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NDUFAF1 encodes a complex I assembly factor protein. De plus, nous expédions NADH Dehydrogenase (Ubiquinone) 1 alpha Subcomplex, Assembly Factor 1 Anticorps (41) et et beaucoup plus de produits pour cette protéine.
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a case of leukodystrophy associated with mitochondrial complex I deficiency due to a novel mutation in the NDUFAF1 gene.
oncogenic K-Ras is able to induce significant alterations in mitochondrial protein expression, and identified NDUFAF1 as an important molecule whose low expression contributes to mitochondrial dysfunction induced by K-Ras.
NDUFAF1 is an important protein for the assembly/stability of complex I (NADH:ubiquinone oxidoreductase (Montrer TXNRD1 Protéines)).
Study shows a mitochondrial function for Ecsit (Montrer ECSIT Protéines) in the assembly of mitochondrial complex I, an N-terminal targeting signal directs Ecsit (Montrer ECSIT Protéines) to mitochondria, where it interacts with assembly chaperone NDUFAF1.
the involvement of NDUFAF1 in the assembly process could be indirect rather than direct via the binding to assembly intermediates
CIA30 is a crucial component in the early assembly of complex I and mutations in its gene can cause mitochondrial disease
This gene encodes a complex I assembly factor protein. Complex I (NADH-ubiquinone oxidoreductase) catalyzes the transfer of electrons from NADH to ubiquinone (coenzyme Q) in the first step of the mitochondrial respiratory chain, resulting in the translocation of protons across the inner mitochondrial membrane. The encoded protein is required for assembly of complex I, and mutations in this gene are a cause of mitochondrial complex I deficiency. Alternatively spliced transcript variants have been observed for this gene, and a pseudogene of this gene is located on the long arm of chromosome 19.
NADH dehydrogenase (ubiquinone) 1 assembly factor 1
, complex I intermediate-associated protein 30, mitochondrial
, NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 1
, NADH-ubiquinone oxidoreductase 1 alpha subcomplex, assembly factor 1
, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1