Cargando…

4‐Hydroxybenzoic acid restores CoQ(10) biosynthesis in human COQ2 deficiency

The clinical phenotypes of human CoQ(10)‐deficiency caused by COQ2 mutations range from fatal neonatal disease to adult‐onset multisystem atrophy. So far, treatment options for these diseases are unsatisfactory. Here, we demonstrate that supplementation of 4‐hydroxybenzoic acid (4‐HBA) fully restore...

Descripción completa

Detalles Bibliográficos
Autores principales:	Herebian, Diran, Seibt, Annette, Smits, Sander H. J., Rodenburg, Richard J., Mayatepek, Ertan, Distelmaier, Felix
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	John Wiley and Sons Inc. 2017
Materias:	Brief Communications
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5740244/ https://www.ncbi.nlm.nih.gov/pubmed/29296619 http://dx.doi.org/10.1002/acn3.486

Ejemplares similares

Human COQ9 Rescues a coq9 Yeast Mutant by Enhancing Coenzyme Q Biosynthesis from 4-Hydroxybenzoic Acid and Stabilizing the CoQ-Synthome
por: He, Cuiwen H., et al.
Publicado: (2017)

Lethal Neonatal CoQ Deficiency due to a COQ9 Variant
por: Finsterer, Josef, et al.
Publicado: (2018)

Manganese-driven CoQ deficiency
por: Diessl, Jutta, et al.
Publicado: (2022)

CoQ10 and Aging
por: de Barcelos, Isabella Peixoto, et al.
Publicado: (2019)

CoQ(10) Deficient Endothelial Cell Culture Model for the Investigation of CoQ(10) Blood–Brain Barrier Transport
por: Wainwright, Luke, et al.
Publicado: (2020)

COQ4 Mutations Cause a Broad Spectrum of Mitochondrial Disorders Associated with CoQ(10) Deficiency
por: Brea-Calvo, Gloria, et al.
Publicado: (2015)

Characterization of Arabidopsis thaliana Coq9 in the CoQ Biosynthetic Pathway
por: Hu, Mei, et al.
Publicado: (2023)

Treatment of CoQ(10) Deficient Fibroblasts with Ubiquinone, CoQ Analogs, and Vitamin C: Time- and Compound-Dependent Effects
por: López, Luis C., et al.
Publicado: (2010)

Novel recessive mutations in COQ4 cause severe infantile cardiomyopathy and encephalopathy associated with CoQ(10) deficiency
por: Sondheimer, Neal, et al.
Publicado: (2017)

A fatal case of COQ7‐associated primary coenzyme Q(10) deficiency
por: Kwong, Anna K.‐Y., et al.
Publicado: (2019)

Effects of CoQ10 Replacement Therapy on the Audiological Characteristics of Pediatric Patients with COQ6 Variants
por: Nam, Dong Woo, et al.
Publicado: (2022)

Identification of novel coenzyme Q(10) biosynthetic proteins Coq11 and Coq12 in Schizosaccharomyces pombe
por: Nishida, Ikuhisa, et al.
Publicado: (2023)

The Important Role of CoQ(10) in Aging
por: López-Lluch, Guillermo
Publicado: (2019)

Effects of Inhibiting CoQ(10) Biosynthesis with 4-nitrobenzoate in Human Fibroblasts
por: Quinzii, Catarina M., et al.
Publicado: (2012)

Balanced CoQ(6) biosynthesis is required for lifespan and mitophagy in yeast
por: González-Mariscal, Isabel, et al.
Publicado: (2017)

Vanillic Acid Restores Coenzyme Q Biosynthesis and ATP Production in Human Cells Lacking COQ6
por: Acosta Lopez, Manuel J., et al.
Publicado: (2019)

Cellular Models for Primary CoQ Deficiency Pathogenesis Study
por: Santos-Ocaña, Carlos, et al.
Publicado: (2021)

Tit-Coq /
por: Gélinas, Gratien
Publicado: (1981)

Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q(10) biosynthesis()
por: Nguyen, Theresa P.T., et al.
Publicado: (2014)

CoQ10-related sustained remission of proteinuria in a child with COQ6 glomerulopathy—a case report
por: Stańczyk, Małgorzata, et al.
Publicado: (2018)

Mitochondrial Disease and the Kidney With a Special Focus on CoQ(10) Deficiency
por: Schijvens, Anne M., et al.
Publicado: (2020)

Evaluation of Coenzyme Q10 (CoQ10) Deficiency and Therapy in Mouse Models of Cardiomyopathy
por: Pu, Tian-Tian, et al.
Publicado: (2023)

CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome
por: Luna‐Sánchez, Marta, et al.
Publicado: (2016)

Fluorene intercalated graphene oxide based CoQ10 imprinted polymer composite as a selective platform for electrochemical sensing of CoQ10
por: Soomro, Anam Naz, et al.
Publicado: (2022)

The Role of Sulfide Oxidation Impairment in the Pathogenesis of Primary CoQ Deficiency
por: Quinzii, Catarina M., et al.
Publicado: (2017)

The Q-junction and the inflammatory response are critical pathological and therapeutic factors in CoQ deficiency
por: González-García, Pilar, et al.
Publicado: (2022)

CoQ10 Deficiency May Indicate Mitochondrial Dysfunction in Cr(VI) Toxicity
por: Zhong, Xiali, et al.
Publicado: (2017)

Primary coenzyme Q10 deficiency due to COQ8A gene mutations
por: Zhang, Linwei, et al.
Publicado: (2020)

Formation of Temporary Negative Ions and Their Subsequent Fragmentation upon Electron Attachment to CoQ(0) and CoQ(0)H(2)
por: Ameixa, João, et al.
Publicado: (2022)

Intragenic suppressor mutations of the COQ8 protein kinase homolog restore coenzyme Q biosynthesis and function in Saccharomyces cerevisiae
por: Awad, Agape M., et al.
Publicado: (2020)

Mechanisms and Therapeutic Effects of Benzoquinone Ring Analogs in Primary CoQ Deficiencies
por: Pesini, Alba, et al.
Publicado: (2022)

COQ8A-Ataxia as a Manifestation of Primary Coenzyme Q Deficiency
por: Paprocka, Justyna, et al.
Publicado: (2022)

Liposome/gold hybrid nanoparticle encoded with CoQ10 (LGNP-CoQ10) suppressed rheumatoid arthritis via STAT3/Th17 targeting
por: Jhun, Jooyeon, et al.
Publicado: (2020)

β‐RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9 (R239X) mice
por: Hidalgo‐Gutiérrez, Agustín, et al.
Publicado: (2018)

Mitochondrial CoQ deficiency is a common driver of mitochondrial oxidants and insulin resistance
por: Fazakerley, Daniel J, et al.
Publicado: (2018)

Effect of vanillic acid on COQ6 mutants identified in patients with coenzyme Q(10) deficiency()
por: Doimo, Mara, et al.
Publicado: (2014)

Self-reported adherence and biomarker levels of CoQ10 and alpha-tocopherol
por: Vitolins, Mara Z, et al.
Publicado: (2018)

Le coq de bruyére /
por: Tournier, Michel
Publicado: (1978)

The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains functional by metalation with cobalt
por: Wang, Ying, et al.
Publicado: (2022)

Propargylation of CoQ0 through the Redox Chain Reaction
por: Pawlowski, Robert, et al.
Publicado: (2021)

Cannot write session to /tmp/vufind_sessions/sess_3j0shmn22aeaa7ukbi1bev4vsc