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NDUFB11 is an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed not to be involved in catalysis. NDUFB11 functions in the transfer of electrons from NADH to the respiratory chain and is located in the inner mitochondrial membrane. The immediate electron acceptor for NDUFB11 is believed to be ubiquinone. Further, NDUFB11 is believed to have NADH dehydrogenase activity, and oxidoreductase activity. Diseases associated with NDUFB11 protein dysfunction include linear skin defects with multiple congenital anomalies and mitochondrial complex I deficiency.
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Protein Aliases: CI-ESSS; complex I NP17.3 subunit; Complex I-ESSS; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial; NADH-ubiquinone oxidoreductase ESSS subunit; Neuronal protein 17.3; Np17.3
Gene Aliases: CI-ESSS; ESSS; LSDMCA3; NDUFB11; Np15; NP17.3; P17.3; UNQ111/PRO1064
UniProt ID: (Human) Q9NX14
Entrez Gene ID: (Human) 54539
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