Coenzyme Q biochemistry and biosynthesis
RM Guerra, DJ Pagliarini - Trends in biochemical sciences, 2023 - cell.com
Coenzyme Q (CoQ) is a remarkably hydrophobic, redox-active lipid that empowers diverse
cellular processes. Although most known for shuttling electrons between mitochondrial …
cellular processes. Although most known for shuttling electrons between mitochondrial …
Structural mechanism of mitochondrial membrane remodelling by human OPA1
A von der Malsburg, GM Sapp, KE Zuccaro… - Nature, 2023 - nature.com
Distinct morphologies of the mitochondrial network support divergent metabolic and
regulatory processes that determine cell function and fate,–. The mechanochemical GTPase …
regulatory processes that determine cell function and fate,–. The mechanochemical GTPase …
Biosynthesis, deficiency, and supplementation of coenzyme Q
C Staiano, L García-Corzo, D Mantle, N Turton… - Antioxidants, 2023 - mdpi.com
Originally identified as a key component of the mitochondrial respiratory chain, Coenzyme Q
(CoQ or CoQ10 for human tissues) has recently been revealed to be essential for many …
(CoQ or CoQ10 for human tissues) has recently been revealed to be essential for many …
COQ4 is required for the oxidative decarboxylation of the C1 carbon of coenzyme Q in eukaryotic cells
Coenzyme Q (CoQ) is a redox lipid that fulfills critical functions in cellular bioenergetics and
homeostasis. CoQ is synthesized by a multi-step pathway that involves several COQ …
homeostasis. CoQ is synthesized by a multi-step pathway that involves several COQ …
Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy
A Jacquier, J Theuriet, F Fontaine, V Mosbach… - Brain, 2023 - academic.oup.com
Distal hereditary motor neuropathy represents a group of motor inherited neuropathies
leading to distal weakness. We report a family of two brothers and a sister affected by distal …
leading to distal weakness. We report a family of two brothers and a sister affected by distal …
Phenotypic, molecular, and functional characterization of COQ7-related primary CoQ10 deficiency: Hypomorphic variants and two distinct disease entities
P Wongkittichote, MLD Lasio, M Magistrati… - Molecular genetics and …, 2023 - Elsevier
Abstract Primary coenzyme Q10 (CoQ 10) deficiency is a group of inborn errors of
metabolism caused by defects in CoQ 10 biosynthesis. Biallelic pathogenic variants in …
metabolism caused by defects in CoQ 10 biosynthesis. Biallelic pathogenic variants in …
Biallelic variants in the COQ7 gene cause distal hereditary motor neuropathy in two Chinese families
X Liu, N Wang, Y Chen, W Lv, JM Hong, GR Xu… - Brain, 2023 - academic.oup.com
It was with great interest that we read the article published by Jacquier et al. 1 regarding
autosomal recessive distal hereditary motor neuropathy (dHMN) caused by a pathogenic …
autosomal recessive distal hereditary motor neuropathy (dHMN) caused by a pathogenic …
New variants expand the neurological phenotype of COQ7 deficiency
MA Fabra, AJ Paredes‐Fuentes… - Journal of Inherited …, 2024 - Wiley Online Library
The protein encoded by COQ7 is required for CoQ10 synthesis in humans, hydroxylating 3‐
demethoxyubiquinol (DMQ10) in the second to last steps of the pathway. COQ7 mutations …
demethoxyubiquinol (DMQ10) in the second to last steps of the pathway. COQ7 mutations …
An ETFDH-driven metabolon supports OXPHOS efficiency in skeletal muscle by regulating coenzyme Q homeostasis
JC Herrero Martín, B Salegi Ansa, G Álvarez-Rivera… - Nature …, 2024 - nature.com
Coenzyme Q (Q) is a key lipid electron transporter, but several aspects of its biosynthesis
and redox homeostasis remain undefined. Various flavoproteins reduce ubiquinone …
and redox homeostasis remain undefined. Various flavoproteins reduce ubiquinone …
The structure of the human LACTB filament reveals the mechanisms of assembly and membrane binding
JA Bennett, LR Steward, J Rudolph, AP Voss… - PLoS …, 2022 - journals.plos.org
Mitochondria are complex organelles that play a central role in metabolism. Dynamic
membrane-associated processes regulate mitochondrial morphology and bioenergetics in …
membrane-associated processes regulate mitochondrial morphology and bioenergetics in …