Porphp Flashcards
1
Q
Porphyrins
A
pigments containing a ring of 4
2
Q
ALA
A
3
Q
PBG
A
4
Q
Sites of Heme Biosynthesis
A
5
Q
How is heme biosynthesis regulated?
A
ALA synthase
6
Q
Heme biosynthesis pathway
A
7
Q
What triggers acute porphyria attacks?
A
Uncontrolled up regulation of ALAS1
- -> lipophilic drugs and chemicals
- -> def of glucose and heme
8
Q
What converts Glycine and Succinate into ALA
A
ALAS1
9
Q
ALA and PBG are:
A
highly water soluble
- -> determined by number of carboxyl groups (-COOH)
- —> they have none
10
Q
Where would you find ALA and PBG
A
Urine and Plasma
not in stool
11
Q
Which precursor can be found in urine, stool and plasma?
A
Coproporphyrin
12
Q
Which precursor is water insoluble?
A
Protoporphyrin
Only found in stool and plasma
13
Q
Soret Band
A
strong absorbance at 400 nm
14
Q
Red fluorescence at:
A
exposure at 366 nm
Red-orange fluorescence at 550-650 nm
15
Q
Classification of Porphyrias
A
LOCATION of enz deficiency
- Hepatic
- Erythropoietic
CLINICAL PRESENTATION
- Acute (sudden, severe)
- Chronic (gradual, cutaneous)
25
Q
A
