heamoproteins 3

Question 1

the common forms of heanglobin:

Answer 1

• HBA adult ( 2 alpha , 2 beta )
• HBF foetl ( 2 alpha 2 gamma )
• HBA2 minot adult form ( 2 alpha 2 delta )
• HBS sickle cell anmeia ( 2 alpha , BS2 )

Question 2

alpha globin genes are located on the human chromosome — and – genes copies per chromsome

Answer 2

chromosome 16
2 genes copies

Question 3

beta globin gene is located in chromosome — and only – gene copy per choromome
other globin genes rathe than beta can be: delta or gamma

Answer 3

• 11
  -1 gene copy

Question 4

— has 2 alpha and 2 beta and the prosthetic group is —- that is tightly bound to the protein and required for the proteins activity

Answer 4

HBA , fe II protprophyin IX

Question 5

– measures the strength of binding to oxygen and is defined by Po2 which % saturation is — %

Answer 5

• p50
• 50%
• HBA: p50 is 26 torr in normal rbc
• mb: p50 = 1 torr

Question 6

– is the natural variant of haemoglobin and consist of —

Answer 6

foetal hemoglobin , 2 alpha 2 gamma

Question 7

— is the main oxygen transport protein in the foetus during the last seven months of the development of the uterus snf in the new born until roughly 6 month

Answer 7

foetal heamoglobin

Question 8

the foetal haemoglobin differs from adults bc its able to bind oxygen with — than adults

Answer 8

greater affinity and is more tightly
p50: 19 torr bc it reaches the 50% saturation w/ 02 at lower 02 pressure

Question 9

-oxygen passes across the placenta from — into the —-.
maternal HBA releases oxygen at the — but the oxygen concentration( partial pressure ) is —

Answer 9

• maternal bloodstream into fotwal bloodstream
• placenta
• low

Question 10

tighter oxygen binding at the HbF allows the foetus to

Answer 10

pick up as much o2 as possible

Question 11

Mutant haemoglobin is due to the changes in — of the globin genes which leads to changes in —- and eventually changes in —- which leads to —-

Answer 11

• dna base sequence
• amino acid sequexzne of globin
• protein structure and function
• inhereted disease
• clinical sydrom vary from mild to severe and can be fatal depending of the nature of the mutation

Question 12

– is a globin defect w/ normal amount of defective globin subunits

Answer 12

haemoglobinopatheis

Question 13

abnormal amount of normal globin subunit refers to —

Answer 13

thalassamia

Question 14

is also known as the qualitative defect

Answer 14

haemoglobinopathesis

Question 15

is also known is qanrative defect

Answer 15

thalassanias

Question 16

haemoglobin M ( HbM) is an example of –

Answer 16

heamoglobinopathy

Question 17

the amino acid substuition refers to — and the proximal histadine residue is replaced by — in either alpha or beta.
This leads to oxidation of haem iron from — to — in the presence of 02 and proximal tyrosine

Answer 17

• Haemoglobin M
• tyrosine
• fe(ii) ferrous to fe( iii) ferric

Question 18

haemoglobin S is an example of —

Answer 18

haemoglbinpathy

Question 19

— refers to the amino acid substition in —
the glutamate ( ch2ch2coo- ) is replaced by —

Answer 19

• haemoglobin S
• exterior of beta subunit
• valine ch(ch3)2

Question 20

in deoxyheamoglobin S , valine is exposed to surface and creates —. The HbS moliucles aggregate to from — with erythrocytes this leads to — and they get stuck in small capillary beds

Answer 20

• sticky patch
• insoluble fiber
• sickle cell anaemia
  ( steps: polar hydrophobic interactions –> superpolymer –> precipitate out )

Question 21

-the short term consequence to HbS is — and long term leads to —
- the diagnosis is by —-
- treatment by —

Answer 21

• bone pain due to the sickle cells are stuck in capillary beds
• chronic anemia due to the destruction of rbc, organic damage as kidgney,heart,lungs, cerebrovacualr accidents
• protein or dna analysis
• repeated blood transfusion

Question 22

— refers to a disorder caused by weakening and destruction of rbc and causing by – or – genes that affects how the body produce —

Answer 22

• thalasmeia ( aka quantitive disorder of too little alpha or beta )
• varient or missing
• haemoglobin

Question 23

alpha thalassamia refers to –
beta thalassemia refers to —
common is mediterian areas and parts of African and south east Asia and syndromes depends on specific mutation

Answer 23

• too little alpha goblin chain
• too little beta globin chain