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Heme Metabolism
James Clark
21
Medical
Graduate
09/22/2011

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Term
Heme-containing proteins
Definition
Hb (O2 transport) and Mb (O2),
microsomal and mitochondrial cytochromes (e- transfer),
enzymes
Term
ALAS
Definition
E1, aminolevulinc acid synthase, vitamin B6 cofactor, requires glycine and succinyl CoA (from Kreb's Cycle), works in mitochondrion, ALA transport into cytoplasm
Term
Ferrochelatase
Definition
E8, inserts Fe2+ ion into porphyrin prosthetic group, produces heme in mitochondrion
Term
Regulation of ALAS
Definition
heme functions as direct feedback inhibitor on enzyme
also upregulates transcription factors which decrease ALAS gene transcription
Term
Sideroblastic anemia
Definition
x-linked ALAS-E deficiency, hereditary
Term
Porphyrias
Definition
diseases resulting from excretion and accumulation of heme biosynthetic intermediates due to deficiency in some enzymatic step
Term
Acquired porphyrias
Definition
lead poisoning, iron deficiency
Term
Neurologic porphyria
Definition
acute intermittent porphyria, defiency in E3, ALA buildup resembles GABA, stimulates GABA receptors--> nerve damage,
phenobarbital aggravates
Term
Protoporphyria
Definition
Ferrochelatase activity decreased, excretion of protoporphyrin IX
affects liver most
Term
Heme degradation
Definition
heme-->bilirubin + Fe3+ by heme oxygenase
heme is pro-oxidative and pro-inflammatory
bilirubin is anti-oxidant
biliverdin reductase further reduces to bilirubin IX alpha
iron is pro-oxidant (but is sequestered by ferritin)
Term
Bilirubin
Definition
uptake from circulation by liver (albumin)
intracellular storage
conjugation with glucuronic acid to make water soluble
secreted into bile-> intestine-> feces or to kidney for urine
Term
Hyperbilirubinemia
Definition
Elevation of unconjugated bilirubin and/or conjugated bilirubin
sign is jaundice
hepatitis or cirrhosis
Term
Crigler-Najjar syndrome
Definition
Type I and type II
absence of glucuronyl transferase which is required for conjugation to glucuronic acid
Term
Gilbert's syndrome
Definition
neonatal or physiological jaundice
resolves self as liver matures, or neonate must be placed under blue UV light
Term
Anemia
Definition
absence of healthy RBCs
underproduction of mature RBCs or loss of RBCs
Term
idiopathic aplastic anemia
Definition
bone marrow fails to make mature RBCs
autoimmune
Term
Fanconi's anemia
Definition
abnormal gene that damages cells that would repair damaged DNA
Term
Anemia due to folic acid and/or vitamin B12 deficiency
Definition
megaloblastic anemia, too large of RBCs
pernicious anemia, decrease in RBCs
Term
Underproduction of Hb
Definition
hypochromic anemia
thalassemias
Term
hemolytic anemia
Definition
premature destruction of RBCs
abnormal immune system response
blood clots
infections
medications
Term
congenital spherocytic anemia
Definition
disorder of membranes of RBCs
sphere like RBCs that breakdown prematurely
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