A patient with abetalipoproteinemia frequently manifests with delated blood clotting. This is due to inability to
Type-III: Familial Dys-Beta Lipoproteinaemia:Synonyms: Broad Beta disease, 'Remnant' removal diseaseThe disease is characterised by:Increase in b-lipoproteins (LDL |)Increase in Pre-b-lipoproteins (VLDL |);Actually rise is in IDL (VLDL remnant). This appears as broad b-band ("floating" b-band)-b- VLDL on electrophoresis. Also, there is hypercholesterolemia and hyperiglyceridemia.Inheritance: Autosomal dominant.Metabolic defects:Increased concentration of apo-E |.Increased synthesis of apo-B |.Conversion of normal VLDL to b-VLDL (IDL) and its degradation without conversion to LDL. Defect is in "Remnant" metabolism.Clinical feature: Presents with Xanthomastuberous and palmar Xanthomas. Premature cardiovascular diseases and atherosclerosis common. Also prone to develop peripheral vascular diseases.Treatment:By weight reductionLow carbohydrate diets, containing unsaturated fats and little cholesterolRef: Textbook of Medical Biochemistry, 8th Edition, Dr (Brig) MN Chatterjea, Rana Shinde, page no: 452,453
Produce VLDL
Synthesize fatty acids
Produce chylomicrons
Synthesize clotting factors
Metabolism of lipid