pyruvate carboxylase deficiency is an inherited metabolic disorder where anaplerosis is greatly reduced. Other anaplerortic substrates such as the odd-carbon containing triglyceride Triheptanoin is used to treat this disorder.
There are 4 reactions classed as anaplerotic, yet the production of oxaloacetate from pyruvate has probably the most physiologic importance.
| From | To | Reaction | Notes |
| Pyruvate | oxaloacetate | pyruvate + CO2 + H2O + ATP oxaloacetate + ADP + Pi + 2H+ | This reaction is catalysed by pyruvate carboxylase, an enzyme activated by Acetyl-CoA, indicating a lack of oxaloacetate. Pyruvate can also be converted to L-malate, another intermediate, in a similar way. |
| Aspartate | oxaloacetate | - | This is a reversible reaction forming oxaloacetate from aspartate in a transamination reaction, via aspartate transaminase. |
| Glutamate | α-ketoglutarate | glutamate + NAD+ + H2O NH4+ + α-ketoglutarate + NADH + H+. | This reaction is catalysed by glutamate-dehydrogenase. |
| β-oxidation of fatty acids | succinyl-CoA | - | When odd-chain fatty acids are oxidized, one molecule of succinyl-CoA is formed per fatty acid. The final enzyme is methylmalonyl-CoA mutase. |