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Literature summary for 7.1.1.2 extracted from

  • DeCorby, A.; Gaskova, D.; Sayles, L.; Lemire, B.
    Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease (2007), Biochim. Biophys. Acta, 1767, 1157-1163.
    View publication on PubMed

Application

Application Comment Organism
medicine gene therapy approaches involving Ndi1p may offer substantial clinical benefits in cases of complex I deficiency Caenorhabditis elegans

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
Caenorhabditis elegans 5739
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
53000
-
SDS-PAGE Caenorhabditis elegans

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
NADH + H+ + ubiquinone Caenorhabditis elegans
-
NAD+ + ubiquinol
-
?
NADH + H+ + ubiquinone Caenorhabditis elegans N2
-
NAD+ + ubiquinol
-
?

Organism

Organism UniProt Comment Textmining
Caenorhabditis elegans
-
-
-
Caenorhabditis elegans N2
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
NADH + H+ + ubiquinone
-
Caenorhabditis elegans NAD+ + ubiquinol
-
?
NADH + H+ + ubiquinone
-
Caenorhabditis elegans N2 NAD+ + ubiquinol
-
?

Synonyms

Synonyms Comment Organism
alternative NADH:ubiquinone oxidoreductase
-
Caenorhabditis elegans
Ndi1p
-
Caenorhabditis elegans

Cofactor

Cofactor Comment Organism Structure
FAD noncovalently bound Caenorhabditis elegans
additional information Ndi1p lacks iron sulfur clusters Caenorhabditis elegans
NADH
-
Caenorhabditis elegans

General Information

General Information Comment Organism
physiological function Ndi1p functionally interacts with the nematode respiratory chain and contributes to the formation of the mitochondrial membrane potential in vivo. Ndi1p expression results in significantly improved reproductive success, better survival under conditions of oxidative stress, fewer signs of premature aging, increased respiration rates and a complete restoration of the in vivo mitochondrial membrane potential in Caenorhabditis elegans nuo-1 mutants. However, Ndi1p is not able to fully replace complex I and cannot support larval development when complex I is missing Caenorhabditis elegans