Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary extracted from

  • Wakitani, S.; Torisu, S.; Yoshino, T.; Hattanda, K.; Yamato, O.; Tasaki, R.; Fujita, H.; Nishino, K.
    Multiple acyl-CoA dehydrogenation deficiency (glutaric aciduria type II) with a novel mutation of electron transfer flavoprotein-dehydrogenase in a cat (2014), JIMD Rep., 13, 43-51 .
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.5.5.1 RT-PCR enzyme expression analysis Felis sp.

Protein Variants

EC Number Protein Variants Comment Organism
1.5.5.1 F231C naturally occuring mutation, the feline patient-specific mutation c.692T>G (p.F231C) in enzyme ETFDH in feline ETFDH is completely conserved in eukaryotes, and is located on the apical surface of enzyme ETFDH, receiving electrons from electron-transferring flavoprotein (ETF) causing multiple acyl-CoA dehydrogenation deficiency (MADD) in the cat, phenotype, overview Felis sp.

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.5.5.1 reduced electron-transferring flavoprotein + ubiquinone Felis sp.
-
 electron-transferring flavoprotein + ubiquinol
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.5.5.1 Felis sp.
-
-
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.5.5.1 reduced electron-transferring flavoprotein + ubiquinone
-
 Felis sp. electron-transferring flavoprotein + ubiquinol
-
 ?

Subunits

EC Number Subunits Comment Organism
1.5.5.1 More enzyme protein structure homology-modeling Felis sp.

Synonyms

EC Number Synonyms Comment Organism
1.5.5.1 ETF dehydrogenase
-
 Felis sp.
1.5.5.1 ETF-ubiquinone oxidoreductase
-
 Felis sp.
1.5.5.1 ETFDH
-
 Felis sp.

Cofactor

EC Number Cofactor Comment Organism Structure
1.5.5.1 ubiquinone
-
 Felis sp.

General Information

EC Number General Information Comment Organism
1.5.5.1 malfunction deficiency of ETF or ETFDH leads to dysfunction of acyl-CoA dehydrogenase, resulting in accumulation of long- and medium-chain fatty acids. Multiple acyl-CoA dehydrogenation deficiency (glutaric aciduria type II, MADD) occurs due to a mutation of electron transfer flavoprotein-dehydrogenase in a cat, that presented with symptoms characteristic of MADD including hypoglycemia, hyperammonemia, vomiting, diagnostic organic aciduria, and accumulation of medium- and long-chain fatty acids in plasma, phenotype, overview Felis sp.
1.5.5.1 additional information enzyme protein structure homology-modeling, overview Felis sp.
1.5.5.1 physiological function ETFDH transfer electrons from ETF to ubiquinone, which exists in the inner mitochondrial membrane and participates in electron transport system to produce ATP. In this process, electrons enter through the flavin center of ETFDH and exit via the 4Fe-4S cluster for delivery to ubiquinone Felis sp.