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Hematology - Heme synthesis, Porphyrias, Lead Poisoning (*Porphyrias and…
Hematology - Heme synthesis, Porphyrias, Lead Poisoning
*Heme Synthesis
- in the MIGHTY DRACUALA mitochondria of bone marrow and liver
- Blood = B6 cofactor pyridoxine for dALA synthase enzyme
MIGHTY DracuALA SUCK blood with his B6 GLISSENING Teeth
- MIGHTY = mitochondria makes Heme
- Dracu - ALA = d ALA = delta - aminolevulinic acid
- SUCKS = succinyl coA
- GLISTENNING = glycine
--> succinyl coA and glycine both combine in MITO to make dALA then HEME
- Blood = B6 cofactor pyridoxine for dALA synthase enzyme
Will Ferrel as a VAMPIRE DracuALA - K STEW Conan on Twighlight couple breaking up
- Will Ferrel = Fe and Ferochelase --> make HEME
- DracuALA --> Heme inhibits dALA dracuALA synthase
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*Porphyrias and Lead poisoning
- all effect heme synthesis
- broken into EARLY vs LATE porphyrias
- EARLY porphyrias
--> GI pain + neuropsych problems
- LATE porphyrias
--> activated by UV sunlight, then oxygen reactive
--> dermatitis int he skin
*LATE porphyrias
- activated by UV sunlight, then oxygen reactive
--> dermatitis int he skin
- anything AFTER PBG Deaminase = late porphyria
--> this is the cutoff
- PCT = Porphyrin Cutaneous Tarde
--> this is the most COMMON porphyria also
- UROD = Uroporphyrinogen Decarboxylase
*PCT = Porphyrin Cutaneous Tarde
- this is the most COMMON porphyria also
- UROD = Uroporphyrinogen Decarboxylase
--> deficient in this enzyme
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*EARLY porphyrias
- GI pain + neuropsych problems
- EARLY porphyrias
*AIP = acute intermittent Porphyria
- inhibits the PBG DA = Deaminase enzyme
- tx = heme
--> for acute AIP attacks
- the heme inhibits ALAS and production of the excess PBG and ALA that builds up
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