Term
|
Definition
Hb (O2 transport) and Mb (O2),
microsomal and mitochondrial cytochromes (e- transfer),
enzymes |
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Term
|
Definition
| E1, aminolevulinc acid synthase, vitamin B6 cofactor, requires glycine and succinyl CoA (from Kreb's Cycle), works in mitochondrion, ALA transport into cytoplasm |
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Term
|
Definition
| E8, inserts Fe2+ ion into porphyrin prosthetic group, produces heme in mitochondrion |
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Term
|
Definition
heme functions as direct feedback inhibitor on enzyme
also upregulates transcription factors which decrease ALAS gene transcription |
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Term
|
Definition
| x-linked ALAS-E deficiency, hereditary |
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Term
|
Definition
| diseases resulting from excretion and accumulation of heme biosynthetic intermediates due to deficiency in some enzymatic step |
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Term
|
Definition
| lead poisoning, iron deficiency |
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Term
|
Definition
acute intermittent porphyria, defiency in E3, ALA buildup resembles GABA, stimulates GABA receptors--> nerve damage,
phenobarbital aggravates |
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Term
|
Definition
Ferrochelatase activity decreased, excretion of protoporphyrin IX
affects liver most |
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Term
|
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) |
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Term
|
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 |
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Term
|
Definition
Elevation of unconjugated bilirubin and/or conjugated bilirubin
sign is jaundice
hepatitis or cirrhosis |
|
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Term
|
Definition
Type I and type II
absence of glucuronyl transferase which is required for conjugation to glucuronic acid |
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Term
|
Definition
neonatal or physiological jaundice
resolves self as liver matures, or neonate must be placed under blue UV light |
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Term
|
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 |
|
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Term
|
Definition
| abnormal gene that damages cells that would repair damaged DNA |
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Term
| Anemia due to folic acid and/or vitamin B12 deficiency |
|
Definition
megaloblastic anemia, too large of RBCs
pernicious anemia, decrease in RBCs |
|
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Term
|
Definition
hypochromic anemia
thalassemias |
|
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Term
|
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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