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Literature summary extracted from

  • Merritt II, J.L.; Matern, D.; Vockley, J.; Daniels, J.; Ngyen, T.V.; Schowalter, D.B.
    In vitro characterization and in vivo expression of human very-long chain acyl-CoA dehydrogenase (2006), Mol. Genet. Metab., 88, 351-358.
    View publication on PubMed

Application

EC Number Application Comment Organism
1.3.8.8 medicine development of a durable gene therapy for VLCAD deficiency Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.3.8.8 into plasmid pCMV, expression in immune competent mice Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.3.8.8 additional information VLCAD deficient fibroblasts and pCMV-hVLCAD transiently transfected VLCAD deficient fibroblasts, show antibody specificity, transfected fibroblasts show correction of the metabolic block as demonstrated by normalization of C14- and C16-acylcarnitine species in cell culture media and restoration of VLCAD activity in cells Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
1.3.8.8 70000
-
 Western blot analysis Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
1.3.8.8 Homo sapiens
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
1.3.8.8
-
 Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.3.8.8 fibroblast
-
 Homo sapiens
-
1.3.8.8 liver
-
 Homo sapiens
-
1.3.8.8 muscle
-
 Homo sapiens
-

Storage Stability

EC Number Storage Stability Organism
1.3.8.8 -20°C Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.3.8.8 palmitoyl-CoA + electron-transfer flavoprotein
-
 Homo sapiens 2-hexadecenoyl-CoA + reduced electron-transfer flavoprotein
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.3.8.8 VLCAD
-
 Homo sapiens