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Article

Variants in glycine decarboxylase activate catabolic mechanisms of mitochondrial energy metabolism in the brain.

The Journal of biological chemistry - 1 Jun 2026

Lopez-Ramirez Alejandro, Worth Andrew J, Wang Ziyi Lindsay, Yilmaz Saliha, Hayes Sebastian, Tang Hsin-Yao, Farris Joseph, Alam Md Suhail, Padmanabhan Prasad, Haldar Kasturi

Abstract excerpt

Brain energy metabolism is produced from glucose by mitochondrial oxidative phosphorylation. Variants in the mitochondrial enzyme glycine decarboxylase (GLDC) cause a rare neurological disease, nonketotic hyperglycinemia, with expected hallmarks of brain glycine elevation and responsiveness to folate deficiency but the consequences for energy mechanisms remain unknown. We find that brains of young-attenuated...

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Identifiers

PMID: 42055339
DOI: 10.1016/j.jbc.2026.113098
PMCID: PMC13235479

Genes

GLDC
GCSH

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 8 Sept 2026.

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