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Chem 2 Exam 3 > Porphyrins and Hemoglobin > Flashcards

Porphyrins and Hemoglobin Flashcards

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1
Q

porphyrin basic structure

A

Cyclic
4x pyrrole rings
joined by methine bridges
Intermediates in heme synthesis. myoglobin, cytochromes

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2
Q

Porphyrias

A

Rare disorders resulting from disturbances in heme synthesis

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3
Q

Defects
Hemoglobinothapthies vs. thalaseemias

A

Hemoglobinopathies: Qualitative defects in the molecules
Thalassemias: Quantitative defects in the molecules

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4
Q

Myoglobin found in..

A

Cardiac and skeletal muscle tissues

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5
Q

Porphyrin isomers

A

4 isomers per porphyrin compound
type I/II occur naturally
Type III forms Heme
Sometimes Type I found in excess

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6
Q

Porphyrin color

A

Photoactive
absorb @ 400 nm/emit @ 600-650 nm

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7
Q

Uroporphyrin

A

8 carboxylic groups
Most soluable
Excreted renally

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8
Q

Coproporphyin

A

4 carboxylic acid constituents
intermediate solubility
blood, urine, feces

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9
Q

Heme made per ALA

A

1 heme per 8 ALA

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10
Q

reaction locations for heme production

A

First and last 3: mitochondrion
all others: cytosol

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11
Q

Heme controlled through __________

A

negative feedback loop

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12
Q

Neuropsychiatric symptoms

A

Ab. pain, nausea, hypertension, paresthesia, etc
Excess of early precursors (ALA, PBG)
Porphyrias: ADP, AIP

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13
Q

Cutaneous symptoms

A

Excess porphyrin intermediates
Conditions: CEP, PCT, EPP,XLP
Fragility of skin: CEP/PCT
Burning of skin: EPP/XLPP

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14
Q

Neurocutaneous symptoms

A

excess precursor/intermediates
porphyrias: HCP, VP

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15
Q

Acute porphyrias

A

Serious acute attacks
ADP, AIP: Neuropsychiatric
HCP, VP: neurocutaneous
AIP most common

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16
Q

Non-acute porphyrias

A

CEP,PCT, EPP, XLPP
PCT most common
CEP - extreme photosensitivity

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17
Q

Acute porphyrias testing

A

PBG in urin
if pos -> measure PBG to ID AIP
Fecal for AIP, VP, HCP

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18
Q

Cutaneous Porphyrias

A

Porphyrin in random/timed urine
urine -> PCT/CEP
Plasma/whole blood -> EPP/XLPP

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19
Q

Watson-Schwartz assay

A

Ehrlich’s reagent
max of 555nm

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20
Q

HPLC

A

Porphyrin intermediates in urine

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21
Q

Chromatographic analysis

A

IDs CEP

22
Q

fecal HPLC

A

differentiates acute AIP, HCP, VP

23
Q

Hemoglobin alpha genes

A

Chromosome 19

24
Q

Hemoglobin non-alpha genes

A

chromosome 11

25
Q

Hgb Lepore

A

Delta beta globin chain

26
Q

HGB Kenya

A

Gama beta globin chain

27
Q

Hemoglobinopathy Groups
Amino acid sub

A

Hgb S, C ,D, E, O, G

28
Q

Hemoglobinopathy Groups
Amino acid deletion

A

3 or multiples of 3 nucleotides
Hgb Gun Hill

29
Q

Hemoglobinopathy Groups
Elongated globin chains

A

Chain termination, frame shift
Constant Spring

30
Q

Hemoglobinopathy Groups
fused or hybrid chain

A

nonhomologous crossover
Lepore and Kenya

31
Q

normal heme synthesis

A

Heme in the mitochondria
globin in the cytoplasm
adequate iron supply

32
Q

Hemoglobin S

A

6th position glutemic acid swapped for valine
HbAS: sickle cell trait
HbSS: sickle cell disease

33
Q

Hbg S testing

A

Solubility test (nonspecific)
Cellulose acetate with alkaline pH

34
Q

Hbg C

A

6th position swapped for lysine
Target cells and crystal structures

35
Q

Hgb C testing

A

Cellulose acetate. Moves with Hgb A2
Negative solubuility test

36
Q

Hgb SC

A

Most common mixed hemoglobinopathy
No normal B chains
bird cells

37
Q

Hgb SC testing

A

Pos solubility testing
Cellulose acetate - equals amounts Hgb S and C

38
Q

Hgb E

A

26th position swap of lysine for glut. acid
Microcytes and target cells

39
Q

Hgb E testing

A

Cellulose acetate - E moves with A2, C, O
Citrate agar - E migrates w/ A

40
Q

Hgb D

A

121st position glysine subbed for glut. acid

41
Q

Hgb D testing

A

Cellulose acetate - D migrates w/ S
citrate agar - D migrates w/ A

42
Q

Thalassemia

A

Reduced production of chains, but normal function

43
Q

Thal Minor

A

Heterozygous, asymptomatic, looks like iron deficiency

44
Q

Thal Major

A

Usually lethal before birth or shortly after

45
Q

Alpha thals

A

Chromosome 16, total of 4 genes
Hydrop Fetalis - most severe. Hgb Bartz, tot. absence of a-chain. Stillborn
Hgb H - a chain synth , .5 B chain synth. Moderate hemolytic anemia
a-thal trait - two gene deletions. Micro/hypo anemia
silent carrier - missing 1 gene. normal production

46
Q

B-thal

A

caused by gene mutation vice deletions
homozygous - Cooley’s anemia
B+ -> reduced chain prod
B0 -> no B-chains

47
Q

Analytical methods for porphyrins

A

Cellulose acetate EP - alkaline buffer (8.4-8.6)
Citrate agar - acid pH (6-6.2)
Hgb A2 quantification
Acid Elution for Hgb F - the baby vs mom staining

48
Q

DNA testing

A

Definitive test for thals and hemopath.
Specifics:
gap-PCR
HRM
MLPA
ASO
SSCP
NGS

Don’t worry too much about memorizing just kinda toss it around in the ol’ noggin

49
Q

Review EP slides

A

Just do it. Slides 46/47

50
Q

Myoglobin

A

Skeletal and cardiac muscle
only release on low O2 Tension
1 polypeptide and 1 heme group
main role: xport O2 from muscle membrane to mitochondria

51
Q

Utility os myoglobin testing

A

muscle (cardiac) damage
Rhabdomylosis
MI
muscular dystrophy

Chem 2 Exam 3 (6 decks)

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