Chapter 8: 1.5 - Porphyrin Disorder

Question 1

-intermediate compounds in the production of heme

Answer 1

Porphyrins

Question 2

main sites of porphyrin production (2)

Answer 2

bone marrow and liver

Question 3

porphyrin:

intermediates in the synthesis of hemoglobin is produced where?

Answer 3

bone marrow

Question 4

porphyrin:

intermediates in the synthesis of heme proteins such as myoglobin is produced where?

Answer 4

Liver

Question 5

overproduction of porphyrin intermediates/ or their precursors cause increased:

Answer 5

urinary, fecal and tissue accumulation of this substance.

Question 6

The synthesis of heme can be blocked at a number of stages.

- what is the collective term for disorders that block porhyrin metabolism?

Answer 6

Porphyrias

Question 7

causes of acquired Porphyria:

Answer 7

lead poisoning
excessive alchohol exposure
iron deficiency
chronic liver disease
renal disease

Question 8

Porphyria:
urine color
misc

Answer 8

dark red, wine colored

• red teeth and bones
• skin photosensitivity
• hemolytic anemia w/ splenomegaly

Question 9

Solubility property of porphyrin compound:

most soluble and readily appear in the urine

Answer 9

ALA, porphobilinogen, uroporphyrin

Question 10

Solubility property of porphyrin compound:

less soluble, found in urine

Answer 10

coproporphyrin

Question 11

Solubility property of porphyrin compound:

not seen in the urine

Answer 11

protoporphyrin

Question 12

Porphyria Test: (2)

Answer 12

1. Ehrlich

2. Fluorescence under UV 550-600 nm

Question 13

Porphyria Test:
Fluorsecence
color

Answer 13

violet, pink or red