Reactome | COQ4 decarboxylates MHDB

COQ4 decarboxylates MHDB

Stable Identifier

R-HSA-2162195

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

MHDB is decarboxylated to DMPhOH

ReviewStatus

5/5

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COQ4 is a zinc-dependent enzyme that catalyzes the decarboxylation step in CoQ biosynthesis (Pelosi et al., 2024 and Nicoll et al., 2024). However, the substrate and the product of the decarboxylation step remain ambiguous since Nicoll et al. reported a decarboxylation of 3-methoxy-4-hydroxy-5-decaprenylbenzoate (MHDB) into 2-methoxy-6-decaprenylphenol (DMPhOH), whereas Pelosi et al. proposed that COQ4 catalyzes an oxidative decarboxylation which yields a hydroxylated product. In this case, DHB rather than MHDB was proposed a substrate for COQ4, generating 3-polyprenyl-4-hydroxyphenol as a product. We are provisionally showing the first reaction until more data is available.

Literature References

PubMed ID	Title	Journal	Year
38425362	In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis Nicoll, CR, Alvigini, L, Gottinger, A, Cecchini, D, Mannucci, B, Corana, F, Mascotti, ML, Mattevi, A	Nat Catal	2024
38014142	COQ4 is required for the oxidative decarboxylation of the C1 carbon of Coenzyme Q in eukaryotic cells Pelosi, L, Morbiato, L, Burgardt, A, Tonello, F, Bartlett, AK, Guerra, RM, Ferizhendi, KK, Desbats, MA, Rascalou, B, Marchi, M, Vázquez-Fonseca, L, Agosto, C, Zanotti, G, Roger-Margueritat, M, Alcázar-Fabra, M, García-Corzo, L, Sánchez-Cuesta, A, Navas, P, Brea-Calvo, G, Trevisson, E, Wendisch, VF, Pagliarini, DJ, Salviati, L, Pierrel, F	bioRxiv	2023