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Hypoalphalipoproteinemias (Hypoalphalipoproteinemia)

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Conditions with abnormally low levels of ALPHA-LIPOPROTEINS (high-density lipoproteins) in the blood. Hypoalphalipoproteinemia can be associated with mutations in genes encoding APOLIPOPROTEIN A-I; LECITHIN CHOLESTEROL ACYLTRANSFERASE; and ATP-BINDING CASSETTE TRANSPORTERS.

Also Known As:

Hypoalphalipoproteinemia; Familial High-Density Lipoprotein Deficiency Disease; Familial alpha-Lipoprotein Deficiency Disease; HDL Lipoprotein Deficiency Disease; High-Density Lipoprotein Deficiency Disease, Familial; Hypo alpha Lipoproteinemia; Hypoalphalipoproteinemia, Primary; Familial High Density Lipoprotein Deficiency Disease; Familial Hypoalphalipoproteinemia; Familial Hypoalphalipoproteinemias; Familial alpha Lipoprotein Deficiency Disease; High Density Lipoprotein Deficiency Disease, Familial; Hypo alpha Lipoproteinemias; Hypoalphalipoproteinemias, Familial; Hypoalphalipoproteinemias, Primary; Lipoproteinemia, Hypo alpha; Lipoproteinemias, Hypo alpha; Primary Hypoalphalipoproteinemia; Primary Hypoalphalipoproteinemias; alpha Lipoprotein Deficiency Disease, Familial; alpha Lipoproteinemia, Hypo; Hypoalphalipoproteinemia, Familial; Lipoprotein Deficiency Disease, HDL, Familial; alpha-Lipoprotein Deficiency Disease, Familial

Networked: 154 relevant articles (4 outcomes, 19 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hypertriglyceridemia
2.	Hyperlipidemias (Hyperlipidemia)
3.	Coronary Artery Disease (Coronary Atherosclerosis)
4.	Tangier Disease
5.	Atherosclerosis

Experts

1.	Calabresi, Laura: 8 articles (11/2013 - 02/2002)
2.	Kastelein, John J P: 7 articles (03/2015 - 10/2002)
3.	Franceschini, Guido: 7 articles (11/2013 - 02/2002)
4.	Hovingh, G Kees: 5 articles (03/2015 - 06/2003)
5.	Hayden, Michael R: 5 articles (08/2006 - 10/2002)
6.	Simonelli, Sara: 4 articles (11/2013 - 06/2011)
7.	Stroes, Erik S G: 3 articles (12/2014 - 06/2003)
8.	Wong, Norman C W: 3 articles (08/2014 - 07/2012)
9.	Haas, Michael J: 3 articles (08/2014 - 07/2012)
10.	Mooradian, Arshag D: 3 articles (08/2014 - 07/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hypoalphalipoproteinemias:

1.	HDL CholesterolIBA 02/01/2011 - "Except for isolated hypoalphalipoproteinemia (a low level of high-density lipoprotein cholesterol), therapies will start with a statin. " 11/01/2007 - "For example, this strategy showed that a small but relevant proportion of individuals within the <5% tail of plasma HDL-cholesterol distribution have mutations in genes that cause familial hypoalphalipoproteinemia. " 07/01/2007 - "Because hypoalphalipoproteinemia is highly prevalent in Mexico, we screened the ABCA1 coding sequence in Mexican individuals with low and high HDL cholesterol levels to seek functional variants. " 02/01/2004 - "The mild changes in ABCA1 activity due to genomic variation might be associated with interindividual variations in serum high-density lipoprotein cholesterol (HDL-C) and apoAI levels, or primary hypoalphalipoproteinemia in the general population. " 04/01/1995 - "Microsomal enzyme inducers raise plasma high-density lipoprotein cholesterol levels in healthy control subjects but not in patients with primary hypoalphalipoproteinemia."
2.	Lipoproteins (Lipoprotein)IBA 01/01/1989 - "The results of lipoprotein studies performed in 67 members of 10 kindreds with familial hypoalphalipoproteinemia are presented. " 11/01/2007 - "By contrast, successes in defining the molecular basis of rare single-gene disorders, such as familial hypoalphalipoproteinemia, have shown the value of experimental designs that focus on genomic analysis of individuals within the tails of Gaussian distributions of quantitative lipoprotein traits. " 04/01/2006 - "Genetic variation of PLTP modulates lipoprotein profiles in hypoalphalipoproteinemia." 11/01/1990 - "Subjects over 85 years (13%) had markedly less disturbance in lipoprotein metabolism of high atherogenic potency (hyper-beta-, hypo-alpha-lipoproteinemia) than did subjects 65-75 years old (23%). " 11/01/2013 - "Mutations in the LCAT gene leads to two rare disorders, familial LCAT deficiency and fish-eye disease, both characterized by severe hypoalphalipoproteinemia associated with several lipoprotein abnormalities. "
3.	Gemfibrozil (Lopid)FDA LinkGeneric 03/01/2000 - "To assess the effects of nicotinic acid (NA), gemfibrozil and combination therapy on the lipid profile of patients with clinical atherosclerotic disease and isolated hypoalphalipoproteinemia. " 03/01/2000 - "Gemfibrozil, nicotinic acid and combination therapy in patients with isolated hypoalphalipoproteinemia: a randomized, open-label, crossover study." 12/08/1989 - "Comparison of lovastatin and gemfibrozil in normolipidemic patients with hypoalphalipoproteinemia." 03/01/2000 - "Twenty-three patients with clinically well-defined atherosclerosis and isolated hypoalphalipoproteinemia were prospectively randomized to receive gemfibrozil, NA or combination therapy in an open-label, crossover design trial to assess the effects on serum lipids. " 04/01/1993 - "Effect of gemfibrozil and lovastatin on postprandial lipoprotein clearance in the hypoalphalipoproteinemia and hypertriglyceridemia syndrome."
4.	Triglycerides (Triacylglycerol)IBA 01/01/2014 - "Subjects (n = 5,306) were evaluated for the presence of MS; four mutually-exclusive MS groups were considered: mixed dyslipidemia (altered triglycerides and HDL-C), hypoalphalipoproteinemia: (normal triglycerides but low HDL-C), hypertriglyceridemia (elevated triglycerides and normal HDL-C) and without dyslipidemia (normal triglycerides and HDL-C). " 11/01/1983 - "Concentrations of cholesterol, triglycerides and LPHD cholesterol were normal in the majority of patients but dyslipoproteinemia of various types including hypo-alpha-lipoproteinemia, was noted in some patients. " 01/01/2002 - "Hyperlipidemia (HLE) type II was most frequent in group 1 while HLE type IV or hypoalphalipoproteinemia without rise in cholesterol or triglycerides--in groups 2 and 3. In IHD without CVI dyslipidemia in most cases was associated with one of the additional risk factors (hypertension, smoking, diabetes mellitus) while in CVI it combined with two or three additional risk factors." 04/01/1995 - "To determine whether high CETP activities are associated with isolated hypoalphalipoproteinemia, CETP activities were measured in 109 adult men with HDL cholesterol < 35 mg/dL, plasma triglycerides < 200 mg/dL, and LDL cholesterol < 160 mg/dL; the results were compared with those of 50 normolipidemic (HDL cholesterol > 40 mg/dL) male subjects. " 08/27/2004 - "Furthermore, the staggerer mice display lowered plasma apoA-I/-II, decreased plasma high density lipoprotein cholesterol and triglycerides, and develop hypo-alpha-lipoproteinemia and atherosclerosis. "
5.	LDL CholesterolIBA 02/01/2012 - "We resequenced ANGPTL3 in 4 members of 3 kindreds originally identified for very low levels of low-density lipoprotein cholesterol and high-density lipoprotein cholesterol (0.97±0.16 and 0.56±0.20 mmol/L, respectively) in whom no mutations of known candidate genes for monogenic hypobetalipoproteinemia and hypoalphalipoproteinemia had been detected. " 01/01/1994 - "Although there is consensus that lipid variables, especially lipoprotein(a), are heritable and that elevated LDL cholesterol levels should be treated, there are no clear definitions of the common familial lipid disorders associated with premature CHD (lipoprotein(a) excess, FCH, familial dyslipidemia, familial hypoalphalipoproteinemia, familial hypercholesterolemia), nor do we have clear guidelines for the treatment of most of these disorders. " 07/12/1993 - "Clinical management of dyslipidemias has focused primarily on the low-density lipoprotein cholesterol (LDL-C) fraction; however, lipid disorders accompanied by low levels of high-density lipoprotein cholesterol (HDL-C) (hypoalphalipoproteinemia) are common, particularly among subjects with the diagnosis of coronary artery disease prior to age 55 years. " 04/01/1995 - "To determine whether high CETP activities are associated with isolated hypoalphalipoproteinemia, CETP activities were measured in 109 adult men with HDL cholesterol < 35 mg/dL, plasma triglycerides < 200 mg/dL, and LDL cholesterol < 160 mg/dL; the results were compared with those of 50 normolipidemic (HDL cholesterol > 40 mg/dL) male subjects. " 11/16/1983 - "The criteria for the definition of familial hypo-alpha-lipoproteinemia are, therefore, as follows: 1) low HDL-cholesterol level in the presence of normal VLDL and LDL-cholesterol levels; 2) absence of diseases or factors to which hypo-alpha-lipoproteinemia might be secondary; 3) presence of a similar lipoprotein pattern in a first degree relative."
6.	Lovastatin (Mevacor)FDA LinkGeneric 12/08/1989 - "Comparison of lovastatin and gemfibrozil in normolipidemic patients with hypoalphalipoproteinemia." 04/01/1993 - "Effect of gemfibrozil and lovastatin on postprandial lipoprotein clearance in the hypoalphalipoproteinemia and hypertriglyceridemia syndrome." 01/10/1994 - "Lipoprotein responses to treatment with lovastatin, gemfibrozil, and nicotinic acid in normolipidemic patients with hypoalphalipoproteinemia." 04/01/1993 - "Eleven men with hypoalphalipoproteinemia (HPAL; fasting plasma high density lipoprotein (HDL) cholesterol level of < 0.9 mmol/l), mild hypertriglyceridemia (HTG; triglycerides (TG) level of 1.75-7.5 mmol/l) and a normal calculated LDL cholesterol level (< 3.7 mmol/l) participated in a randomized, double-blind, double-placebo, crossover trial to compare the effect of two drugs, lovastatin (40 mg once daily) and gemfibrozil (600 mg twice daily), on clearance of postprandial lipoproteins. "
7.	avasimibeIBA 07/01/2001 - "The safety and efficacy of avasimibe (CI-1011), a new, unique, wholly synthetic ACAT inhibitor, was evaluated in the treatment of 130 men and women with combined hyperlipidemia and hypoalphalipoproteinemia (low levels of high-density lipoprotein cholesterol [HDL-C]). " 01/01/2003 - "Clinical data are scarce, but in a study performed in 130 men and women with combined hyperlipidemia and hypoalphalipoproteinemia, avasimibe, 50-500 mg/day, significantly reduced plasma total triglyceride and VLDL-cholesterol. "
8.	ChylomicronsIBA 10/01/1992 - "Delayed clearance of postprandial chylomicrons and their remnants in the hypoalphalipoproteinemia and mild hypertriglyceridemia syndrome."
9.	BezafibrateIBA 08/01/2001 - "Improvement in endothelial dysfunction in patients with hypoalphalipoproteinemia and coronary artery disease treated with bezafibrate."
10.	CholesterolIBA 09/02/2011 - "These results provide further evidence that the rate of release of cholesterol from late endosomes/lysosomes is a critical regulator of ABCA1 expression and activity, and an explanation for the hypoalphalipoproteinemia seen in CESD patients." 06/01/2003 - "The role of the ABCA1 transporter and cholesterol efflux in familial hypoalphalipoproteinemia." 02/01/2002 - "Fifty-five subjects with primary hypoalphalipoproteinemia (HypoALP) or hyperalphalipoproteinemia (HyperALP) were compared with fifty-five control subjects with average HDL-C levels, matched for sex, age, and plasma cholesterol. " 10/01/2000 - "Decreased cellular cholesterol efflux is a common cause of familial hypoalphalipoproteinemia: role of the ABCA1 gene mutations." 04/01/1995 - "These findings indicate that microsomal enzyme inducers are unsuitable to increase plasma HDL levels in high-risk patients with primary hypoalphalipoproteinemia, and they disclose a new mechanism, that is, decreased CETP-mediated transfer of cholesterol out of HDL, for the HDL-raising effect of microsomal enzyme inducers in healthy individuals."

Therapies and Procedures

1.	Art Therapy 01/01/2008 - "One hundred five infected patients, randomly selected from a Mexican HIV clinic, and 105 age- and gender-matched noninfected community volunteers, were enrolled to study the prevalence of each of three highly atherogenic lipoprotein phenotypes [high apolipoprotein (Apo)B/ApoA-I ratio, hypertriglyceridemia with high ApoB and hypoalphalipoproteinemia with high ApoB], and the relationship between time of exposure to antiretroviral therapy (ART) drug class and lipid changes. "
2.	Drug Therapy (Chemotherapy) 07/12/1993 - "Pharmacotherapy selected according to the dyslipidemia that accompanies the HDL-C disorder is indicated for subjects who manifest premature coronary artery disease or who have a familial history of coronary artery disease and hypoalphalipoproteinemia." 05/27/1996 - "Nicotinic acid may be useful in combined drug therapy for secondary prevention of coronary heart disease, and if higher doses cannot be tolerated, use of a lower dose should still be useful for producing a moderate rise in HDL-C levels in patients with hypoalphalipoproteinemia."
3.	Kidney Transplantation 05/01/2012 - "After renal transplantation, proband's lipid profile, HDL subclass distribution, and plasma cholesterol esterification were almost at normal levels, suggesting a mild contribution of the LCAT P406L variant to his pretransplantation severe hypoalphalipoproteinemia and impairment of plasma cholesterol esterification. "