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This gene encodes the L protein of the mitochondrial glycine cleavage system. The L protein, also named dihydrolipoamide dehydrogenase, is also a component of the pyruvate dehydrogenase complex, the alpha-ketoglutarate dehydrogenase complex, and the branched-chain alpha-keto acide dehydrogenase complex. Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency.
2610024N01Rik; AI429208; COXPD21; RGD1308283; TARS2; Tarsl1; threonine--tRNA ligase, mitochondrial; Threonyl-tRNA synthetase; threonyl-tRNA synthetase 2, mitochondrial (putative); threonyl-tRNA synthetase, mitochondrial; threonyl-tRNA synthetase-like 1; ThrRS
150 µL
100 µg
100 µL
100 µL
50 µg
100 µL
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