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Questions and Answers
What is the primary site of heme synthesis in the body?
What is the primary site of heme synthesis in the body?
- Cytosol
- Nucleus
- Ribosomes
- Mitochondria (correct)
Which enzyme is crucial for inserting iron into the heme structure?
Which enzyme is crucial for inserting iron into the heme structure?
- Ferrochelatase (correct)
- Globin synthetase
- Heme oxidase
- Porphyrinase
Which chromosome is responsible for the production of the alpha globin chain?
Which chromosome is responsible for the production of the alpha globin chain?
- Chromosome 11
- Chromosome 16 (correct)
- Chromosome 10
- Chromosome 7
In which tissue is the majority of heme formed?
In which tissue is the majority of heme formed?
What role do mitochondria play in heme synthesis?
What role do mitochondria play in heme synthesis?
Flashcards
Citrate Agar (pH 6.0 to 6.2)
Citrate Agar (pH 6.0 to 6.2)
A specialized agar used to differentiate variant hemoglobins, particularly for distinguishing hemoglobin S from D/G and hemoglobin C from E/O Arab/Charlem
Hemoglobin Variant Differentiation
Hemoglobin Variant Differentiation
Using citrate agar to distinguish different types of abnormal hemoglobins
HEME (Ferroprotoporphyrin IX)
HEME (Ferroprotoporphyrin IX)
The iron-containing part of hemoglobin, crucial for oxygen transport.
Porphyrins
Porphyrins
A class of pigments, including heme, that have a specific ring structure.
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Heme Synthesis Site
Heme Synthesis Site
Mitochondria, the energy-producing part of the cell.
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Ferrochelatase (Heme Synthetase)
Ferrochelatase (Heme Synthetase)
Enzyme that inserts iron into the heme molecule.
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Globin Synthesis Site
Globin Synthesis Site
Ribosomes in the cytoplasm of red blood cell precursors (normoblasts).
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α (alpha) globin chain gene location
α (alpha) globin chain gene location
Chromosome 16 dictates production of the α globin chain.
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Cytosol
Cytosol
The liquid part of the cytoplasm outside the mitochondria.
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Mitochondria
Mitochondria
Organelles that generate energy (ATP) and involved in heme synthesis
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Citrate Agar Electrophoresis
- Used to confirm variant hemoglobins
- Differentiates hemoglobin S from D and G, and hemoglobin C from E, O Arab, and Charlem hemoglobins
Hemoglobin Synthesis
A) Heme Synthesis
- Heme, aka Ferroprotoporphyrin IX
- Belongs to porphyrins
- Site of synthesis: mitochondria
- Occurs in metabolically active cells with mitochondria
- Primarily in bone marrow and liver
- Erythroid marrow is the major heme-forming tissue (85%)
B) Globin Synthesis
- Site: ribosomes in the normoblast cytoplasm
- Chromosome 16 dictates alpha globin chains
- Chromosome 11 dictates other globin chains (except theta)
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