Hemoglobin Synthesis Flashcards
Hemoglobin contribute to _______ balance.
acid-base
How many amino acids are there in a Hb structure?
574 amino acids
It is responsible for Hb affinity to O2 and release O2 from heme.
2-3, diphosphoglycerate
Increase in 2,3 DPG production indicates
low Hb’s O2 affinity
Give shift RIGHT consequences on:
- O2 affinity
- pH
- temperature
- adult or young RBC?
- low O2 affinity
- increase pH (acidic)
- increase temperature
- adult RBC
Give shift LEFT consequences on:
- O2 affinity
- pH
- temperature
- adult or young RBC?
- high O2 affinity
- high pH (basic)
- low temperature
- young RBC (HbF)
balance maintenance through uptake and release
iron
apical brush border of duodenal enterocytes
Divalent Metal Transporter 1
iron storage
ferritin
basolateral border of duodenal enterocytes
Ferroportin 1
allows macrophages to recycle iron
Ferroportin 1
What is hemochromatosis?
Iron overload
What happens in hemochromatosis?
Increase ferroportin
Increase iron
plasma protein transporter
transferrin
regulates iron distribution and metabolism (absorption and mobilization)
hepcidin
insuffienct hepcidin leads to
hemochromatosis
overexpression of hepcidin leads to
microcytic anemia
Name the condition when there is an absence of any enzymes involved in heme synthesis
porphyria
True or False:
Sideroblastic anemia accumulates large amount of ferritin in RBCs
True
Give the globin chain.
Gower I
zeta + epsilon
Give the globin chain.
Gower II
alpha + epsilon
Give the globin chain.
Portland
zeta + gamma
Give the globin chain.
HbF
alpha + gamma
Give the globin chain.
HbA
alpha + beta
Give the globin chain.
HbA2
alpha + delta
Give the globin chain.
Hb A1C
alpha + beta + glucose
Variant Forms of Hemoglobin
has a cherry red color and has a greater affinity to CO than O2
carboxyhemoglobin
Variant Forms of Hemoglobin
forms greenish derivative, contains sulfur, cannot transport O2
sulfhemoglobin
Variant Forms of Hemoglobin
ferric state, cannot combine with oxygen
methemoglobin
Metabolic Activities of Erythrocytes
uses glucose to generarte ATP and maintains cellular energy
Embden-Meyerhof Glycolytic Pathway
Metabolic Activities of Erythrocytes
What is the ATP net gain Embden-meyerhof Glycolytic Pathway?
2 ATP’s
Metabolic Activities of Erythrocytes
prevents heme iron oxidation
Methemoglobin Reductase Pathway
Metabolic Activities of Erythrocytes
ferrous to ferric
Methemoglobin Reductase Pathway
Metabolic Activities of Erythrocytes
regulates Hb’s O2 affinity (2,3-DPG)
Luebering-Rapoport Pathway
Metabolic Activities of Erythrocytes
permits 2,3-DPG accumulation
Luebering-Rapoport Pathway
Metabolic Activities of Erythrocytes
also known as:
“Oxidative Pathway”
“Hexose Monophosphate Shunt”
“Pentose Phosphate Shunt”
Phosphogluconate Pathway
Metabolic Activities of Erythrocytes
prevents globin denaturation by oxidation
Phosphogluconate Pathway
Metabolic Activities of Erythrocytes
G6PD deficiency and Heinz bodies presence
Phosphogluconate Pathway