Unit 5 Flashcards
What is heme composed of?
Protoporphyrin ring + Fe
What is hemoglobin composed of?
Heme + 4 globin chains
What is the storage form of iron?
Fe3+
Ferric
What is the functional form of iron?
Fe2+
Ferrous
What are porphyrins?
Any class of pigments whose molecules contain a flat ring of four linked heterocyclic groups
What is Porphyrias
Diseases caused by enzyme deficiencies in the Heme production pathway.
*People will generally look purple.
What is Hemin
A black inorganic compound which forms from heme when RBCs are lysed and exposed to air.
What is the most common Primary Porphyrias?
Porphyria Cutanea Tarda (PCT)
What is Porphyria Cutanea Tarda deficient in?
Uroporphyrinogen Decarboxylase
What test is ordered to confirm PCT
Serum porphyrin
What is PCT defined by?
Blistering
What is Acute Intermittent Porphyria deficient in
PBG Deaminase
What is AIP defined by
Blistering
Nerve Impairments
What test is ordered to confirm AIP
Urine porphobilinogen
What is Erythropoietic Porphyria deficient in
Ferrochelatase
What will Erythropoietic Porphyria show an increase in
Total erythrocyte protoporphyrin
What is Erythropoietic Porphyria defined by
Blistering
What is the rate limiting step in porphyrin synthesis
ALA-Dehydratase
How do we test for ALA-Dehydratase Deficiency
Delta-aminolevulinic
What is the most common presenting symptom of ALA-Dehydratase deficiency?
Abdominal pain
What does ALA-Dehydratase deficiency cause
Accumulation of d-aminolevulinic acid
What does lead poisoning do?
Inhibition of most of the enzymes in the synthetic pathway
What secondary porphyria has an elevated level of succinylacetone
Hereditary tyrosinemia
What method do we use for Porphobilinogen
Watson-Schwartz