Coenzyme Q supports distinct developmental processes in Caenorhabditis elegans

Invertebrate Models for Coenzyme Q₁₀ Deficiency

“…Coq-8 knockouts do not present differences in longevity in respect to the wild-type worm if fed a CoQ-rich diet with wild-type E. coli , although the lifespan is reduced to a half if they are fed the CoQ-deficient GD1 bacteria [Asencio et al, 2009]. However, no improvement of fertility was observed with a CoQ-rich diet, but embryo production was increased, and most of the embryos completed the development if uridine was added simultaneously to the plates containing CoQ-repleted E. coli, indicating that the pyrimidine nucleotide pathway necessary for DNA synthesis (and the subsequent embryo arrest after fertilization) cannot be restored by external CoQ [Asencio et al, 2009].…”

“…However, no improvement of fertility was observed with a CoQ-rich diet, but embryo production was increased, and most of the embryos completed the development if uridine was added simultaneously to the plates containing CoQ-repleted E. coli, indicating that the pyrimidine nucleotide pathway necessary for DNA synthesis (and the subsequent embryo arrest after fertilization) cannot be restored by external CoQ [Asencio et al, 2009].…”

“…First-generation homozygous coq-8 mutants show developmental delay, decreased fertility, and both extracellular matrix degradation and severe abnormalities in the hypodermis, suggesting that it is detached from muscle cells [Asencio et al, 2009]. Most of the embryos produced are arrested between the second and eighth cell division after fertilization, and their second-generation progenies, which lack the maternal inherited CoQ 9 , become sterile because they do not properly develop gonads.…”

Coenzyme Q10 Deficiencies in Neuromuscular Diseases

“…There is no current demonstration of the exact role of coq-8 in CoQ biosynthesis, but based on sequence similarity, coq-8 belongs to a protein kinase family harboring an ATP-binding cassette similar to those found in regulatory kinases [30]. C. elegans coq-8 knockout animals showed embryo development arrest that coincided with the triggering of expression of coq-8 in embryo, and these animals are the only coq knockout strain that maintain a basal CoQ biosynthesis level [4]. This gene encodes probably a regulatory protein of the CoQ biosynthesis complex through a phosphorylation mechanism.…”

“…COQ8 gene, also called ADCK3 or CABC1 , function is still unknown in CoQ biosynthesis pathway, although it has been proposed that could act as a protein kinase, since several kinase motifs have been found in its amino acid sequence [30], and it has been demonstrated to have regulatory functions in C. elegans [4]. In 2008, 2 groups reported independently and simultaneously mutations in this gene associated with CoQ deficiency and an ataxic form with seizures.…”