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Literature summary for 5.4.99.2 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)

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

Protein Variants

Protein Variants Comment Organism
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

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Homo sapiens P22033
-
-

Source Tissue

Source Tissue Comment Organism Textmining
liver
-
Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms

Synonyms Comment Organism
MuT
-
Homo sapiens