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Ueki T, Kaneda Y, Tsutsui H, Nakanishi K, Sawa Y, Morishita R, Matsumoto K, Nakamura T, Takahashi H, Okamoto E, et al

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Importantly, in the cytosol, clearance of 2HG is inefficient due to the limited availability of repair enzymes (D2HGDH and L2HGDH), whereas mitochondrial 2HG can be partially converted back to -KG ( D -2HG formation, impair D2HGDH, and disrupt PHD/EGLN activity, thereby stabilizing HIF-1 and creating a pseudo-hypoxic state ( D -2HG concentrations ( L -2HG over L -MAL