The nucleotide prodrug CERC ‐913 improves mtDNA content in primary hepatocytes from DGUOK‐deficient rats-Reference-Cited by-同舟云学术

The nucleotide prodrug CERC ‐913 improves mtDNA content in primary hepatocytes from DGUOK‐deficient rats

Published:2021-01-15 Issue:2 Volume:44 Page:492-501
ISSN:0141-8955
Container-title:Journal of Inherited Metabolic Disease
language:en
Short-container-title:J of Inher Metab Disea

Author:

Vanden Avond Mark A.¹,Meng Hui¹,Beatka Margaret J.¹,Helbling Daniel C.¹,Prom Mariah J.¹,Sutton Jessica L.¹,Slick Rebecca A.¹,Dimmock David P.²,Pertusati Fabrizio³,Serpi Michaela³,Pileggi Elisa³,Crutcher Patrick⁴,Thomas Stephen⁴,Lawlor Michael W.¹

Affiliation:

1. Department of Pathology and Laboratory Medicine and Neuroscience Research Center Medical College of Wisconsin Milwaukee Wisconsin USA

2. Rady Children's Institute for Genomic Medicine, Rady Children's Hospital San Diego California USA

3. School of Pharmacy and Pharmaceutical Sciences, Cardiff University Cardiff UK

4. Discovery R&D, Cerecor Inc. Rockville Maryland USA

Publisher

Wiley

Subject

Genetics (clinical),Genetics

Link

https://onlinelibrary.wiley.com/doi/pdf/10.1002/jimd.12354

Reference29 articles.

1. Mitochondrial DNA depletion anddGKgene mutations

2. Novel Deoxyguanosine Kinase Gene Mutations in the Hepatocerebral Form of Mitochondrial DNA Depletion Syndrome

3. Deoxyguanosine kinase. Distinct molecular forms in mitochondria and cytosol

4. Mitochondrial DNA depletion can be prevented by dGMP and dAMP supplementation in a resting culture of deoxyguanosine kinase-deficient fibroblasts

5. In vitro supplementation with dAMP/dGMP leads to partial restoration of mtDNA levels in mitochondrial depletion syndromes

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