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Literature summary for 1.3.8.8 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

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

Cloned(Commentary)

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

Protein Variants

Protein Variants Comment Organism
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]

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
-
Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
Homo sapiens
-
liver
-
Homo sapiens
-
muscle
-
Homo sapiens
-

Storage Stability

Storage Stability Organism
-20°C Homo sapiens

Substrates and Products (Substrate)

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

Synonyms

Synonyms Comment Organism
VLCAD
-
Homo sapiens