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Block 6 amino products, Heme and Bilirubin

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Question
Answer
Three specialized products derived from tyrosine?   Thyroid hormones: T3, T4  
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Two specialized products derived from Tryptophan?   Serotonin, Niacin (NAD, NADP)  
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Two specialized products derived from Arginine?   Creatine (arginine + glycine) & Nitric Oxide  
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Two specialized products derived from Glutamate?   Glutamate as a neurotransmitter & GABA  
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One specialized product of Histidine?   Histamine  
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What three amino acids are essential in the synthesis of purines or pyrimdines?   glycine, glutamine, aspartate  
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One specialized product that uses the entire structure of glycine?   Heme  
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Where does Heme synthesis occur?   liver  
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What are some heme-containing proteins?   hemoglobin, myoglobin, cytochromes (P450, ETC)  
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What are some heme-containing enzymes?   peroxidases, catalases  
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What 2 enzymes does lead poisoning inhibit?   ALA dehydratase & Ferrochelatase  
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What enzyme combines succinyl-CoA and glycine to start the Heme pathway?   ALA (aminolevulinic acid) synthetase  
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What is porphyria?   rare enzyme deficiency in the heme pathway  
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Why does skin begin chronic inflammation and blistering with porphyria?   UV-light converts accumulated intermeadiates from the Heme pathway  
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What is the difference in the concentration of ALA in Vitamin B6 deficiency and lead poisoning?   Ala decreased in B6, increased in Lead poisoning  
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From where does bilirubin originate?   older RBCs lysed in spleen, heme released and converted to bilirubin by spleenic macrophages  
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How does bilirubin travel from the liver to the gall bladder?   It is insoluble so it rides albumin  
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What is a way the hepatocytes make bilirubin more soluble?   conjugate with glucuronic acid, in a UDP-glucuronate...conjugated bilirubin or bilirubin glucuronate  
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What does bilirubin contribute to the gall bladder, it's storage site?   green color is due to bilirubin glucuronate  
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What makes poop brown?   bilirubin glucuronate conveted to urobilinogen which becomes sterocobilin (stands for brown sheete)  
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How could a big pile of feces indicate you had a bile duct blockage?   it would be clay colored  
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What makes urine yellow?   Lemonade...or a portion of urobilinogen converted to urobilin and excreted in urine  
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Summarize what makes the following colors? Brown Feces, Green Bile, Yellow urine   urobilinogen to stercobilin in feces billirubin glucuronate in gall bladder urobilinogen to urobilin in urine  
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You can indirectly measure bilirubin by checking the level of:   unconjugated bilirubin  
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What conditions increase indirect/insoluble bilirubin?   Hemolysis from sickle cell, thalassmia, or G6PDH; Crigler - Najar; Gilberts syndrome; Low levels of UDP-Glucuronate in newborn  
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What conditions increase direct/soluble bilirubin?   Hepatic damage that results in failure to secrete conjugated bilirubin into bile duct and/or bile duct obstruction  
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What causes Jaundice?   increase in indirect or direct bilirubin, or both.  
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What is the problem with accumulation of indirect, lipid soluble billirubin?   crosses blood brain barrier to cause death  
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Why does conjugated bilirubin not act in this manner?   It is secreted in the urine  
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What is total bilirubin?   unconjugated + conjugated bilirubin  
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What are three examples of hemolytic crisis?   episodes of hemolysis in G6PDH deficiency Sickle Cell crisis Thalassemia  
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What happens in Crigler-Najar syndrome   genetic defect in UDP-glucuronyl Transferase  
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What is a symptom of the less severe Gilbert syndrome   inferior sclera is faint yellow  
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If you damage your liver through alcoholism, what enzymes are increased in the blood?   AST is greater than ALT  
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What increases in viral Hepatitis in the blood?   ALT is greater than AST  
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