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Literature summary for 1.6.1.2 extracted from

  • Ronchi, J.A.; Figueira, T.R.; Ravagnani, F.G.; Oliveira, H.C.; Vercesi, A.E.; Castilho, R.F.
    A spontaneous mutation in the nicotinamide nucleotide transhydrogenase gene of C57BL/6J mice results in mitochondrial redox abnormalities (2013), Free Radic. Biol. Med., 63, 446-456.
    View publication on PubMed

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrial inner membrane
-
Mus musculus 5743
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
NADPH + NAD+ Mus musculus
-
NADP+ + NADH
-
r
NADPH + NAD+ Mus musculus C57/BL6J
-
NADP+ + NADH
-
r

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-
Mus musculus C57/BL6J
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
heart
-
Mus musculus
-
liver
-
Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
NADPH + NAD+
-
Mus musculus NADP+ + NADH
-
r
NADPH + NAD+
-
Mus musculus C57/BL6J NADP+ + NADH
-
r

Synonyms

Synonyms Comment Organism
NNT
-
Mus musculus

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
assay at Mus musculus

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
6.5
-
assay at Mus musculus

General Information

General Information Comment Organism
malfunction spontaneous C57BL/6J(B6J-NntMUT) mutant mice show major redox alterations in respiring mitchondria, including an absence of transhydrogenation between NAD+ and NADP+, higher rates of H2O2 release, the spontaneous oxidation of NADPH,the poor ability to metabolize organic peroxide, and a higher susceptibility to undergo Ca2+-induced mitochondrial permeability transition. Liver mitochondria from B6J-NntMUT mice do not possess NNT activity. The mitochondria of mutant B6J-NntMUT mice exhibit increased oxidized/reduced glutathione ratios as compared to wild-type B6JUnib-NntW mice, phenotypes, overview Mus musculus
physiological function the enzyme functions as a high-capacity source of mitochondrial NADPH and that its functional loss due to the Nnt mutation results in mitochondrial redox abnormalities, most notably a poor ability to sustain NADP+ and glutathione in the reduced states. The enzyme is assembled across the inner mitochondrial membrane and translocates H+ to the mitochondrial matrix as NADP+ is reduced at the expense of NADH on the matrix side. The electrochemical gradient across the inner mitochondrial membrane shifts the NNT equilibrium to the right Mus musculus