Exam (elaborations)
PORPHYRINS HEME SYNTHESIS TEST
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PORPHYRINS HEME SYNTHESIS TESTThe major clinical symptoms of heme
metabolism deficiency are
Anaemia, and/or
Jaundice
heme metabolism deficiency
More rarely, ______, single gene disorders
involving heme synthesis, are observed.
Sometimes symptoms are precipitated by a
triggering factor
porphyrias
Porphyrias
• Errors in heme synthesis ____, characterized by the buildup of
intermediates in ____ and by ____
and skin discoloration
• Acute, Chronic, Hepatic, Non-hepatic,
Erythropoietic
• ______ Regulation of heme synthesis guides
treatment for acute porphyrias
enzymes
urine
blisters
Metabolic
Microcytic Anemia
• ____ poisoning of enzymes in heme synthesis
• Vitamin ___ deficiency
• ____ deficiency
• ____ deficiency
Lead
B6
ALAS-2
Iron
, Hyperbilirubinemia
• __ or ____ bilirubin
• Etiology of _____ (an introduction)
Unconjugated or conjugated
jaundice
A porphyrin is a cyclic molecule consisting of four ____ rings connected
through a methenyl bridge. The conjugated double bonds in the
tetrapyrrole ring structure affords a ____ color to the porphyrin.
Substitutions at the vertices can be one of four types, acetyl, propionyl,
methyl or vinyl. (next porphyrinogens). Naturally occurring porphyrins
are _____ in the 4th ring substituents. The porphyrin associates with a
divalent metal ion.
pyrrole
red
asymmetrical
Protoporphyrin IX is the _____ prosthetic group. When bound by iron (ii)
it forms Heme ___, the main naturally occurring heme molecule in
_____. The enzyme involved in addition of ferrous iron is mitochondrial
_______, which is inhibited by lead. When the iron in the heme
prosthetic group becomes oxidized, it is called ___. This will occur
spontaneously for isolated heme when exposed to ____, although
protein bound heme is protected from oxidation by the protein
environment.
apo-heme
b
hemoglobin
ferrochelatase
Hemin
air
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