Aani CP: Haem Synthesis Flashcards
Which cells do porphyrias affect? (2)
Liver cells and Erythroid cells
What is liver cytochrome needed for?
Detoxification of drugs
What is haem cytochrome needed for?
Electron transport chain in mitochondria
What is at the start of the haem synthesis chain?
Succinyl CoA + Glycine
Which enzyme is needed to convert Succinyl Co-A and Glycine into 5 Aminolaeuvulinic Acid (ALA)?
Where does this reaction take place?
ALA Synthase
In the mitochondria
What does 5 ALA turn into? Which enzyme is used?
PBG (porphobilinogen) using PBG synthase or ALA dehydratase
What does PBG turn into? Which enzyme is used?
HMB (hydroxymethylbilane) using HMB synthase of PBG deaminase
What does HMB turn into? Which enzyme is used?
Uroporphyrinogen III and Uroporphyrinogen I using uroporphyrinogen III synthase
What does Uroporphyrinogen III turn into? Which enzyme is used?
Coproporphyrinogen III using Uroporphyrinogen III decarboxylase
Which mnemonic is used to remember the molecule order for haem synthesis?
Suhaila & Ghausal
ALA Placed Hot Urine Cup Produces Perfect Haem
What does Coproporphyrinogen III turn into? Which enzyme is used? Where does this reaction take place?
Protoporphyrinogen 9 using coproporphyrinogen oxidase. Takes place in mitochondrial wall
What does Protoporphyrinogen 9 turn into? Which enzyme is used?
Protoporphyrin 9 using Protoporphrinogen oxidase
What does Protoporphyrin turn into? Which enzyme is used?
HAEM! Using Ferrochetalase
What is the triad of symptoms in acute Porphyrias?
Abdo pain
Psych symptoms
Neurological/neurovisceral
Which enzyme is affected in Acute Intermittent Porphyria? (AIP)
HMB synthase (How My Bear? - aCUTE)