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Biochem Chp 03 Globular Proteins
Lipincott
33
Biology
Graduate
11/13/2009

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Term
Ferris
Ferric
Definition
Fe2+
Fe3+
Term
Hemoglobin is rich in this secondary structure.
Definition
alpha helixes
Term
The curve for the hemoglobin oxygen binding chart is...
Definition
Sigmoidal; high affenity at low O2
Term
what is apoHb
Definition
no heme
Term
MetHb
Definition
F3+ or ferric heme
Term
What AA is on the N-terminus of the beta hemoglobin?
Definition
Valine
Term
HbF,what is it and how does it respond to 2,3BPG
Definition
alpha2 gama2, weaker affentity for 2,3BPG
Term
what are things that favor the T state

What do they do to the Ox binding curve
Definition
Raise in pH, 2,3 BPG, lo H+, lo Km; strong affenity

bring oxygen binding curve to the left, positive
Term
EmbronicHb
Definition
AKA gower 1, zeta2,epsilon2
Term
Hb A2
Definition
alpha2 delta 2, 12 weeks after birth, 2%of adult hemogloin.
Term
What is the life span of HbS.
Definition
20 days
Term
(+)--HbX--HbY--HbZ--(-)

and the mutations
Definition
X-HbA glu
Y-Hbs-Val
Z-HbC-Lys
Term
Hb C disease symptoms
Definition
Mild, Chronic Hemolytic anemia
Term
What is cooly anemia?
Definition
In thalassemia Beta major
Term
What is the diference between Thalassimia beta major and minor?
Definition
Minor- fine, mild syptoms
Major-cooly anemia; death at 15-25 months
Term
Name the severity of Alpha thalacimias according to genes defective.
Definition
1 gene- silent
2 -heterozygous form- mild symptoms
4 - Hydrops fetalis - fetal death
3 - HbH -Mild to moderate symptoms
Term
Methemoglobinemia
Definition
Fe2--> Fe3 ferric

caused by action of drugs

Termed Chocolate cyanosis bc of color of blood.
Term
What vitamin is required for Protoporphiren synthesis?
Definition
B6
Term
what is the Y axis for the O2 binding chart?
Definition
Theta= [MbO2]/[Mb]+[MbO2]
Term
Where does glucose attach to on HbA1C?
Definition
to valines of the beta chain?
Term
When there is a Right shift on a Hb binding graph what happens to the km
Definition
Increases
Term
what is formed in an acidic inviroment with the Hb?
Definition
Carbamate on the N termini of the subunit.
Term
What charge does 2,3BPG have
Definition
(-)
Term
What ethnicity is alpha thalassemia high in?
Definition
Asian
Term
What ethnicity is beta thalassemia high in?
Definition
Meditaranian, greek ect.
Term
What is the cause of familial methemaglominia?
Definition
no reductase
Term
What is the drug that can cause methemaglobimia?
Definition
nitrates and nitites
Term
What is hemochromotosis
Definition
Over production of iron.
Term
what gene is implicatited in hemochromotosis?
Definition
HFE gene, it shows incomplete penitrance
Term
What is the treatment for hemochromotosis?
Definition
deferaxamine- Fe chelator, claw
Phlebectomy- removal of some or all of a vein.
Term
What is gereditary spherocytosos? and what protien is implicated?
Definition
Biconcave RBC due to a spectrin protein malfunction. Spectrin is a RBC membrane protien for support and shape.
Term
What happens to the O2 binding curve in Pyruvate kinase deficiency and why?
Definition
the glycolytic pathway is blocked and 2,3BPG accumulates causing a right or negative shift.
Term
What happens to the O2 binding curve in Hexokinase dificency?
Definition
Production of 2,3 BPG is halted and the ot binding curve shifts to the left or positivly?
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