lipoprotein lipase deficiency


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lipoprotein lipase deficiency

An AR condition characterized by lack of lipoprotein lipase, resulting in massive hypertriglyceridemia of neonatal onset and recurrent episodes of pancreatitis Clinical Fatty food intolerance, eruptive xanthomas, hepatosplenomegaly that regresses with dietary control

lipoprotein

any of the macromolecular complexes that are the form in which lipids are transported in the blood. They consist of a core of hydrophobic lipids covered by a layer of phospholipids and apoproteins, which make the complex water-soluble. There are four main classes of lipoproteins: chylomicrons, in which lipids are transported after a meal from the intestine to tissues, where they are stored or used; very low density lipoproteins (VLDL); low density lipoproteins (LDL); and high density lipoproteins (HDL). VLDL and HDL are produced by both the liver and the intestine; LDL is produced by the metabolism of VLDL.

α-lipoprotein
high density lipoproteins which migrate in the alpha position in paper chromatography. Inherited deficiency of these proteins is described in humans but there is no known animal model of the disease.
lipoprotein factor Xa inhibitor
a blood coagulation inhibitor present in the low density lipoprotein fraction of plasma.
high density lipoprotein (HDL)
a fraction of lipoproteins separable by ultracentrifugation.
intermediate density lipoprotein (IDL)
intermediate in density between LDL and VLDL; migrate in electrophoresis with β-globulins.
lipoprotein lipase
specific lipase hydrolyzing lipoproteins.
lipoprotein lipase deficiency
References in periodicals archive ?
Management of patients with severe hypertriglyceridaemia during pregnancy: report of two cases with familial lipoprotein lipase deficiency.
Hyperinsulinemia and abdominal obesity affect the expression of hypertriglyceridemia in heterozygous familial lipoprotein lipase deficiency.
Detection and characterisation of the heterozygous state of lipoprotein lipase deficiency.
uniQure announced today it has received approval from the European Commission for the gene therapy Glybera (alipogene tiparvovec), a treatment for patients with lipoprotein lipase deficiency (LPLD, also called familial hyperchylomicronemia) suffering from recurring acute pancreatitis.
The company's lead product Glybera, a gene therapy for lipoprotein lipase deficiency (LPLD), is currently under review by the European Medicines Agency (EMA).
Glybera is a gene therapy for the genetic disorder lipoprotein lipase deficiency.
The company's lead product Glybera(R), a gene therapy for lipoprotein lipase deficiency (LPLD), is currently under review by the European Medicines Agency (EMA).
Amsterdam Molecular Therapeutics (Euronext: AMT), a leader in the field of human gene therapy, today released data showing that its gene therapy Glybera (alipogene tiparvovec) significantly reduces the risk of pancreatitis in patients with Lipoprotein Lipase Deficiency (LPLD).
AMT in 2010 submitted a Marketing Authorization Application (MAA) for their lead product GLYBERA, a gene therapy for the treatment of lipoprotein lipase deficiency (LPLD), to the European Medicines Agency (EMA, formerly known as EMEA).
Amsterdam Molecular Therapeutics (Euronext: AMT), a leader in the field of human gene therapy, today announced that it has received an opinion on its Marketing Authorisation Application (MAA) for Glybera (alipogene tiparvovec) as a potential therapy for Lipoprotein Lipase Deficiency ("LPLD").
The company's lead product Glybera(R), a gene therapy for the treatment of lipoprotein lipase deficiency (LPLD), is currently under review by the European Medicines Agency (EMA).

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