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BCR 1 > Physiology - Haemoglobin and Haemoglobin genes > Flashcards

Physiology - Haemoglobin and Haemoglobin genes Flashcards

1
Q

What is adult Haemoglobin made up of ?

a. 2 alpha chains + 2 beta chains
b. 2 alpha chains and 2 gamma chains
c. 2 alpha chains and 2 beta chains 6 glu->val

A

a.2 alpha chains + 2 beta chains

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2
Q

what dictates the shape and properties of a protein?

a. polypeptide bonds
c. hydrogen bonds
d. amino acids
e. carboxyl groups

A

d.amino groups

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3
Q

proteins fold into conformations of?

a. lowest energy
b. highest energy
c. lowest bonds
d. highest bonds

A

a.lowest energy

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4
Q

what is the role of proteins known as molecular chaperones?

a. form polypeptide bonds
b. increase efficiency of folding
c. strength and support

A

b. increase efficiency of folding

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5
Q

the linear sequence of amino acids refers to what level of protein structure?

a. primary
b. secondary
c. tertiary
d. quaternary

A

a.primary

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6
Q

the folding and stabilisation of the primary structure into regular elements eg a helix and b pleated sheet by H bonds and others
refers to what level of protein structure?

a. primary
b. secondary
c. tertiary
d. quaternary

A

b.secondary

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7
Q

the folding and stabilisation of secondary structure into a 3d shape refers to what level of protein structure?

a. primary
b. secondary
c. tertiary
d. quaternary

A

c.tertiary

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8
Q

intergration of distinct polypeptide chains into oligomeric complexes refers to what level of protein structure ?

a. primary
b. secondary
c. tertiary
d. quaternary

A

d.quaternary

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9
Q

polar side chains form which element of the compact protein conformation?

a. hydrophobic core region
b. hydrophillic outside of the molecule

A

b.hydrophillic outside of the molecule

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10
Q

which directions to genes run?

a. 5’ to 3’
b. 3’ to 5’

A

a.5’ to 3’

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11
Q

how do genes run within a protein?

a.5’ to 3’
b.3’ to 5’
c. N terminus to C terminus
d,C terminus to N terminus

A

c. N terminus to C terminus

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12
Q

what best describes an alpha helix ?

a. left handed corkscrew
b. right handed corkscrew
c. stretched out structure

A

b. right handed corkscrew

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13
Q

where do the hydrogen bonds run in an alpha helix?

a. between peptide bonds
b. between C-O and NH groups

A

a. between peptide bonds

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14
Q

Where do H bonds run in a beta pleated sheet?

a. between peptide bonds
b. between C-O and NH groups

A

b. between CO and NH groups

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15
Q

what does the proline side chain do to the alpha helix to stabilise it ?

a. make it too flexible
b. make it too rigid

A

b. make it too rigid

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16
Q

what does the glycine side chain do to the alpha helix to stabilise it ?

a. make it too flexible
b. make it too rigid

A

a,make it too flexible

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17
Q

what effect do oxidants have on the disulphide bonds in a proteins tertiary structure?

a. changes their angle
b. changes their properties
c. breaks them
d. forms them

A

d.forms them

18
Q

what effect do reductants have on the disulphide bonds in a proteins tertiary structure?

a. changes their angle
b. changes their properties
c. breaks them
d. forms them

A

c.breaks them

19
Q

what are the distinct regions pf a protein known as?

a. bonds
b. sections
c. domains

A

c.domains

20
Q

what are gene families?

a. similar genes evolved from an evolutionary precursor often regulated in a coordinated manner and arranged in clusters on a chromosome
a. multiple copies of the same gene evolved from an evolutionary precursor often regulated in a coordinated manner and arranged in clusters on a chromosome

A

a.similar genes evolved from an evolutionary precursor often regulated in a coordinated manner and arranged in clusters on a chromosome

21
Q

What is fetal Haemoglobin made up of ?

a. 2 alpha chains + 2 beta chains
b. 2 alpha chains and 2 gamma chains
c. 2 alpha chains and 2 beta chains 6 glu->val

A

b.2 alpha chains and 2 gamma chains

22
Q

What is sickle cell Haemoglobin made up of ?

a. 2 alpha chains + 2 beta chains
b. 2 alpha chains and 2 gamma chains
c. 2 alpha chains and 2 beta chains 6 glu->val

A

c. 2 alpha chains and 2 beta chains 6 glu->val

23
Q

the affinity of fetal Hb for O2 is ….. than the affinity of maternal Hb for O2?

a. lower
b. greater
c. the same

A

b.greater

24
Q

in sickle cell anaemia what causes the haemoglobinopathy?

a. mutations in Hb genes leading to altered amino acids, so altered properties of the Hb protein chains
b. defective regulation of Hb gene expression , altered ratios of the Hb chains, altered properties of the complex

A

a.mutations in Hb genes leading to altered amino acids, so altered properties of the Hb protein chains

25
Q

in thalassaemias what causes the haemoglobinopathy?

a. mutations in Hb genes leading to altered amino acids, so altered properties of the Hb protein chains
b. defective regulation of Hb gene expression , altered ratios of the Hb chains, altered properties of the complex

A

b.defective regulation of Hb gene expression , altered ratios of the Hb chains, altered properties of the complex

26
Q

what is the phenotype of people hetrozygous for the sickle cell gene mutation?

a. sickle cell disease
b. normal red blood cells

A

b. normal red blood cells

27
Q

in sickle cell anaemia which chain of the haemoglobin molecule has a point mutation?

a. beta
b. alpha

A

a. beta

28
Q

what is the effect of the point mutation occuring in the B chain of haemoglobin in sickle cell anaemia?

a. Val -> Glu
b. Val -> Gly
c. Glu-> Val
d. Gly-> Val

A

c. Glu-> Val

29
Q

what is the effect of glu being switched for val ?

a. an acidic non polar residue becomes hydrophobic
b. an acidic polar residue becomes hydrophobic
c. an alkanline polar residue becomes hydrophillic
d. an alkaline polar residue becomes hydrophobic

A

b.an acidic polar residue becomes hydrophobic

30
Q

what is the effect of an acidic polar residue in the Hb B protein chain becoming hydrophobic?

a. 6 th amino acid switched from Glu -> val
b. small sticky patches form on the surface of the B chains that cause them to polymerise and form fibres
c. b.small sticky patches form on the surface of the B chains that cause them to break down

A

b.small sticky patches form on the surface of the B chains that cause them to polymerise and form fibres

31
Q

At which amino acid does the point mutation occur in the B chain that causes sickle cell anaemia?

a.6th
b5th
c.4th
d.3rd

A

a.6th

32
Q

What causes the sickle shape of RBC in sickle cell anaemia?

a. fibres of polymerised B cells being broken down
b. fibres of polymerised B cells breaking off to form platelets
c. HbS fibres distorting the shape of the RBC

A

c.HbS fibres distorting the shape of the RBC

33
Q

How do sickle cells lead to anaemia?

A

cells more rigid so block capillaries and have a shorter life span

34
Q

Hb SS represents which state?

a. adult Haemoglobin
b. adult homozygous for sickle cell
c. adult hetrozygous for sickle cell

A

b. adult homozygous for sickle cell

35
Q

Hb AS represents which state?

a. adult Haemoglobin
b. adult homozygous for sickle cell
c. adult hetrozygous for sickle cell

A

c.adult hetrozygous for sickle cell

36
Q

At what age does sickle cell disease manifest?

a. 2 years
b. 3 months
c. 12 months
d. 6 months

A

d. 6 months

as the normal HbF decreases to adult levels

37
Q

what happens to sickle cells in low O2 environments?

a. cells die
b. altered affinity for one another and form filaments
c. b.small sticky patches form on the surface of the B chains that cause them to break down

A

b. altered affinity for one another and form filaments

38
Q

where are sickle shaped red cells destroyed?

a. kidney
b. liver
c. spleen

A

c.spleen

39
Q

patient with spenomegaly (enlarged spleen) tests show patient is anaemic. what is the most likley diagnosis?

a. sickle cell anaemia
b. iron deficiency anaemia
c. thalassaemia

A

a.sickle cell anaemia

40
Q

Which of these is not an effect of a patient with sickle cell being in a low O2 environment?

a. vessels clocked causing blockage and poor tissue viability
b. splenomegaly
c. bone marrow goes into overdrive to make enough RBC (hyperplasia)
d. sickle cells decrease further in size and deformed shape

A

d. sickle cells decrease further in size and deformed shape

BCR 1 (60 decks)

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