterol, apolipoproteins A-I, A-IV, B, C-III, and E, and lipoprotein subspecies Lp A-I, Lp A-I:A-II, Lp B, Lp B:C-III, and Lp B:E, as well as LDL and HDL particle sizes. Our data indicate that women have significantly higher values of HDL-C, apoA-I, apoE, and Lp A-I; larger LDL and HDL particle sizes; and lower values of triglyceride, apoB, and Lp B:C-III particles than men, with no difference in Lp A-I:A-II. Postmenopausal status was associated with significantly higher values of total cholesterol, triglyceride, VLDL-C, and LDL-C; increased levels of apoB, C-III, and E; elevated values of Lp B, Lp B:C-III, and Lp B:E; and lower levels of HDL-C along with smaller HDL particle size. Moreover, we noted a strong correlation between LDL and HDL particle size. Our data are consistent with the concepts that male gender confers decreases in HDL subspecies due to lower Lp A-I levels; while postmenopausal status results in higher levels of all apoB-containing lipoproteins (Lp B, Lp B:C-III, and Lp B:E). The lipoprotein alterations associated with male gender and postmenopausal status would be expected to increase CHD risk.

Adult↗
PubMed1996

Serum Lp AI lipoprotein particles and other antiatherogenic lipid parameters in normolipidaemic obese subjects.

The android pattern of body fat distribution has been shown to increase the risk of metabolic and coronary heart disease. Protective lipid markers against cardiovascular disease were studied in 98 obese normolipidaemic, non diabetic, non-smoker subjects over 18 years of age according to regional distribution of adipose tissue as estimated by the waist ship ratio (WHR) and overall obesity as estimated by the body mass index (BMI). WHR was inversely correlated with Lp AI (r = 0.46) and HDL-cholesterol (r = 0.37). BMI was not correlated with protective lipid parameters but only with triglycerides. After adjustment, Lp AI was lower in men and in upper body obese women (p < 0.05). Lp AI is a better indicator of body fat distribution than HDL-cholesterol or apo AI, and its variations appear to be indirectly related to gender, menopause, and age, thereby influencing body fat distribution (the main factor accounting for Lp AI variation). Lp AI was inversely correlated with WHR in gluteal-femoral obese women but not in abdominally obese women or men, possibly because of a threshold effect.

Adult↗
PubMed1996

Cigarette smoking is associated with differences in nutritional habits and related to lipoprotein alterations independently of food and alcohol intake.

OBJECTIVE: To assess the effects of smoking on serum lipoprotein levels taking into account current nutritional intakes. DESIGN: Case-control study comparing smokers and non-smokers. SETTING: Ambulatory subjects of the Urban Community of Lille examined at home or at the Health Care Center. SUBJECTS: Men, between 45 and 65 years of age; n = 89 smokers (cases) and n = 91 non-smokers (controls); non-smokers were randomly selected from the voter's registration lists, 7 smokers and 2 non-smokers were excluded for hyperlipidemia. INTERVENTIONS: Blood sampling, medical examination and three-day food records validated by a registered dietician. RESULTS: Smokers were younger (P < 0.001) and thinner (P = 0.003), chose more frequently visible fats of animal origin (P = 0.03) and reported significantly larger daily meat servings (P = 0.01) than non-smokers. Smokers had a significantly higher intake of non-alcoholic energy (P < 0.05), lipids of animal origin (P = 0.003), saturated fat (P < 0.01) and monounsaturated fat (P < 0.01) and a lower P/S ratio (P = 0.02) than non-smokers. After adjustment on age and body mass index, smokers had significantly higher levels of mean serum triglycerides (P = 0.03), VLDL-cholesterol (P = 0.0003) and systolic blood pressure (P = 0.02) and lower values of HDL-cholesterol (P = 0.02) than non-smokers. Additional adjustment for alcohol consumption showed significantly lower apo A-I values (P = 0.03) in smokers than non-smokers. Further adjustment for the intake of lipids of animal origin revealed higher apo B values (P = 0.04) in smokers than non-smokers. CONCLUSIONS: (1) Smoking is associated with serum lipid and lipoprotein alterations independently of nutritional factors, (2) Nutritional habits of smokers are less 'healthy' than those of non-smokers (3) Smokers is a group of particular importance in terms of multifactorial public health intervention.

Aged↗
PubMed1995

Regulation of rat liver apolipoprotein A-I, apolipoprotein A-II and acyl-coenzyme A oxidase gene expression by fibrates and dietary fatty acids.

The regulation by fibrates and dietary fatty acids of the hepatic gene expression of apolipoproteins (apo) A-I and A-II, the major protein constituents of high-density lipoproteins, as well as of acyl-CoA oxidase, the rate-limiting enzyme of the peroxisomal beta-oxidation pathway, was studied in vivo in the rat and in vitro in primary cultures of rat hepatocytes. In primary hepatocytes, different fibrates decreased apo A-I and increased acyl-CoA oxidase mRNA levels, whereas apo A-II mRNA only decreased in level after treatment with fenofibric acid, but not after bezafibrate, gemfibrozil or Wy-14643 treatment. Treatment with fenofibric acid counteracted the increase in apo A-I mRNA levels observed after dexamethasone or all-trans retinoic acid treatment, whereas simultaneous addition of fenofibric acid together with all-trans retinoic acid or dexamethasone resulted in a superinduction of acyl-CoA oxidase mRNA. Addition of the n-3 polyunsaturated fatty acids (PUFAs), docosanohexaenoic acid and eicosanopentaenoic acid, or the fatty acid derivative alpha-bromopalmitate, decreased apo A-I and increased acyl-CoA oxidase mRNA in a dose-dependent and time-dependent manner, whereas apo A-II mRNA did not change significantly. Nuclear run-on experiments demonstrated that fenofibric acid and alpha-bromopalmitate decreased apo A-I and increased acyl-CoA oxidase gene expression at the transcriptional level. When rats were fed isocaloric diets enriched in saturated fat (hydrogenated coconut oil), n-6 PUFAs (safflower oil) or n-3 PUFAs (fish oil), a significant decrease in liver apo A-I and apo A-II mRNA levels was only observed after fish oil feeding. Compared to feeding low fat, liver acyl-CoA oxidase mRNA increased after fat feeding, but this effect was most pronounced (twofold) in rats fed fish oil. Results from these studies indicate that fish oil feeding reduces rat liver apo A-I and apo A-II gene expression, similar to results obtained after feeding fenofibrate. Fibrates and n-3 fatty acids (and the fatty acid derivative, alpha-bromopalmitate) down-regulate apo A-I and induce acyl-CoA oxidase gene expression through a direct transcriptional action on the hepatocyte. In contrast, only fenofibric acid, but not the other fibrates or fatty acids tested, decrease apo A-II gene expression in vitro.

Acyl-CoA Oxidase↗
PubMed1995

Changes in plasma apolipoprotein B-containing lipoparticle levels following therapy with fluvastatin and cholestyramine. European Fluvastatin Study Group.

Epidemiologic studies have demonstrated that apolipoprotein (apo) B-containing lipoparticles (LpE:B, LpC-III:B) are associated with the risk of coronary artery disease whereas apo A-1-containing lipoparticles (LpA-I) are protective against coronary artery disease. The effect on lipoparticle levels of the 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitor fluvastatin, in combination with cholestyramine, was assessed in a double-blind randomized study. A total of 144 patients with primary hypercholesterolemia were recruited, who had successfully completed an original study comparing the effects of fluvastatin and cholestyramine on plasma lipoparticle levels. All subjects fulfilled the following inclusion criteria: plasma low density lipoprotein cholesterol (LDL-C) levels > 160 mg/dL, with premature coronary artery disease and 2 associated risk factors; or LDL-C > 190 mg/dL, no coronary artery disease, and triglycerides < 300 mg/dL, after a lipid-lowering diet. Patients were randomized to 1 of 3 combination therapy groups: fluvastatin 20 mg/day plus cholestyramine 4 g/day; fluvastatin 20 mg/day plus cholestyramine 8 g/day; and fluvastatin 20 mg/day plus cholestyramine 16 g/day. The study length was 6 weeks and patients were examined at 3-week intervals. Fluvastatin plus cholestyramine produced a significant (p < 0.001), dose-dependent reduction in levels of cholesterol (range, -29 to -34%), LDL-C (range, -30 to -44%), apo B (range, -23 to -34%), and apo E (range, -33 to -43%). LpE:B levels were also reduced (range, -19 to -26%), but not significantly.(ABSTRACT TRUNCATED AT 250 WORDS)

Anticholesteremic Agents↗
PubMed1995

Detection of autoantibodies against oxidized low-density lipoproteins and of IgG-bound low density lipoproteins in patients with coronary artery disease.

The role of oxidized low-density lipoprotein (ox-LDL) in the pathogenesis of atherosclerosis has been the object of intense investigation. It has been proposed that, due to the antigenic properties of ox-LDL, the anti-ox-LDL antibody titre could represent a useful index of in vivo LDL oxidation. On the other hand, LDL immune complexes (LDL-IC) have been demonstrated in patients with coronary disease and could play an atherogenic role. The goal of our study was to investigate anti-malondialdehyde (MDA)-LDL autoantibodies and LDL-IC in a cohort of patients with coronary artery disease. Seventy control subjects and 70 coronary angiographically documented patients were compared; in addition 32 healthy male non-smokers were compared with 32 healthy male smokers (> 10 cigarettes/day). All patients were matched for age and cholesterolemia. Enzyme-linked immunosorbent assay was used to measure anti-MDA-LDL autoantibodies and LDL-IC. Titres of anti-MDA-LDL autoantibodies were not larger in patients with documented coronary artery stenosis and in smokers than they were in controls and non-smokers. The titre of LDL-IC was not higher in patients with coronary artery stenosis than in controls. The results thus indicate that in populations matched for age and cholesterolemia the titres of anti-MDA-LDL autoantibodies and the titre of LDL-IC are not increased in patients suffering from coronary artery stenosis. Furthermore, cigarette smoking does not induce higher titres of anti-MDA-LDL autoantibodies in healthy patients.

Aged↗
PubMed1995

Modulation of cholesterol efflux from Fu5AH hepatoma cells by the apolipoprotein content of high density lipoprotein particles. Particles containing various proportions of apolipoproteins A-I and A-II.

The influence of apolipoproteins (apo) A-I and A-II on the ability of high density lipoproteins (HDL) to remove cholesterol from cultured Fu5AH rat hepatoma cells was studied independently on alterations in the overall structure and lipid composition of the lipoprotein particles. To this end, apoA-I was progressively replaced by apoA-II in ultracentrifugally isolated HDL3 without inducing changes in other remaining lipoprotein components. As apoA-II was progressively substituted for apoA-I in HDL3 (A-II:A-I+A-II percentage mass: 29.5, 47.6, 71.5, 97.4, and 98.9%), the rate of cholesterol efflux from Fu5AH hepatoma gradually and significantly decreased after 2 or 4 h of incubation at 37 degrees C (cholesterol efflux: 30.4 +/- 0.8, 24.1 +/- 1.0, 19.8 +/- 1.2, 15.7 +/- 1.4, and 13.4 +/- 1.3%/2h, respectively; 38.4 +/- 1.5, 29.2 +/- 0.9, 27.0 +/- 0.2, 20.4 +/- 0.4, and 17.5 +/- 1.0%/4h, respectively) (p < 0.01 with all A-II-enriched HDL3 fractions as compared with non-enriched homologues). In agreement with data obtained with total HDL3, increasing the A-II:A-I+A-II percentage mass in HDL3 particles containing initially only apoA-I (HDL3-A-I) progressively reduced cellular cholesterol efflux. After 2 h of incubation, cholesterol efflux correlated negatively with A-II:A-I+A-II percentage mass (r = -0.86; p < 0.0001; n = 20), but not with either free cholesterol:phospholipid ratio, A-I+A-II:total lipid ratio or mean size of HDL3. As determined by using Spearman rank correlation analysis, the A-II:A-I+A-II% mass ratio correlated negatively with the apparent maximal efflux (Vmax(efflux)) (rho = -0.68; p < 0.05, n = 10), but not with the HDL3 concentration required to obtain 50% of maximal efflux (Km(efflux)) (rho = -0.08; not significant, n = 10). It was concluded that the apoA-I and apoA-II content of HDL3 is one determinant of its ability to promote cholesterol efflux from Fu5AH rat hepatoma cells.

Animals↗
PubMed1995

Tissue-specific expression of the human gene for lecithin: cholesterol acyltransferase in transgenic mice alters blood lipids, lipoproteins and lipases towards a less atherogenic profile.

Lecithin:cholesterol acyltransferase (LCAT) is a key enzyme in the reverse cholesterol pathway but its role in lipid metabolism is still unclear. We have generated mice transgenic for a 7-kb genomic DNA fragment comprising the 6 exons and 5 introns of the LCAT gene with 1932 bp of 5' flanking and 908 bp of 3' flanking sequences. One line had integrated about 30 copies and expressed about 40-fold increased LCAT activity in a human test system. The expression showed correct tissue specificity of the human LCAT gene. Increased LCAT activity resulted in a decrease of plasma triacylglycerols below 50% of fasting controls. This reduction was seen in all lipoprotein fractions. Lipoprotein lipase activity did not change significantly, whereas hepatic triacylglycerol lipase increased markedly. Plasma total cholesterol was similar in fasting transgenic and control mice, but low-density lipoprotein and very low-density lipoprotein cholesterol were reduced to about 50%. High-density lipoprotein cholesterol increased about 20%, accompanied by a correspondingly increased size and a higher cholesterol efflux-stimulating activity of transgenic LCAT high-density lipoprotein. Both apolipoprotein A-I and A-II plasma concentrations increased in transgenic mice. Plasma triacylglycerol and cholesteryl ester fatty acid distribution showed an increased proportion of palmitic acid, whereas oleic, linoleic and arachidonic acid decreased, thus resembling more closely the human situation. Overexpression of the human LCAT gene provokes major changes in plasma lipoprotein and apolipoprotein concentrations, resulting in a less atherogenic plasma lipoprotein profile through a reduction in atherogenic and an increase in anti-atherogenic lipoproteins.

Animals↗
PubMed1995

[Detection of the Arg 3500-->Gln mutation of B apolipoprotein. Value in clinical practice].

OBJECTIVE: Familial defective apolipoprotein B-100, caused by a mutation in position 3500 of apolipoprotein B, induces hypercholesterolaemia, a major risk factor of coronary artery disease. The objective is to evaluate the interest of the detecting subjects bearing the Arg3500-->Gln mutation and to observe the response of these subjects to different physiological and therapeutic situations, in clinical practice. METHODS: We performed a systematic screening among hypercholesterolaemic outpatients attending a lipid clinic. The heterozygote subjects were followed up during more than one year and, in some of them, we compared the efficiency of cholesterol-lowering drugs belonging to the different classes: fibrate and statine. RESULTS: Two probands and 3 related subjects were detected. Three patients were treated. For two patients, reduction of LDL-cholesterol plasma levels was observed with both drugs but was significantly higher with statine than with fibrate. For the third one, carrying the E2E4 phenotype, the statine did not seemed to have a major effect. CONCLUSION: The number of probands is in agreement with the frequency reported in literature. A possible interaction of the effect of statine with the apo E2 isoform is discussed.

Adult↗
PubMed1995

A 700-bp fragment of the human antithrombin III promoter is sufficient to confer high, tissue-specific expression on human apolipoprotein A-II in transgenic mice.

The human antithrombin III-encoding gene (hAT-III) promoter (phAT-III) was used to generate transgenic mice producing a human hepatic apolipoprotein, apolipoprotein A-II (hApoA-II). Integration of the transgene into the mouse genome resulted in the efficient production of hApoA-II in plasma, reaching up to 0.40 g/l in two transgenic lines. The human ApoA-II mRNA was detected at high levels, both in the liver and in the kidney of transgenic mice. The rat AT-III gene shows the same expression pattern. In contrast, as previously described, the same promoter permitted the expression of the SV40 large T antigen only in the liver of transgenic mice. In view of the extra-hepatic distribution of the ApoA-II mRNA, a preliminary characterization of the hAT-III proximal promoter (phAT-III), driving the expression of the transgene, was realized. Using DNase I footprinting analysis with liver nuclear extracts, four protected regions (I-IV) were identified in the first 175 bp of the 5' region of hAT-III. Electrophoretic mobility shift assays performed with liver and kidney nuclear extracts indicate that region III (nucleotides (nt) -67 to -90) interacts with the liver-enriched HNF4 nuclear factor. Furthermore, our data suggest that region I (nt -123 to -138) interacts with the liver-enriched HNF3 transcription factor family, both in liver and kidney. Taken together, these results demonstrate that phAT-III is a useful tool to create transgenic mice producing high plasma levels of a human apolipoprotein due to expression of the transgene in liver and kidney.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1995

Serum lipoprotein profile in Algerian patients with celiac disease.

Celiac disease is characterized by a gluten-induced villous atrophy of the upper small intestine which has an active role in the lipoprotein metabolism. In the present study the lipoprotein profiles of different patients were analyzed to determine the effect of impaired enterocyte function in celiac disease. We compared serum lipid parameters in controls and in celiac disease patients. The major differences between celiac disease patients and the control group were a diminution of cholesterol and phospholipids in HDL and LDL in the former. These differences persisted after treatment; in addition, a lower level of cholesterol in VLDL was observed. Plasma LpAI and apo A-I levels were significantly lower (-17 and -15%) in celiac disease patients than in controls. Both levels remained low after gluten-free diet. In Algerian patients, treatment with gluten-free diet did not give any return towards normal lipids concentrations.

Adolescent↗
PubMed1995

Native like structure and stability of apo AI in a n-propanol/water solution as determined by 13C NMR.

To elucidate the molecular details of the conformation of apolipoprotein AI (apo AI), we have developed an approach related to the solubilization of this protein in 30% n-propanol. We have previously reported the promotion of a native-like structure for apo AI solubilized in n-propanol, as depicted by circular dichroism, fluorescence, and limited proteolytic digestion as compared to the lipid associated form of apo AI. In the present study, we labeled the Lys residues of apo AI with 13C by reductive methylation and used 13C NMR to confirm the formation of a native-like structure of apo AI in this environment. Furthermore, by the above criteria (circular dichroism and 13C NMR) and by using urea and temperature as denaturing agents, we show that the denaturation of the native-like structure of apo AI in n-propanol is a biphasic process. These studies show that in 30% n-propanol, apo AI contains two independently folded structural domains, of markedly different stabilities that might correspond to the amino-terminal and the carboxy-terminal halves of the molecule.

1-Propanol↗
PubMed1995

Prevention of in vitro low-density lipoprotein oxidation by an albumin-containing Lp A-I subfraction.

High-density lipoprotein (HDL) and two lipoprotein particles, those containing apo A-I and apo A-II (LpA-I:A-II) and those containing only apo A-I (LpA-I) were examined for their effect on Cu(2+)-catalyzed oxidation of human low-density lipoprotein (LDL). Lipoproteins and lipoprotein particles were prepared from plasma samples of five healthy subjects. LDL and HDL were purified by ultracentrifugation, LpA-I and LpA-I:A-II were isolated by an immunoaffinity chromatography procedure. The contaminating albumin often linked to the LpA-I affinity purified particles was eliminated by selected affinity immunosorption. The presence of HDL, LpA-I or LpA-I:A-II, at an apo A-I-containing lipoproteins/LDL ratio of 1, did not prevent LDL oxidation when assessed by oxidation kinetics, electrophoretic mobility, amounts of thiobarbituric acid-reactive products and fragmentation of apo B-100. On the other hand, when the albumin removing step was omitted, the subfraction of albumin-containing LpA-I particles impeded and even inhibited the oxidation of LDL in an albumin dose-dependent manner.

Copper↗
PubMed1995

Lipoprotein pattern and plasma lecithin cholesterol acyl transferase activity in children with Alagille syndrome.

Alagille syndrome is frequently associated with hyperlipidemia and xanthoma. The aim of the study was to assess the lipid profile (plasma lipoproteins, apolipoproteins (apo)) and lecithin cholesterol acyl transferase (LCAT) activity, with and without treatment with cholestyramine in Alagille syndrome. Five children (mean age = 6 +/- 4 years) with Alagille syndrome were studied at two different times while receiving no treatment, and while receiving cholestyramine. They were compared with 12 normal controls, who were not different from patients for age and sex. In Alagille syndrome, total serum cholesterol, triglycerides and phospholipids were elevated compared with the controls (P < 0.008). VLDL-cholesterol, LDL-cholesterol, HDL-triglycerides, LDL-triglycerides and VLDL-phospholipids were higher, whereas HDL-cholesterol was lower than controls (P < 0.03). Apo B, CIII, E and lipoprotein particles Lp AI were higher (P < 0.001), whereas Lp AI:AII was lower than controls (P < 0.03). Lipoprotein-X was present in the 5 children with Alagille syndrome and explained in part the elevation of plasma cholesterol, phospholipids, and apo CIII. LCAT activity was decreased (P < 0.01) and might cause some abnormalities of HDL with lower cholesterol, higher triglycerides, apo E and apo CIII contents than controls, and abnormalities of VLDL and LDL with higher cholesterol, triglycerides, phospholipids and apo B contents than controls. Some of the risk factors of atherosclerosis were found in Alagille syndrome, namely high levels of plasma cholesterol, LDL cholesterol, apo B, apo B/apo AI. Treatment with cholestyramine resulted in a few modifications to the lipid profile, while lipoprotein-X and the decrease of LCAT activity persisted.

Adolescent↗