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1 284
Repost from 𝗡𝗲𝗿𝗱𝘀 8
-Incidence increase with high blood concentrations of cholesterol, especially with high levels of LDL
-Decrease with high HDL
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Repost from 𝗡𝗲𝗿𝗱𝘀 8
"Some diseases in chapter lipid metabolism "
🔶 A peta lipoprotenemia
🔸 autusomal resesive diseases
🔸 caracterized by fat malabsorption
🔸 results from mutations in the gene encoding the large subunit of MTTP.
🔸 complete absence of plasma apo B–containing lipoproteins, namely CMs, VLDL, and LDL.
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🔶 Lipid Malabsorption (Steatorrhea)
🔸 due to: a defect in pancreatic lipase secretion, bile salt release, or intestinal mucosal cells.
🔸 Symptoms can be reduced by limiting the dietary lipid intake, but this could lead to specific deficiency syndromes due to impaired absorption of lipid-soluble vitamins.
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🔶Dicarboxylic acid urea
🔸 Deficiency in mitochondrial medium-chain acyl-CoA dehydrogenas
🔸 Characterized by excretion of C6-C10 w-dicarboxylic acids and by nonketogenic hypoglycemia
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🔶 Jamaican Vomiting Sickness
🔸 Caused by eating the unripe fruit of akee tree which contains the toxin hypoglycine, that inhibits activity of acyl- CoA dehydrogenase leading to inhibition of b-oxidation and hypoglycemia.
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🔶sphingolipidosis
🔸 inherited disease, causes defect of lysosomal enzymes that degrad sphingolipids
🔸 farber’s disease —->⬇️ ceramidase
🔸 fabry’s disease —->⬇️ alpha galactosidase
🔸Krabb’s disease —->⬇️ beta galactosidase
🔸 Gaucher’s disease —->⬇️ beta glucosidase
🔸metachromatic leukodystrophy disease—->⬇️ acyl sulfatase A
🔸Niemann pick disease —->⬇️ sphingomyelinase
🔸 Tay sachs disease —->⬇️ Hexosaminidase A
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🔶 problems from cholesterol
🔸Obesity and Gall stone, and atherosclerosis and cardiovascular disease
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🔶Type 1 Hyperlipidemia
🔸 Elevated blood concentrations of chylomicrons.
🔸 Deficiency in ApoC-II and Deficiency in lipoprotein lipase
🔸 Symptoms:• Plasma triacylglycerol levels > 1000 mg/dL and Eruptive xanthomas and pancreatitis
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🔶 Tangiers Disease and Familial HDL Deficiency
🔸 defects in the gene encoding the ABC1 protein.
🔸 Cholesterol-depleted HDL is rapidly removed from the circulation so plasma HDL levels are 1-5% of normal
🔸 Patients accumulate cholesterol in the lymphoreticular system (hepatomegaly and spleenomegaly).
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🔶 Familial Hypercholesterolemia
🔸 Genetic disease that arises from any one of many mutant alleles for the LDL receptor gene.
🔸 • LDL uptake into cells is defective, which results in cholesterol accumulation in the blood.
• Homozygotes: 680 mg/dL (atherosclerosis in childhood)
• Heterozygotes: 300 mg/dL (atheroclerosis in middle age)
• Healthy Level: 175 mg/d
🔸 Treated with inhibitors of de novo cholesterol biosynthesis and Cholestyramine and statin to stop HMG COA reductase
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🔶Lipoprotein a or Lp (a)
🔸Lp (a) is structurally releated to LDL because both lipoproteins
possess one molecule of apo B-100 per particle with similar lipid
compositions. Unlike LDL, Lp(a) also contains a carbohydrate-rich
protein [apo(a)], which is covalently bound to the apo B-100 through
a disulfide linkage.
🔸 structurally homologous to plasminogen, the precursor of a
blood protease whose target is fibrin, the main protein component of
blood clots.
🔸It is hypothesized that ⬆️ Lp(a) slows the breakdown of blood
clots that trigger heart attacks because it competes with plasminogen
for binding to fibrin.
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🔶 diseases and cholesterol
🔸 cholecystolithiasis (gallstone in the gallbladder),
🔸choledocholithiasis (gallstone in a bile duct.
🔸 Cholelithiasis —->
-Cholesterol-gallstone formation results from an imbalance in cholesterol metabolism, which involves hepatic biosynthesis, intestinal absorption, formation of bile salts, and biliary excretion of cholesterol.
-Risk factors: obesity, type 2 diabetes mellitus, hyperinsulinemia, and dyslipidemia.
-The solubility of cholesterol in bile depends on the relative proportion of bile salts, phosphatidylcholine, and cholesterol itself.
🔸 Atherosclerosis —->
-Obstruction of blood vessels due to pathological accumulation of cholesterol
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⭕️ Lipid metabolism
⭕️ Amino acid metabolism
⭕️ Integration
⭕️ Bioenergatics
⭕️ Carbohydrates metabolism
⭕️ Hormone
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✅Remember
الي ياخد في cholesterol من tissues membrane ويرده للHDL اسمه
ATP binding cassette transporter(ABCA1)
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Which of the following statements is not correct about cholesterol biosynthesis
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Repost from N/a
Which of the following not need as co factor for acetyl co A carpoxylase
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Repost from N/a
Acetyl CoA produced by fatty acid oxidation cannot reuse it in fatty acid synthesis
