AnatomyDiseasesChemicals and DrugsInformation Science
AbetalipoproteinemiaAcanthocytesLipoproteinsLipoproteins, LDLLipoproteins, HDLApolipoproteins BHypobetalipoproteinemiasHypolipoproteinemiasLipoproteins, VLDLApolipoproteinsTriglyceridesLipoprotein LipaseCholesterolLipoprotein(a)Carrier ProteinsEncyclopedias as TopicBody Water
High-density lipVLDLMetabolismLipaseBeta-lipoproteinsChylomicronsDeficiencySigns and symptoms of abetalipoproteinemiaTriglyceridesAcanthocytosisDisorderLipidsLipid profileDensity lipoproteinMutationsAutosomalParticlesApolipoproteinsAtherosclerotic cardiovascular diseaseGenesBiological membranesTriglycerideAtherosclerosisAtherogenicFatsGenePlasmaElevationsHereditaryInfancyCholesterol estersLevelsAcanthocytesAccumulationIndividualsPrimaryFree
High-density lip9
  • The 4 major lipoproteins are very low-density lipoprotein (VLDL), low-density lipoprotein (LDL), high-density lipoprotein (HDL), and chylomicrons (CMs). (medscape.com)
  • This finding of a low high-density lipoprotein (HDL) cholesterol level can be useful as an independent factor in assessing coronary artery disease (CAD) risk and further management. (medscape.com)
  • Currently, clinical trial results suggest that raising high-density lipoprotein (HDL) levels reduces risk. (medscape.com)
  • In addition, they sometimes lower triglyceride (TG) levels and may modestly elevate high-density lipoprotein (HDL) cholesterol levels. (medscape.com)
  • High-density lipoprotein (HDL) cholesterol is used in the assessment of coronary or other vascular pathology risk. (medscape.com)
  • The reference range of high-density lipoprotein cholesterol (HDL-C) is 40-50 mg/dL in men and 50-60 mg/dL in women. (medscape.com)
  • High-density lipoprotein cholesterol (HDL-C), which consists mostly of cholesterol, phospholipid, and protein, is produced and secreted by the liver and intestine. (medscape.com)
  • In the latter setting, an abnormal high-density lipoprotein binds to receptors on the outer surface of the red cell membrane and induces the characteristic conformational changes. (forwardonclimate.org)
  • however, an increase in high-density lipoprotein ( HDL ) has a positive effect on the vessels. (amboss.com)
VLDL8
  • ABL is a rare disease associated with a unique plasma lipoprotein profile in which LDL and very low-density lipoprotein (VLDL) are essentially absent. (medscape.com)
  • 20%), the LDL goal is under 100 mg/dL, and the goal for non-HDL cholesterol (LDL plus very-low-density lipoprotein [VLDL]) is below 130 mg/dL. (medscape.com)
  • Lipoprotein disorders can be described as abnormalities in the level of the lipids, which include cholesterol and triglycerides, or as abnormalities in the levels of lipoproteins that include LDL , HDL, VLDL and chylomicrons . (wikidoc.org)
  • In abetalipoproteinemia (Bassen-Kornzweig syndrome), virtually no LDL cholesterol is present, and the body cannot make chylomicrons and very-low-density lipoproteins (VLDL). (msdmanuals.com)
  • These triglycerides are transported in association with β-lipoproteins in very-low-density lipoproteins (VLDL). (unboundmedicine.com)
  • We found that tPA, partially through the lysine-binding site on its Kringle 2 domain, binds to the N terminus of apoB, blocking the interaction between apoB and microsomal triglyceride transfer protein (MTP) in hepatocytes, thereby reducing very-low-density lipoprotein (VLDL) assembly and plasma apoB-lipoprotein cholesterol levels. (bvsalud.org)
  • Humans with PAI-1 deficiency have smaller VLDL particles and lower plasma levels of apoB-lipoprotein cholesterol. (bvsalud.org)
  • Familial hypertriglyceridemia (type IV familial dyslipidemia) is a genetic disorder characterized by the liver overproducing very-low-density lipoproteins (VLDL). (mdwiki.org)
Metabolism6
  • The handling of lipoprotein particles in the body is referred to as lipoprotein particle metabolism. (wikipedia.org)
  • Abetalipoproteinemia (ABL) and familial hypobetalipoproteinemia (FHBL) are relatively uncommon inherited disorders of lipoprotein metabolism that cause low cholesterol levels. (medscape.com)
  • these apolipoproteins have lipid-soluble segments, the beta apolipoproteins, which remain part of the lipoprotein throughout its metabolism. (medscape.com)
  • Other apolipoproteins (A, C, D, E, and their subtypes) are soluble and are exchanged between lipoproteins during metabolism. (medscape.com)
  • [ 1 ] which is an autosomal recessive abnormality of lipoprotein metabolism resulting in ataxia combined with acanthocytosis. (medscape.com)
  • These lipoproteins control fat metabolism and have different proportions of bound fat as well as different functions. (amboss.com)
Lipase5
  • We considered 3 possible causes of an abetalipoproteinemia-like lipid profile in AN: (1) depletion of hepatic substrate for apolipoprotein B synthesis, (2) lack of exogenous fatty acids with exhaustion of endogenous stores of triglycerides in adipose tissue, and (3) preservation of the lipoprotein lipase (LPL) mass. (elsevierpure.com)
  • This agent increases plasma catabolism and the clearance of TG-rich particles by lipoprotein lipase induction and the suppression of the hepatic production of apo C-III through the activation of PPARs. (medscape.com)
  • Triglycerides in the chylomicrons are cleared from the blood by tissue lipoprotein lipase. (unboundmedicine.com)
  • Individuals with the disorder are mostly heterozygous in an inactivating mutation of the gene encoding for lipoprotein lipase (LPL). (mdwiki.org)
  • Inactivity of lipoprotein lipase (LPL) plays the predominant role in the development of familial hypertriglyceridemia. (mdwiki.org)
Beta-lipoproteins4
  • Most MTTP gene mutations lead to the production of microsomal triglyceride transfer protein with reduced or absent function and unable to help in the formation of beta-lipoproteins. (medlineplus.gov)
  • The MTTP gene gives the body instructions to make a protein needed for creating beta-lipoproteins. (rareguru.com)
  • Mutations in the MTTP result in a lack of beta-lipoproteins, leading to an inability to absorb and transport these substances. (rareguru.com)
  • characterized by severe deficiency of beta-lipoproteins and abnormal red blood cells (acanthocytes) and abnormally low cholesterol levels. (wordinn.com)
Chylomicrons2
  • Plasma phospholipid levels are very low, while plasma apolipoprotein B, chylomicrons, very-low-density lipoproteins (VLDLs), and low-density lipoproteins (LDLs) are absent. (medscape.com)
  • In addition, low blood levels of vitamin E may be associated with abetalipoproteinemia, presumably due to a lack of the ability to form very low-density lipoproteins and chylomicrons in the intestinal absorptive cells of affected persons. (testcatalog.org)
Deficiency2
  • Therefore, it is apparent that diseases linked to VE deficiency will only occur in association with infrequent syndromes of impairment of VE intake and its maintenance, such as abetalipoproteinaemia or the familial isolated VE deficiency syndrome. (nature.com)
  • Abetalipoproteinemia is a congenital lipid disorder in which a deficiency of apolipoproteins (hypolipoproteinemia) leads to impaired intestinal absorption of fats and fat-soluble vitamins . (amboss.com)
Signs and symptoms of abetalipoproteinemia2
Triglycerides7
  • The role of lipoprotein particles is to transport fat molecules, such as triglycerides, phospholipids, and cholesterol within the extracellular water of the body to all the cells and tissues of the body. (wikipedia.org)
  • The interaction of these apolipoproteins with enzymes in the blood, with each other, or with specific proteins on the surfaces of cells, determines whether triglycerides and cholesterol will be added to or removed from the lipoprotein transport particles. (wikipedia.org)
  • Characterization in human plasma Lipoproteins are complex particles that have a central hydrophobic core of non-polar lipids, primarily cholesteryl esters and triglycerides. (wikipedia.org)
  • It is divided into two pathways, exogenous and endogenous, depending in large part on whether the lipoprotein particles in question are composed chiefly of dietary (exogenous) lipids or whether they originated in the liver (endogenous), through de novo synthesis of triglycerides. (wikipedia.org)
  • While adipocytes are the main storage cells for triglycerides, they do not produce any lipoproteins. (wikipedia.org)
  • Cholesterol and triglycerides are transported from sites of synthesis to sites of utilization in the form of lipoproteins. (medscape.com)
  • They are generally characterized by elevated levels of cholesterol , triglycerides , and/or lipoproteins in the blood, which are often associated with atherosclerotic cardiovascular disease ( ASCVD ). (amboss.com)
Acanthocytosis2
  • When it was originally described, it was also frequently compared with Bassen-Kornzweig disease in that both combined neurologic abnormalities with acanthocytosis, but the Levine-Critchley syndrome had normal lipoproteins as well as a later age of onset. (medscape.com)
  • We report the case of a 17-year-old woman with anorexia nervosa (AN) who developed an abetalipoproteinemia-like lipid profile and acanthocytosis. (elsevierpure.com)
Disorder3
  • Abetalipoproteinemia is an inherited disorder that impairs the normal absorption of fats and certain vitamins from the diet. (medlineplus.gov)
  • Abetalipoproteinemia is a rare disorder. (medlineplus.gov)
  • Acanthocytes are found in 50-90% of cells on peripheral blood smear findings in abetalipoproteinemia, which is a rare autosomal recessive disorder with only about 100 cases described worldwide. (forwardonclimate.org)
Lipids5
  • Lipoproteins are aggregates of proteins and lipids that facilitate the circulation of hydrophobic lipids in the body. (wikidoc.org)
  • Hyperlipoproteinemias generally cause elevations in the affected lipids/lipoproteins and hypolipoproteinemia s generally cause reductions in the affected lipids/lipoproteins. (wikidoc.org)
  • Notably, human genetic variants in the MIR541, ZNF101, and CASZ1 loci are significantly associated with plasma lipids and lipoprotein levels. (bvsalud.org)
  • In the maternal circulation, apoB-containing low-density lipoproteins (LDL) and apoA1-containing high-density lipoproteins (HDL) transport lipids. (bvsalud.org)
  • Lipids circulating in the bloodstream are transported in lipoproteins (contain hydrophilic apolipoproteins ) because the hydrophobic lipids are insoluble in plasma. (amboss.com)
Lipid profile1
  • [2] This is to say that in cases of mixed (elevated and decreased) findings on the lipid profile , clinicians must evaluate the secondary causes of the dysipidemia and manage the affected lipoproteins accordingly. (wikidoc.org)
Density lipoprotein6
  • Reducing low-density lipoprotein (LDL) levels is the primary goal. (medscape.com)
  • According to NCEP ATP III guidelines, the primary goal of therapy is to lower low-density lipoprotein (LDL) cholesterol levels. (medscape.com)
  • These medications usually lower low-density lipoprotein (LDL) cholesterol levels. (medscape.com)
  • Beta-lipoprotein cholesterol is a synonym for low-density lipoprotein (LDL) cholesterol. (rareguru.com)
  • OBJECTIVE: To identify a genetic cause of hypobetalipoproteinemia in a patient with levels of low density lipoprotein cholesterol at the detection limit of 0.1 mmol/l. (bvsalud.org)
  • The major underlying defect in FH is mutations of the low-density lipoprotein (LDL) receptor gene i.e., mutations in the pro-protein convert as subtilisin/ kexin type 9 gene and mutations in the apolipoprotein B100 gene 4 . (ijpsr.com)
Mutations4
  • Abetalipoproteinemia is caused by mutations in the MTTP gene, which provides instructions for making a protein called microsomal triglyceride transfer protein. (medlineplus.gov)
  • Most of the symptoms are due to defects in the absorption and transport of vitamin E . Abetalipoproteinemia is caused by mutations in the MTTP gene and is inherited in an autosomal recessive manner. (rareguru.com)
  • Abetalipoproteinemia is caused by changes ( mutations ) in the MTTP gene. (rareguru.com)
  • Mutations in MTP can cause abetalipoproteinemia. (utsouthwestern.edu)
Autosomal2
  • Abetalipoproteinemia is inherited in an autosomal recessive manner. (rareguru.com)
  • Familial hypercholesterolemia (FH) is an autosomal-dominant condition that is characterized by elevated levels of plasma LDL cholesterol and apo-lipoprotein B resulting in an increased risk for atherosclerosis 1 . (ijpsr.com)
Particles3
Apolipoproteins1
Atherosclerotic cardiovascular disease1
  • Apolipoprotein B (apoB)-lipoproteins initiate and promote atherosclerotic cardiovascular disease. (bvsalud.org)
Genes1
  • The hepatic knockdown of orthologous Zfp961 and Casz1 genes in mice altered plasma lipoproteins and reduced atherosclerosis without causing hepatic lipid accumulation, most likely by lowering hepatic triglyceride production, increasing HDL cholesterol efflux capacity, and reducing lipogenesis. (bvsalud.org)
Biological membranes2
  • Detergents are usually required to isolate transmembrane lipoproteins from their associated biological membranes. (wikipedia.org)
  • One of these is vitamin E (VE), an antioxidant and an essential component of biological membranes and circulating lipoproteins. (nature.com)
Triglyceride1
  • Protein disulfide isomerase (PDI) completes the heterodimeric microsomal triglyceride transfer protein, which has been shown to play a central role in lipoprotein assembly. (utsouthwestern.edu)
Atherosclerosis2
  • High apoB-containing low-density lipoproteins (LDL) and low apoA1-containing high-density lipoproteins (HDL) are associated with atherosclerosis. (bvsalud.org)
  • This study identifies miR-541-3p and Znf101/Casz1 as potential therapeutic agent and targets, respectively, to reduce plasma lipoproteins and atherosclerosis without causing liver steatosis. (bvsalud.org)
Atherogenic1
  • Plasma tissue plasminogen activator (tPA) activity is negatively associated with atherogenic apoB-lipoprotein cholesterol levels in humans, but the mechanisms are unknown. (bvsalud.org)
Fats2
  • Abetalipoproteinemia is a condition characterized by the inability to fully absorb dietary fats, cholesterol and fat-soluble vitamins. (rareguru.com)
  • These lipoproteins are necessary for the body to absorb fats, cholesterol, and fat-soluble vitamins (vitamins A, D, E and K), and for transporting these substances in the blood. (rareguru.com)
Gene1
  • Hyperlipidemia resulting from single-gene disorders , e.g., familial hypercholesterolemia , can cause severe elevations in lipoprotein levels and early atherosclerotic complications. (amboss.com)
Plasma3
  • A lipoprotein is a biochemical assembly whose primary function is to transport hydrophobic lipid (also known as fat) molecules in water, as in blood plasma or other extracellular fluids. (wikipedia.org)
  • In abetalipoproteinemia, plasma cholesterol levels are very low, less than 50 mg/dL. (medscape.com)
  • Heterozygous mutant mice display altered plasma lipid and lipoprotein profiles. (utsouthwestern.edu)
Elevations1
  • Secondary dyslipidemias, on the other hand, may cause elevations in some lipoproteins and reductions in others. (wikidoc.org)
Hereditary1
  • A few rare hereditary disorders, such as abetalipoproteinemia and hypoalphalipoproteinemia, result in lipid levels low enough to have serious consequences. (msdmanuals.com)
Infancy1
  • Abetalipoproteinemia typically presents in infancy with failure to thrive, diarrhea, vomiting, and malabsorption of fat. (nih.gov)
Cholesterol esters1
Levels2
  • On the other hand, given the relatively narrow margin between minimal and maximal VE levels in circulating lipoproteins and in tissues, there is only a limited scope for VE manipulation by intervention therapy. (nature.com)
  • Lipoprotein disorders must be initially classified broadly into hypolipidemias and hyperlipidemias corresponding to low or high lipid levels, respectively. (wikidoc.org)
Acanthocytes1
Accumulation1
Individuals1
Primary1
Free1