32 g with pancreatic exocrine insufficiency. Intraluminal maldigestion, mucosal malabsorption, or lymphatic obstruction may produce steatorrhea"-:Harrison 17th/250Hepatic Lipase Deficiency Harrison 17th/2421HL is a member of the same gene family as LPL and hydrolyzes triglycerides and phospholipids in remnant lipoproteins and HDLs. HL deficiency is a very rare autosomal recessive disorder characterized by elevated plasma levels of cholesterol and triglycerides (mixed hyperlipidemia) Table : Harrison 17th/2419 Frederickson Classification of HyperlipoproteinemiasPhenotypeIIIaIIbIIIIVVLipoprotein, elevatedChylomicronsLDLLDL and VLDLChylomicron and VLDL remnantsVLDLChylomicrons and VLDLTriglycerides++++--++++ to +++++++++Cholesterol+ to +++++++ to +++++ to +++-- to +++ to +++LDL-cholesterol HDL-cholesterol+++++++++++++Plasma appearanceLactescentClearClearTurbidTurbidLactescentXanthomasEruptiveTendon, tuberousNonePalmar, tuberoerup- tiveNoneEruptivePancreatitis+++0000+++Coronary athe rosclerosis0++++++++++/-+/-Peripheral atherosclerosis0+++ ++/-+/-Molecular defectsLPL and apoC-IILDL receptor, ApoB- 100, PCSK9, ARH, ABCG5 and ABCG8UnknownApoEApoA-V and UnknownApoA-V and UnknownGenetic nomenclatureFCSFH, FDB. ADH, ARH, sitosteroiemiaFCHLFDBLFHTGFHTGNote: LPL, lipoprotein lipase; apo. apolipoprotein; PCS, familial chylomicrortemia syndrome. FH. familial hypercholesterolemia: FDB. familial defective apoB: ARH. aulosomai recessive hypercholesterolemia; ADH, autosomal dominant hypercholesterolemia; FCHL. familial combined HLAlso Remember:Triacylglycerol undergoes hydrolysis by a hormone-sensitive lipase to form free fatty acids and glycerol. This lipase is distinct from lipoprotein lipase, which catalyzes lipoprotein triacylglycerol hydrolysis before its uptake into extrahepatic tissues.Hormone-sensitive lipase is activated by ACTH, TSH, glucagon, epinephrine, norepinephrine, and vasopressin and inhibited by insulin, prostaglandin PS, and nicotinic acid,Lingual lipase in saliva,Gastric lipase in gastric juice.Pancreatic lipase in pancreatic juice.Intestinal lipase in intestinal epithelial cell,Adipolytic lipase in adipose tissue, andSerum lipase.Pancreatic Lipase#Pancreatic lipase is essential for absorption of dietary fats in small intestine-Warper 28th/460#It deficiency definciency cause Steatorrhea, but hypertriglyuceridemia is unlikelyAcetyl Co A carboxylase is enzyme for lipogenesisQ- Harper 28th/196"Succinate thiokinase is a enzyme of TCA cycle"- Harper 28th/145Serum Lipase is marker of acute pancreatitis- Harper 28th/59">