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Hema - Midterms > Hemoglobin > Flashcards

Hemoglobin Flashcards

1
Q

Components of hemoglobin

A

4 heme groups and 2 heterogenous pairs of polypeptide chains

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2
Q

Concentration of hemoglobin in rbc

A

34 g/dL

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3
Q

Hemoglobin carries how many ferrous ions

A

2

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4
Q

1 g of hemoglobin can carry how many mL of oxygen?

A

1.34 mL

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5
Q

It is the main respiratory pigment and main component seen in RBC

A

Hemoglobin

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6
Q

Functions of hemoglobin

A
  • transport O2 from lungs to tissue
  • transport CO2 from tissue to lungs
  • contribute to acid base balance by binding and releasing hydrogen ions
  • modulates vascular dilation by transporting NO
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7
Q

True or false

One heme group is embedded in each polypeptide chain

A

True

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8
Q

A qualitative disorder that pertains to the variation / mutation within amino acid sequence

A

Hemoglobinopathies

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9
Q

A quantitative disorder that pertains to a gene defect that don’t code for a globin chain

A

Thalassemia

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10
Q

Range of amino acids that a globin chain contains

A

141 - 146

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11
Q

Each globin chain is divided into

A

8 helices and 7 non helical segments

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12
Q

True or false

The hemoglobin molecule can be described by its protein stucture

A

True

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13
Q

A predominant adult Hb that is composed of 2 alpha and 2 beta globin chain

A

Hb A

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14
Q

Globin chains loop to form a ________ for heme

A

Cleft pocket

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15
Q

Shape of a hemoglobin molecule

A

Spherical

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16
Q

Hemoglobin can carry up to how many O2,?

A

4

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17
Q

Hemoglobin that has a sugar attached js known as

A

Glycated

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18
Q

True or false

Small percentage of Hb F is glycated

A

False

Hb A

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19
Q

Chain arrangements in helices and non helices

A

Secondary structure

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20
Q

Combination of amino acid sequence and polypeptide chains

A

Primary structure

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21
Q

Arrangement of helices in a pretzel-like configuration

A

Tertiary structure

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22
Q

Heme biosynthesis occurs in the

A

Mitochondria and cytoplasm of rbc bone marrow precursor

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23
Q

True or false

The loss of mitochondria and ribosomes results to the cessation of hemoglobin synthesis

A

True

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24
Q

Give the enzymes responsible for heme biosynthesis in order

A 
ALA synthase
ALA dehydratase
PBG deaminase
Uroporphyrinogen III cosynthase
Uroporphyrinogen III decarboxylase
Corpophyrinigen oxidase
Protoporphyrinogen IX oxidase
Ferrochelatase
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25
It is also called the heme synthase
Ferrochelatase
26
Ion added to the heme synthase to make heme
Ferrous ion
27
Where does heme biosynthesis occurs
Mitochondria and cytoplasm
28
Transferrin bound to transferrin receptor are brought in the cell in a form of
Endosome
29
Alpha and zeta globin chain can be found in what chromosome
Chromosome 16
30
Chromosome 11 is associated with what globin chains?
Beta gamma delta epsilon
31
Production of globin biosynthesis occurs in
Eryhthroid precursor
32
Acute intermittent porphyria is associated with this enzyme
PBG deaminase
33
Ferrochelatase can cause what disease?
Erythropoietin protoporphyria
34
What disease is assoc with coproporphyrinogen?
Hereditary coproporphyria
35
Uroporphyrinogen III decarboxylase can be assoc with what disease
Porphyria cutanea tarda
36
Deficiency in specific enzymes involved in heme synthsesis
Porphyria
37
Where does the heme attach in the globin chains?
Cleft pocket
38
Disease with protoporphyrinogen oxidase and the product it produce
Variegate porphyria | Protoporphyrin IX
39
Enumerate in order the the chain with from highest affinity to lowest affinity
Alpha gamma delta
40
Combination of 2 heterodimer
Tetramer
41
Completes the hemoglobin molecule
Tetramer
42
Composition of heterodimer
Globin chain and heme molecule
43
Globin chains of Hb A
2 alpha and 2 beta
44
Globin chains that hold the dimers in stable
Alpha 1 - beta 1 and alpha 2 - beta 2 bonds
45
Alpha 1 - beta 2 and alpha 2 - beta 1 bond is important for
Stability of the quaternary structure
46
Small portion of Hb A that is glycated
Hb A1c
47
Disease assoc. with glycated hemoglobin
Uncontrolled diabetes mellitus
48
2 alpha and 2 delta globin chains comprise what Hb
Hb A2
49
F or A/F cells are what?
Small portion of RBCs with Hb F
50
Embryonic hemoglobins:
Gower 1, Gower 2, Portland
51
Predominant Hb in the 2nd and 3rd trimester
Hb F
52
Hb in adulthood
Hb A Hb F Hb A2
53
Hydroxymethlybilane sythase is acted on what reaction
PBG to hydroxymethylbilane
54
<3.5% of Total Hb is what Hb?
Hb A2
55
Embryonic hemoglobin is formed by the combination of these globin chains
Alpha and gamma globin chains and zeta and epsilon globin chains
56
Heme inhibits
ALA synthase Ferrochelatase ALA dehydratase PBG deaminase
57
Ferrochelatase is inhibited by
Heme and protoporphyrin IX
58
Abnormal production if alpha chain
Alpha thalassemia
59
Excess globin chains, protoporphyrin IX and iron can cause
Damage to the cell leading to reduce life span
60
Regulator role in heme synthesis
Ferrochelatase
61
Key regulating step
Glycine + succinyl coenzyme a —> ALA
62
DNA Sequences in globin production
Cis acting promoters, enhancers, silencers
63
Binds to DNA or one another to promote or suppress transcription
Trans acting factor
64
Inhibition of transcription of a particular globin gene requires:
``` Promoter DNA sequences Krüppel like factor 1 TAL 1 GATA1 LDB 1 Ikaros P45 NF E2 Locus control region ```
65
Key regulator in gamma beta switching
Krüppel like factor 1
66
Repressors of gamma globin gene transcription
BCL11A | MYB
67
Ferric oxidation product of heme
Hemin
68
Components of Hb that reduce RBC survival when in excess
Unpaired chains Protoporphyrin Iron
69
Controls the rate of globin synthesis
Hemin
70
Absence of hemin will lead to
Inhibitor accumulation
71
1 gram hemoglobin eqauls to
1.34 ml of oxygen
72
1 mRNA =
1 globin
73
Tissue hypoxia occurs in
Insufficient hemoglobin | Defect of hemoglobin in carrying oxygen
74
Tissue hypoxia is detected by
Peritubular cells in kidney
75
High or low affinity for oxygen To transport oxygen: To unload oxygen:
High | Low
76
Hahahahaha
Funny
77
Graphic expression of hemoglobin
Oxygen dissociation curve of hemoglobin
78
The curve in oxygen dissociation curve is termed
Sigmoidal
79
Hugh
Jackman
80
High hemoglobin affinity for oxygen at _______ oxygen tension
High
81
Shift in curve due to change in hydrogen ion (pH change)
Bohr effect
82
``` Shift to left or right Lower oxygen affinity: High pH Low body temp Presence of Hb variants with high affinity for oxygen Acidosis ```
``` Right Left Left Left Right ```
83
Tense confirmation has oxygenated or deoxygenated hemoglobin?
Deoxygenated
84
R state or relax state hax oxygenated or deoxygenated hemoglobin?
Oxygenated
85
MW of myoglobin
17,000 Dalton
86
Myoglobin release in plasma is what disease
Rhabdomyolysis
87
Facilitates the reaction of producing carbonic acid
Carbonic anhydrase
88
Production of carbonic acid occurs in
RBC
89
Ion that diffuses in to cell form plasma to maintain neutrality across membrane
Chloride

Hema - Midterms (1 decks)

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