Answer these questions for bds 1st year biochemistry board exam of kathmandu university
heme biosynthesis pathway regulation ALA synthase enzymes steps

| # | Topic | Key Point to Remember |
|---|---|---|
| Q1 | Porphyria + Heme regulation | 2 types (hepatic/erythropoietic); ALAS is rate-limiting; feedback by heme |
| Q2a | Hemoglobin structure | Tetramer; 4 heme groups; T & R states; 2,3-BPG |
| Q2b | G6PD deficiency | X-linked; no NADPH → no GSH → oxidative hemolysis; Heinz bodies |
| Q2c | Sickle cell Hb | Glu→Val mutation; HbS polymerizes when deoxygenated; diagnose by electrophoresis |
| Q2d | Glycolytic products in RBCs | ATP, NADH, 2,3-BPG, Lactate - no mitochondria |
| Q2f | Porphyria (short note) | AIP: PBG deaminase deficiency; port-wine urine; neurovisceral attacks |
| Q3/Q4 | Iron absorption/hepcidin | DCYTB→DMT-1→Ferroportin; Hepcidin degrades ferroportin |
| Q5 | HMP shunt importance | NADPH (protection, synthesis); Ribose-5-P (nucleotides) |
| Q6 | Glycolysis + enzymes | 3 rate-limiting: Hexokinase, PFK-1 (most important), Pyruvate kinase |
| Q7 | Acute Intermittent Porphyria | PBGD deficiency; ALA+PBG accumulate; Rx: glucose + hematin |