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Literature summary for 1.3.8.6 extracted from

  • Gao, J.; Zhang, C.; Fu, X.; Yi, Q.; Tian, F.; Ning, Q.; Luo, X.
    Effects of targeted suppression of glutaryl-CoA dehydrogenase by lentivirus-mediated shRNA and excessive intake of lysine on apoptosis in rat striatal neurons (2013), PLoS ONE, 8, e63084.
    View publication on PubMedView publication on EuropePMC

Application

Application Comment Organism
pharmacology targeted suppression of GCDH by lentivirus-mediated shRNA and excessive intake of lysine may be a useful cell model of glutaric aciduria type 1, overview Rattus norvegicus

Protein Variants

Protein Variants Comment Organism
additional information constructionof a lentiviral vector containing short hairpin RNA targeted against the GCDH gene expression (lentivirus-shRNA) in neurons Rattus norvegicus

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
glutaryl-CoA + FAD Rattus norvegicus
-
(E)-but-2-enoyl-CoA + CO2 + FADH2
-
?
glutaryl-CoA + FAD Rattus norvegicus Sprague-Dawley
-
(E)-but-2-enoyl-CoA + CO2 + FADH2
-
?

Organism

Organism UniProt Comment Textmining
Rattus norvegicus
-
-
-
Rattus norvegicus Sprague-Dawley
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
neuron striatal, from neonatal rats Rattus norvegicus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
glutaryl-CoA + FAD
-
Rattus norvegicus (E)-but-2-enoyl-CoA + CO2 + FADH2
-
?
glutaryl-CoA + FAD
-
Rattus norvegicus Sprague-Dawley (E)-but-2-enoyl-CoA + CO2 + FADH2
-
?

Synonyms

Synonyms Comment Organism
GCDH
-
Rattus norvegicus

Cofactor

Cofactor Comment Organism Structure
FAD
-
Rattus norvegicus

General Information

General Information Comment Organism
malfunction in glutaric aciduria type 1, glutaryl-CoA dehydrogenase deficiency is responsible for the accumulation of glutaric acid and striatal degeneration, GA1-induced striatal degeneration is partially caspase-dependent, mechanism, overview Rattus norvegicus