Term
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Definition
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Term
Hemoglobin is rich in this secondary structure. |
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Definition
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Term
The curve for the hemoglobin oxygen binding chart is... |
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Definition
Sigmoidal; high affenity at low O2 |
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Definition
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Definition
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What AA is on the N-terminus of the beta hemoglobin? |
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Definition
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Term
HbF,what is it and how does it respond to 2,3BPG |
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Definition
alpha2 gama2, weaker affentity for 2,3BPG |
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Term
what are things that favor the T state
What do they do to the Ox binding curve |
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Definition
Raise in pH, 2,3 BPG, lo H+, lo Km; strong affenity
bring oxygen binding curve to the left, positive |
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Term
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Definition
AKA gower 1, zeta2,epsilon2 |
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Term
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Definition
alpha2 delta 2, 12 weeks after birth, 2%of adult hemogloin. |
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Term
What is the life span of HbS. |
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Definition
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Term
(+)--HbX--HbY--HbZ--(-)
and the mutations |
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Definition
X-HbA glu Y-Hbs-Val Z-HbC-Lys |
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Term
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Definition
Mild, Chronic Hemolytic anemia |
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Term
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Definition
In thalassemia Beta major |
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Term
What is the diference between Thalassimia beta major and minor? |
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Definition
Minor- fine, mild syptoms Major-cooly anemia; death at 15-25 months |
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Term
Name the severity of Alpha thalacimias according to genes defective. |
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Definition
1 gene- silent 2 -heterozygous form- mild symptoms 4 - Hydrops fetalis - fetal death 3 - HbH -Mild to moderate symptoms |
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Term
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Definition
Fe2--> Fe3 ferric
caused by action of drugs
Termed Chocolate cyanosis bc of color of blood. |
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Term
What vitamin is required for Protoporphiren synthesis? |
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Definition
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Term
what is the Y axis for the O2 binding chart? |
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Definition
Theta= [MbO2]/[Mb]+[MbO2] |
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Term
Where does glucose attach to on HbA1C? |
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Definition
to valines of the beta chain? |
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Term
When there is a Right shift on a Hb binding graph what happens to the km |
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Definition
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Term
what is formed in an acidic inviroment with the Hb? |
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Definition
Carbamate on the N termini of the subunit. |
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Term
What charge does 2,3BPG have |
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Definition
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Term
What ethnicity is alpha thalassemia high in? |
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Definition
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Term
What ethnicity is beta thalassemia high in? |
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Definition
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Term
What is the cause of familial methemaglominia? |
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Definition
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Term
What is the drug that can cause methemaglobimia? |
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Definition
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Term
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Definition
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Term
what gene is implicatited in hemochromotosis? |
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Definition
HFE gene, it shows incomplete penitrance |
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Term
What is the treatment for hemochromotosis? |
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Definition
deferaxamine- Fe chelator, claw Phlebectomy- removal of some or all of a vein. |
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Term
What is gereditary spherocytosos? and what protien is implicated? |
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Definition
Biconcave RBC due to a spectrin protein malfunction. Spectrin is a RBC membrane protien for support and shape. |
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Term
What happens to the O2 binding curve in Pyruvate kinase deficiency and why? |
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Definition
the glycolytic pathway is blocked and 2,3BPG accumulates causing a right or negative shift. |
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Term
What happens to the O2 binding curve in Hexokinase dificency? |
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Definition
Production of 2,3 BPG is halted and the ot binding curve shifts to the left or positivly? |
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