8. carbohydrate metabolism 2 Flashcards
where does glycolysis occur?
in the cytosol
where is the TCA cycle located
matrix of mitochondria
what is the first thing that happens before the TCA cycle?
pyruvate forms acetyl coA by oxidative decarboxylation
pyruvate dehydroganse cataylses
Importance of PDH reaction
- Commits pyruvate to TCA cycle
- Controls the entry of glucose to TCA cycle
- Rate limiting step
- Irreversible
- Regulated
–Allosterically
–Covalently
–Hormonally (insulin activates)
Pyruvate Dehydrogenase structure
- Multienzyme complex
- Molecular mass 4 - 10 million daltons big
- Consists of 3 enzyme complexes
- 5 coenzymes
3 enzyme activities of PDH
- E1 - Pyruvate decarboxylase
- E2 - Dihydrolipoyl transacetylase
- E3- Dihydrolipoyl dehydrogenase
5 coenzymes of PDH
•Thiamine pyrophosphate (TPP) thiamine (vitamin B1)
•Lipoamide: lipoic acid - 10-carbon fatty acid with sulphydryl groups on C8 and C10
•CoA: pantothenic acid (vit B5)
•FAD+: riboflavin (vit B2). Prosthetic group bound covalent to E3 protein
•NAD+: niacin (vit B3)
3 medical problems associated with PDH
- Beri-beri (thiamine deficiency)
–Damage to peripheral nervous system and weakened muscle – CV disorders.
–Far East where polished rice is major component of diet.
2.Mercury and arsenite poisoning. Binds to dihydrolipoyl groups on E2. CNS pathologies.
•Vitamin deficiencies - riboflavin and niacin are components of FAD and NAD
3.Pyruvate Dehydrogenase deficiency genetic
overall molecules produced from one molecule of acetyl coA oxidised
3 NADH + 1 FADH2 +2CO2 + 1GTP
(4 pairs of electrons)
not a lot of control
medical problems associated with TCA cycle
none- major defect dont survive