29 results on '"Karunanidhi, Anuradha"'
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2. Messenger RNA rescues medium-chain acyl-CoA dehydrogenase deficiency in fibroblasts from patients and a murine model
3. EFFECT OF TRIHEPTANOIN AND MEDIUM BRANCHED-CHAIN FATTY ACIDS ON LYSINE SUCCINYLATION IN HUMAN MCAD DEFICIENT FIBROBLAST CELLS AND MURINE KO MODEL
4. ANAPLEROTIC COMPOUNDS IMPROVE O CONSUMPTION AND RESTORE SUCCINYLLYSINE ANTIGENIC SIGNAL IN LONG-CHAIN FATTY ACID OXIDATION DEFICIENT CELLS
5. ELAMIPRETIDE RESTORES MITOCHONDRIAL FUNCTION IN TRIFUNCTIONAL PROTEIN DEFICIENCY MICE AND HUMAN FIBROBLASTS
6. A multiomics approach to understanding pathology of Combined D,L-2- Hydroxyglutaric Aciduria and phenylbutyrate as potential treatment
7. Heptanoic and Medium Branched-Chain Fatty Acids as Anaplerotic Treatment for Medium Chain Acyl-CoA Dehydrogenase Deficiency
8. Synthetic mRNA rescues very long-chain acyl-CoA dehydrogenase deficiency in patient fibroblasts and a murine model
9. ITCH deficiency clinical phenotype expansion and mitochondrial dysfunction
10. Treatment of VLCAD-Deficient Patient Fibroblasts with Peroxisome Proliferator-Activated Receptor δ Agonist Improves Cellular Bioenergetics
11. Treatment of VLCAD deficient patient fibroblasts with peroxisome-proliferator activated receptor δ agonist improves cellular bioenergetics
12. HEPTANOATE AND DERIVATIVES AS THERAPY FOR MCAD DEFICIENCY: PRECLINICAL EVIDENCE OF EFFICACY AND IMPLICATIONS FOR ANAPLEROTIC DRUG CLASS THERAPY
13. Mitochondrial dysfunction associated with TANGO2 deficiency
14. Medium branched chain fatty acids improve the profile of tricarboxylic acid cycle intermediates in mitochondrial fatty acid β‐oxidation deficient cells: A comparative study
15. Development and characterization of a mouse model for Acad9 deficiency
16. Characterization of variants of uncertain significance in isovaleryl-CoA dehydrogenase identified through newborn screening: An approach for faster analysis
17. ACAD10 protein expression and Neurobehavioral assessment of Acad10-deficient mice
18. Clinical, biochemical, mitochondrial, and metabolomic aspects of methylmalonate semialdehyde dehydrogenase deficiency: Report of a fifth case
19. Reticular Dysgenesis and Mitochondriopathy Induced by Adenylate Kinase 2 Deficiency with Atypical Presentation
20. ETHE1 and MOCS1 deficiencies: Disruption of mitochondrial bioenergetics, dynamics, redox homeostasis and endoplasmic reticulum-mitochondria crosstalk in patient fibroblasts
21. Mitochondrial energetics is impaired in very long-chain acyl-CoA dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers
22. Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency
23. Investigating the link of ACAD10 deficiency to type 2 diabetes mellitus
24. Complex I assembly function and fatty acid oxidation enzyme activity of ACAD9 both contribute to disease severity in ACAD9 deficiency
25. Long-chain Acyl-CoA Dehydrogenase Deficiency as a Cause of Pulmonary Surfactant Dysfunction
26. Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency
27. Preclinical Toxicology Studies of Recombinant Human Platelet-Derived Growth Factor-BB Either Alone or in Combination with Beta-Tricalcium Phosphate and Type I Collagen
28. Cellular Uptake of Functional Nanogels Prepared by Inverse Miniemulsion ATRP with Encapsulated Proteins, Carbohydrates, and Gold Nanoparticles
29. Response of Bone Subjected to Optimized High Dose Irradiation
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