Oxidative stress in branched-chain organic acidemias using thiol-disulfide homeostasis

The parameters of the TDH are altered in patients with OA, especially in those with MMA and PA, indicating a significant level of oxidative stress. The neurodevelopmental outcome in OA may be exacerbated by this oxidative imbalance. The utilization of TDH as a biomarker may be a potential provider of oxidative status in OA patients and facilitate informing therapeutic strategies.

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Publication Details

Journal
Journal of Pediatric Endocrinology and Metabolism
Published
2025-05-30
DOI
https://doi.org/10.1515/jpem-2025-0184
Primary Topic
Metabolism and Genetic Disorders
Type
article
Field-Weighted Citation Impact
0.00
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article

Oxidative stress in branched-chain organic acidemias using thiol-disulfide homeostasis

Hakim Çelık, Emine Pektaş, Mehmet Keskın, Burcu Kumru Akın
Journal of Pediatric Endocrinology and Metabolism
Metabolism and Genetic Disorders
article

Oxidative stress in branched-chain organic acidemias using thiol-disulfide homeostasis

Hakim Çelık, Emine Pektaş, Mehmet Keskın, Burcu Kumru Akın
article en

Abstract

The parameters of the TDH are altered in patients with OA, especially in those with MMA and PA, indicating a significant level of oxidative stress. The neurodevelopmental outcome in OA may be exacerbated by this oxidative imbalance. The utilization of TDH as a biomarker may be a potential provider of oxidative status in OA patients and facilitate informing therapeutic strategies.

Journal of Pediatric Endocrinology and MetabolismVol. 38(8)
Gaziantep Children's Hospital (TR), Gaziantep University (TR), Adnan Menderes University (TR), Harran University (TR)
Openalex Percentile: Top 85%
Metabolism and Genetic Disorders
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