Patel ASSISTANT PROFESSOR BIOCHEMISTRY GMCS NORMAL ADULT HUMAN HEMOGLOBIN Structure and Function of Hb Oxygen dissociation curve P50 T amp R form of Hb Structure and Function of Hb ID: 934009
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Slide1
Hemoglobin
Dr.
Sarita
Patel
ASSISTANT PROFESSOR
BIOCHEMISTRY, GMCS
Slide2NORMAL ADULT HUMAN HEMOGLOBIN
Slide3Slide4Slide5Slide6Slide7Slide8Slide9Structure and Function of
Hb
Slide10Oxygen dissociation curve
P50 ???
Slide11Slide12Slide13Slide14Slide15T & R form of Hb
Slide16Structure and Function of
Hb
Slide17Co-operative binding of oxygen
Slide18Bohr effect
Slide19Bohr’s effect
Slide20Slide21Slide22Slide23Role of 2,3 BPG
Slide24Slide25Slide26Slide272,3 BPG leve
l
Increase -
High altitude
Chronic lung disease
Anemia
Decrease- Hypophosphatemia
Slide282,3 BPG has less affinity for HbF
Gamma globin and
β
globin proteins are 72
% identical at the amino acid level
one
significant amino acid difference exists in the
2,3BPG binding
pocket.
In the
γ
globin protein
, His 143 is replaced by a
Ser
in the
2,3BPGbinding site
.
The
effect of this amino
acid substitution
is the loss of two positive charges (one from each
γ subunit relative
to the
β
subunits
).
Slide292,3 BPG IN STORED BLOOD????Biochemical changes in stored whole blood
PH?
K+?
2,3 BPG
Plateletes
Role of Preservative for storage of whole blood?
Slide30Slide31Slide32CARBAMINO-HEMOGLOBIN
Hb
combine with C02.
Slide33Methemoglobin
Carboxyhemoglobin
Slide34Slide35Slide36Slide37Slide38Slide39Slide40Slide41Regulation in
erythroid
cell.
Regulated by
Ferochelatase
and
Uroporphyrinogen
Synthase
No Rate limiting step.
Slide42Slide43Slide44Slide45Slide46Slide47Reason of Pain and neuropsychiatric symptoms????Reason of Photosensitivity?
Anemia
is not a predominant feature
Slide48Slide49Slide50Precipitating Factor
Drinking alcohol
Excessive intake of iron
Smoking
Fasting
Drugs like Barbiturates, Sedatives
Sun exposure
Slide51Slide52Treatment
Symptomatic treatment for
Pain
Nausea,
Vomitting
Intravenous
Hemin
Intravenous Glucose
Avoidance of triggering factor
Antioxidant – vitamin E, beta carotene.
Slide53Role of Glucose in Porphyria
ALA synthase enzyme is a rate limiting enzyme.
Transcription of ALA synthase is
upregulated
by Peroxisome Proliferator activated receptor coactivator-1 (PGC-1).
PGC-1 is regulated by Glucose level.
Slide54Hypoglycemia induce PGC-1 production
Increase ALA synthase
Increase
Heme
production
Slide55Normal red cell breakdown
haemoglobin
haem
protoporphyrin
iron
Bilirubin
(free)
CO
Expired air
transferrin
erythroblast
Bilirubin glucuronides
Stercobilin(ogen)
Urobilin(ogen)
Urine
Liver
conjugation
faeces
globin
Amino acids