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Catalyzes the transfer of endogenously produced octanoic acid from octanoyl-acyl-carrier-protein onto the lipoyl domains of lipoate-dependent enzymes. De plus, nous expédions LIPT2 Protéines (2) et beaucoup plus de produits pour cette protéine.
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The authors report on the identification of biallelic LIPT2 mutations in three affected individuals from two families with severe neonatal encephalopathy where a normalization of lipoylation was observed after expression of wild-type LIPT2, arguing for LIPT2 requirement in intramitochondrial lipoate synthesis.
Catalyzes the transfer of endogenously produced octanoic acid from octanoyl-acyl-carrier-protein onto the lipoyl domains of lipoate-dependent enzymes. Lipoyl-ACP can also act as a substrate although octanoyl-ACP is likely to be the physiological substrate (By similarity).
lipoate-protein ligase B
, lipoyl/octanoyl transferase
, octanoyl-[acyl-carrier-protein]-protein N-octanoyltransferase
, putative lipoyltransferase 2, mitochondrial
, putative octanoyltransferase, mitochondrial