Medical Biochemistry

•Name the five classes of steroid hormones (glucocorticoids, mineralocorticoids, androgens, estrogens and progestins) •Discuss steroid hormone synthesis related to STAR and desmolase (CYP11A) •Describe the steroid hormones synthesized and released from the adrenal cortex (specified for zona fasciculata, z. glomerulosa and z. reticularis) •Indicate the synthesis and the metabolic actions of cortisol •Indicate the synthesis and the metabolic actions of aldosterone •Indicate the sy

•Outline the catabolic pathway of the following amino acids: –Phenylalanine and tyrosine and associated disorders •Phenylketonuria(Classic-PKU I and tetrahydrobiopterindeficiency –PKU II, maternal PKU), Alkaptonuria, Tyrosinosis –Branched chain amino acids •Maple syrup urine disease •Methylmalonicaciduria –Methionineand cysteine •Homocystinuria •Outline the two possible fates of homocysteine •For each of the disorders listed above –Specify the enzyme deficient and coenzyme requ

Objectives: i.Explain metabolic homeostasis and describe the mechanisms involved in the inter-tissue integration required for metabolic homeostasis ii.Differentiate between the postprandial phase, the post-absorptive phase and the early phase of starvation. iii.Explain the special role of glucose in metabolic homeostasis iv.Describe in general the pathways leading to fatty acid synthesis and cholesterol synthesis in the fed state starting with glucose v.Describe the roles of insulin and glu

–Specify the one-carbon donors in metabolism and the groups they donate (SAM, THF, Cobalamin) –Explain the formation of SAM and the reactions requiring SAM –Analyze the metabolism of homocysteine, vitamins/ coenzymes required for metabolism & interpret the clinical significance of homocysteine –Explain the formation of THF from folate and specify the mechanism of action of their inhibitors –Summarize the formation of one carbon groups from amino acid metabolism and the utilization of 1-C gro

•Identify the differences between N-base, nucleoside and nucleotide with examples •Regarding purinebiosynthesis –Enumerate the C & N donors of the purinering (amino acids and 1-C groups) –Outline purinebiosynthesis –Discuss the regulation of purinebiosynthesis (Identify the regulatory enzyme and positive and negative modulators) –Correlate the mechanism of action of sulfadrugs, trimethoprim, methotrexate, mycophenolicacid to their clinical application –Explain the formation of deoxyribonuc

–Enumerate the donors of C & N atoms to pyrimidine ring –Outline pyrimidine biosynthesis (UTP & CTP). Distinguish purine and pyrimidine biosynthetic pathways –Summarize the regulation of pyrimidine biosynthesis (regulatory enzymes, regulators) –Indicate the formation of deoxyribonucleotides from ribonucleotides –Explain the synthesis of dTMP –Indicate the mechanism of action of 5 fluoro uracil and methotrexate and their clinical application –Regarding orotic aciduria •Specify enzyme defic

Objectives: i.Describe in detail ALA synthaseand ALA dehydratasereactions ii.Outline hemesynthesis starting from glycineand succinylCoAto the formation of heme iii.Distinguish between the different regulatory processes of hemesynthesis in the liver and in the erythroidcells iv.Explain how pyridoxine deficiency affects hemesynthesis v.Discuss the effects of lead poisoning on hemesynthesis vi.Categorize the different types of porphyriasbased on clinical manifestations such as abdominal pain,

Objectives: Hemedegradation and jaundice •Outline the steps in the degradation of hemeto bilirubinin the macrophages. •Explore the relationship of serum albumin and the transport of bilirubin •Explain the biochemical consequence of a block in each of the steps in the uptake and conjugation of bilirubinin liver. Distinguish the activity of UDP-glucuronyltransferasein adults versus premature babies. •Analyze the steps in the processing of conjugated bilirubinin the intestine and its excretion

Objectives: i.Describe how plasma proteins can be separated by electrophoresis and classify plasma proteins based on electrophoreticmobility ii.Describe the functions of serum albumin and globulins iii.Indicate the role of proteins of the complement system iv.Predict the common causes of hypoalbuminemia(liver disease, nephroticsyndrome, protein malnutrition) and explain the biochemical basis for the occurrence of edema in hypoalbuminemia v.Distinguish the functions of proteins that are foun

Objectives: i.Describe how plasma proteins can be separated by electrophoresis and classify plasma proteins based on electrophoreticmobility ii.Describe the functions of serum albumin and globulins iii.Indicate the role of proteins of the complement system iv.Predict the common causes of hypoalbuminemia(liver disease, nephroticsyndrome, protein malnutrition) and explain the biochemical basis for the occurrence of edema in hypoalbuminemia v.Distinguish the functions of proteins that are foun

Objectives: Hemostasis •Outline the four phases of hemostasis. •Discuss vascular spasm and the role of endothelin. •Describe the platelet plug formation. •Explain the role of integrins GPIb and GPIa. •Describe the role of platelet-activating factors (ADP, serotonin, TXA2and thrombin). •Describe the role of integrinGPIIb/IIIain platelet aggregation. •Describe the coagulation cascade, including the extrinsic, intrinsic and common pathways. •Indicate the role of calcium in hemostasis •Disc

Objectives: Liver function tests •List the important functions of the liver –Excretion of bilirubin –Synthesis of plasma proteins –Detoxification of ammonia •Interpret the values of the following laboratory tests in the diagnosis, follow up and prognosis of a patient with liver disease (acute hepatitis, alcoholic liver disease, cholestaticdisease) –Serum (total, conjugated and unconjugated) and urine bilirubin –Serum enzymes (ALT, AST, GGT, 5’NT, LDH) –Serum proteins (albumin and globuli