Porphyrins and Hemoglobin
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chemical intermediates of hemoglobin, myoglobin, cytochromes, and enzymes (catalase and peroxidase)
Porphyrins
What causes red color of hemoglobin?
presence of alternating double bonds of porphyrins allows the structure to absorb light
All porphyrins are macrocyclic structures consisting of a _________ ring linked by______ bridges.
tetrapyrrole, methyl
Porphyrins also fluoresce reddish-pink at ____ nm
400
Four ____ atoms chelate metal atoms (usually iron, competes with zinc) ▪ Iron chelates with protoporhyrin IX to form heme
Nitrogen
Iron in heme can also bind to globin proteins to form ________ & ____________.
HEMOGLOBIN & MYOGLOBIN
fights against reactive oxygen species
catalase
hemoglobin, myoglobin, and cytochromes all contain…
heme
Heme synthesis occurs in all cells, but primarily ____________(erythroidprecursors) and ______.
bone marrow, liver
Iron chelates with ________________ to form heme
protoporhyrin IX
Enzymes in heme synthesis localized in…
mitochondria and cytoplasm
what are the Mitochondrial enzymes involved with heme synthesis?
1) ALA synthase, 2) copropophyinogen oxidase, 3) protoporhyrinoghen oxidase, and 4) ferriochelatase
What are the Cytoplasmic enzymes involved with heme synthesis?
1) ALA dehydrogenase, 2) PBG deaminase, 3) Uroporphyrinogen III synthetase, and 4) Uroporphyrinoghen decarboxylase
ALA & PBG are ________soluble
water
precursors to porphyrins
▪ Porphyrinogens
these spontaneously oxidize (with light oxygen or oxidizing agents) to form three major porphyrins:
Porphyrinogens
three major porphyrins…
• Uroporphyrin [URO]: excreted in urine • Coproporphyrin [CORPO]: excreted in urine and/or feces • Protoporphyrin [PROTO]: excreted in feces
porphyrin that is excreated in urine?
Uroporphyrin [URO]
porphyrin excreted in urine and/or feces
Coproporphyrin [CORPO]
porphyrin that is excreted in feces?
Protoporphyrin [PROTO]
▪Porphyria can be inherited or caused by…
drugs, liver disease, anemias, or toxic metals that cause disturbances in heme synthesis and porphyrin metabolism
How many enzymes are involved in heme synthesis?
8 Deficiency in seven of the eight enzymes involved in heme synthesis ! Leading to a distinct form of porphyria
Lack of enzyme activity –> decrease in formation and excretion of porphyrin or accumulation of its __________.
precursors
Symptoms of Neurological porphyria?
Constipation, nausea, vomiting, severe abdominal pain, tachycardia, hypertension, sweating and urinary retention, *neurotoxicity, psychiatric disturbances, Cutaneous lesions, hyperpigmentation