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Terms in this set (10)
Heme Synthesis occurs in...
-Bone Marrow in RBC Precursors
-Hepatocytes (used in the liver in Cytochrome p450)
Defective heme synthesis that leads to the accumulation of heme precursors
ALA Synthase is inhibited by...
Glucose and Heme! This is the Rate Limiting Step!
X-Linked Sideroblastic Anemia
-ALA Synthase (+Vitamin B6)
-High Doses of Vitamin B6
-Fe accumulates in Erythroid Marrow and deposits in mitochondria, which encircle the nuclei of pro-erythroblasts and result in Sideroblasts.
-ALA Dehydratase and Ferrochelatase
-Aminolevulinic Acid and Erythrocyte Protoporphyrin
Acute Intermittent Porphyria
Congenital Erythropoietic Porphyria
-Uroporphyrinogen III Synthase
Porphyria Cutanea Tarda
-involved with Cytochrome p450 and heme build up in the liver
-product produces free radicals when exposed to sunlight, resulting in blisters!
-Heme Oxygenase (requires Oxygen to work)
-Biliverdin Reductase (requires NADPH to work)
-UDP Glucuronosyl Transferase
G6PD Deficiency causes more severe neonatal Hyperbilirubinemia and Jaundice because...
NADPH cannot be regenerated and birth involves a lot of oxidative stress. This leads to Hemolytic Anemia, a breakdown of Heme Products, and thus Hyperbilirubinemia and Jaundice!
THIS SET IS OFTEN IN FOLDERS WITH...
Anemias by Pathoma
Gene Expression, Translation, and Diagnostic Metho…
Data Acquisition EBM
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