Rate-limiting for bile acid synthesis; up-regulated by bile acid sequestrants
Enzymes & cofactors
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Vitamin D-dependent rickets type 1; stimulated by PTH, suppressed by FGF-23
Zileuton target; leads to leukotrienes
Inhibited by cyanide, carbon monoxide, azide, H2S
Irreversibly acetylated by aspirin; NSAID and coxib target
CYP3A4 handles ~50% of drugs; major site of drug-drug interactions
Inhibited by oligomycin; bypassed by uncouplers (DNP, UCP1)
Carboxylates factors II, VII, IX, X, protein C, S, Z
Produces biliverdin, CO and Fe2+; rate-limiting for heme catabolism
Alkaptonuria: dark urine on standing, ochronosis, arthritis
MAOI + tyramine: hypertensive crisis
Phenylketonuria: musty odour, intellectual disability, hypopigmentation; BH4 defects cause malignant PKU