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Literature summary extracted from

  • Chandler, R.J.; Venditti, C.P.
    Pre-clinical efficacy and dosing of an AAV8 vector expressing human methylmalonyl-CoA mutase in a murine model of methylmalonic acidemia (MMA) (2012), Mol. Genet. Metab., 107, 617-619.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

EC Number Cloned (Comment) Organism
5.4.99.2 functional transgenic enzyme expression in enzyme-deficient Mus musculus using a AAV8-CBA-MUT vector Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
5.4.99.2 additional information neither a species barrier to mitochondrial processing nor an apparent immune response to MUT limits the murine model as an experimental platform to test the efficacy of human gene therapy vectors for methylmalonic acidemia Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
5.4.99.2 mitochondrion
-
 Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
5.4.99.2 (R)-methylmalonyl-CoA Homo sapiens
-
 succinyl-CoA
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
5.4.99.2 Homo sapiens P22033
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
5.4.99.2 liver
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
5.4.99.2 (R)-methylmalonyl-CoA
-
 Homo sapiens succinyl-CoA
-
 ?

Synonyms

EC Number Synonyms Comment Organism
5.4.99.2 MuT
-
 Homo sapiens