Recombinant Human NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (NDUFB11), Truncated

Uniprot Id

Q9NX14

Target Species

Human

Target Name

NDUFB11

Target Full Name

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial

Target Function

Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.

Target Involvement

Linear skin defects with multiple congenital anomalies 3 (LSDMCA3); Mitochondrial complex I deficiency (MT-C1D)

Target Subcellular Location

Mitochondrion inner membrane; Single-pass membrane protein.

Target Protein Families

Complex I NDUFB11 subunit family

Target Tissue Specificity

Ubiquitous.

Target Synonyms

NDUFB11; UNQ111/PRO1064; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial; Complex I-ESSS; CI-ESSS; NADH-ubiquinone oxidoreductase ESSS subunit; Neuronal protein 17.3; Np17.3; p17.3

Target Background

The protein encoded by this gene is a subunit of the multisubunit NADH:ubiquinone oxidoreductase (complex I). Mammalian complex I is located at the mitochondrial inner membrane. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to ubiquinone. Mutations in the human gene are associated with linear skin defects with multiple congenital anomalies 3 and mitochondrial complex I deficiency.