Methaemoglobin (MetHb)
[Ref: JN5:p272-273; WG21:p536-539; KB:p211]
In MetHb,
Iron is ferric (Fe3+) instead of ferrous (Fe2+)
Characteristics
- Dark coloured
- Unable to combine with oxygen
- Causes cyanosis when MetHb > 1.5g/dL
* Cyanosis only occurs when DeoxyHb > 5g/dL
- Normally MetHb is about 1-2% of total Hb
* ?? Reference
Formation
- When oxyhaemoglobin acts as nitric oxide (NO) scavenger
- Drugs
* e.g. prilocaine, benzocaine, nitrites, dapsone
Reduction
Iron is reduced back to ferrous
--> MetHb converted back to Hb
By 4 systems:
1. NADH-methaemoglobin reductase system
- Present in RBC
- Uses NADH provided by Embden-Meyerhof pathway
- Accounts for 2/3 of MetHb-reducing activities
- Deficient in familial methaemoglobinaemia
2. Ascorbic acid
May slowly reduce the iron by direct chemical effect
Accounts for 16% of MetHb reduction
3. Glutathione-based reductive enzymes
Small amount of MetHb reductase activity
4. NADPH-dehydrogenase enzyme in RBC
- Can reduce MetHb using NADPH from pentose phosphate pathway
- Under normal condition, this system has almost no effect
- Regarded as a reserve mechanism
NB:
[KB2:p137]
In presence of methylene blue, activity of NADPH-MetHb reductase (?? same as NADPH-dehydrogenase) increases
--> NADPH-MetHb reductase reduces methylene blue
--> Reduced form of methylene blue reduces MetHb