Page last updated: 2024-10-24

4-hydroxyproline catabolic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways resulting in the breakdown of 4-hydroxyproline, C5H9NO3, a derivative of the amino acid proline. [GOC:ai]

4-Hydroxyproline catabolism is a metabolic pathway that breaks down 4-hydroxyproline, a modified amino acid found in collagen and other structural proteins. This process is essential for the recycling of collagen and the production of energy.

The catabolism of 4-hydroxyproline begins with the oxidation of the hydroxyl group by the enzyme 4-hydroxyproline oxidase. This reaction produces delta-1-pyrroline-4-hydroxy-2-carboxylate. Next, the enzyme pyrroline-5-carboxylate dehydrogenase converts delta-1-pyrroline-4-hydroxy-2-carboxylate to glutamate-gamma-semialdehyde. Glutamate-gamma-semialdehyde is then converted to glutamate by the enzyme glutamate-gamma-semialdehyde dehydrogenase.

The glutamate produced in this pathway can be used for various metabolic purposes, including the synthesis of new proteins and the production of energy through the citric acid cycle.

In addition to its role in collagen recycling, 4-hydroxyproline catabolism is also involved in the degradation of other structural proteins, such as elastin and keratin. This process is important for maintaining the integrity of tissues and organs.

Mutations in the genes encoding enzymes involved in 4-hydroxyproline catabolism can lead to various disorders, including hyperprolinemia type II and hyperprolinemia type III. These disorders are characterized by an accumulation of 4-hydroxyproline in the blood and urine.

In summary, 4-hydroxyproline catabolism is a vital process that breaks down 4-hydroxyproline and plays a crucial role in collagen recycling, energy production, and the degradation of other structural proteins.'
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Proteins (1)

ProteinDefinitionTaxonomy
4-hydroxy-2-oxoglutarate aldolase, mitochondrialA 4-hydroxy-2-oxoglutarate aldolase, mitochondrial that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q86XE5]Homo sapiens (human)

Compounds (2)

CompoundDefinitionClassesRoles
chelidamic acid
dipicolinic aciddipicolinic acid : A pyridinedicarboxylic acid carrying two carboxy groups at positions 2 and 6.pyridinedicarboxylic acidbacterial metabolite