P2 BIOCHEMISTRY Flashcards
heme protein found in cardiac & red skeletal muscles
Myoglobin
Functions of myoglobin
- reservoir for O2
- O2 carrier
type of amino acids present inside myoglobin
Hydrophobic
type of amino acids present on the surface of myoglobin
hydrophilic
the proximal histidine number
His 93
the distal histidine number
His 64
His 93 function
forming bound with heme iron
His 64 function
Stabilising the bound to O2
main function of haemoglobin
Transport of O2
low oxygen affinity form of Hb is known as
deoxy form / T conformation / tense form
high oxygen affinity form of Hb is known as
oxy form / R confirmation / relaxed form
the curve shown below is known as
Oxygen dissociation curve
the normal shape of the curve for haemoglobin
sigmoidal in shape
the normal shape of the curve for myoglobin
Hyperbolic
partial pressure at which Hb is 50% saturated with O2
P50
Incase of disease , Hb O2 affinity curve shift to
the curve shifts to right or left
incase of increased P50 , the curve will shift to
right
incase of decreased P50 , the curve will shift to
left
incase of increased BPG , the curve will shift to
RIGHT
incase of decreased BPG , the curve will shift to
left
type of Hb that has a high affinity for O2
HbF
Substitution of which AA causes sickle cell disease
from glutamic acid to valine acid
Def. in beta chain production with increase in alpha chain
beta thalassemia
Def. in alpha chain production with increase in beta chain
alpha thalassemia
when all of the 4 alpha globin genes are affected it is known as
hydrops fetalis
incase of 2 affected alpha globin the Pt will have
alpha thalassemia trait
incase of 3 alpha globin genes affected Pt will have
HbH disease
name of the SHUNT that is interrupted incase of G6PD def.
hexose monophosphate shunt
main function of NADPH released in pentose phosphate pathway
reduction of glutathione molecule
Enzyme that is used to reduce glutathione molecule
Glutathione reductase
Genetic predisposition of G6PD def.
X-linked recessive
protein that is aggregated and accumulated in G6PD def. seen in blood smear as
heinz bodies
clinical manifestations of G6PD def.
- acute hemolytic anemia
- neonatal jaundice
these cases can cause haemolytic anemia in G6PD def.
- oxidant drugs
- favism
G6PD carriers are protected against
Malaria
severe hemolysis dut to G6PD def is manifested as
methemoglobinemia
