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Literature summary for 1.3.8.8 extracted from

  • Beattie, S.G.; Goetzman, E.; Tang, Q.; Conlon, T.; Campbell-Thompson, M.; Matern, D.; Vockley, J.; Flotte, T.R.
    Recombinant adeno-associated virus-mediated gene delivery of long chain acyl coenzyme A dehydrogenase (LCAD) into LCAD-deficient mice (2008), J. Gene Med., 10, 1113-1123.
    View publication on PubMedView publication on EuropePMC

Application

Application Comment Organism
medicine LCAD gene replacement to the tibialis anterior muscle and livers of partially deficient LCAD mice is described, a surrogate model for human VLCAD deficiency Mus musculus

Cloned(Commentary)

Cloned (Comment) Organism
a vector plasmid pCB-mLCAD for transfection of LCAD-deficient mice is used Mus musculus

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
Mus musculus 5739
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
44000
-
determined by SDS-PAGE and Western Blot analysis Mus musculus

Organism

Organism UniProt Comment Textmining
Mus musculus
-
mice heterozygous for a mutation in LCAD, strain B6.129S6-Acadltm1Uab Hsd
-

Source Tissue

Source Tissue Comment Organism Textmining
hepatocyte
-
Mus musculus
-
tibialis anterior muscle Mus musculus
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
0.06037
-
LCAD enzyme activity in livers of partially deficient LCAD mice 8 weeks after injection of recombinant adeno-associated viral mLCAD vector via the hepatic portal vein Mus musculus
0.2546
-
LCAD enzyme activity in tibialis anterior muscle of LCAD-heterozygous deficient mice 10 weeks post-injection 1 x 10exp11 vg recombinant adeno-associated viral mLCAD Mus musculus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2-methylheptanoyl-CoA + acceptor activity assay Mus musculus ?
-
?
palmitoyl-CoA + acceptor activity assay Mus musculus ?
-
?

Synonyms

Synonyms Comment Organism
LCAD
-
Mus musculus
long chain acyl coenzyme A dehydrogenase
-
Mus musculus

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
32
-
activity assay Mus musculus

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
8
-
activity assay Mus musculus