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NDUFB11 NADH:ubiquinone oxidoreductase subunit B11 [ Homo sapiens (human) ]

Gene ID: 54539, updated on 9-Jun-2025
Official Symbol
NDUFB11provided by HGNC
Official Full Name
NADH:ubiquinone oxidoreductase subunit B11provided by HGNC
Primary source
HGNC:HGNC:20372
See related
Ensembl:ENSG00000147123 MIM:300403; AllianceGenome:HGNC:20372
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
ESSS; Np15; P17.3; NP17.3; CI-ESSS; MC1DN30
Summary
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. [provided by RefSeq, Dec 2016]
Expression
Ubiquitous expression in adrenal (RPKM 29.1), heart (RPKM 25.4) and 25 other tissues See more
Orthologs
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See NDUFB11 in Genome Data Viewer
Location:
Xp11.3
Exon count:
3
Annotation release Status Assembly Chr Location
RS_2024_08 current GRCh38.p14 (GCF_000001405.40) X NC_000023.11 (47142216..47145491, complement)
RS_2024_08 current T2T-CHM13v2.0 (GCF_009914755.1) X NC_060947.1 (46551936..46555216, complement)
RS_2024_09 previous assembly GRCh37.p13 (GCF_000001405.25) X NC_000023.10 (47001615..47004890, complement)

Chromosome X - NC_000023.11Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC105373193 Neighboring gene uncharacterized LOC107985720 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 29580 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 29581 Neighboring gene RNA, U12 small nuclear 2, pseudogene Neighboring gene ATAC-STARR-seq lymphoblastoid active region 29582 Neighboring gene RNA binding motif protein 10 Neighboring gene ITPK1 pseudogene 1 Neighboring gene CDK7 strongly-dependent group 2 enhancer GRCh37_chrX:47037923-47039122 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 29583 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chrX:47050393-47050933 Neighboring gene ReSE screen-validated silencer GRCh37_chrX:47053068-47053256 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 20798 Neighboring gene CDK7 strongly-dependent group 2 enhancer GRCh37_chrX:47057593-47058792 Neighboring gene ubiquitin like modifier activating enzyme 1 Neighboring gene uncharacterized LOC105373194 Neighboring gene inactivation escape 1

  • Project title: Tissue-specific circular RNA induction during human fetal development
  • Description: 35 human fetal samples from 6 tissues (3 - 7 replicates per tissue) collected between 10 and 20 weeks gestational time were sequenced using Illumina TruSeq Stranded Total RNA
  • BioProject: PRJNA270632
  • Publication: PMID 26076956
  • Analysis date: Mon Apr 2 22:54:59 2018

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
Products Interactant Other Gene Complex Source Pubs Description

Markers

Clone Names

  • FLJ20494, MGC111182

Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables protein binding IPI
Inferred from Physical Interaction
more info
PubMed 
Process Evidence Code Pubs
involved_in aerobic respiration NAS
Non-traceable Author Statement
more info
PubMed 
involved_in proton motive force-driven mitochondrial ATP synthesis NAS
Non-traceable Author Statement
more info
PubMed 
Component Evidence Code Pubs
located_in membrane IEA
Inferred from Electronic Annotation
more info
 
located_in mitochondrial inner membrane IDA
Inferred from Direct Assay
more info
PubMed 
located_in mitochondrial inner membrane IEA
Inferred from Electronic Annotation
more info
 
located_in mitochondrial inner membrane TAS
Traceable Author Statement
more info
 
located_in mitochondrion HTP PubMed 
located_in mitochondrion IDA
Inferred from Direct Assay
more info
PubMed 
located_in mitochondrion IEA
Inferred from Electronic Annotation
more info
 
part_of respiratory chain complex I IBA
Inferred from Biological aspect of Ancestor
more info
 
part_of respiratory chain complex I IDA
Inferred from Direct Assay
more info
PubMed 
Preferred Names
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial
Names
NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa
NADH-ubiquinone oxidoreductase ESSS subunit
complex I NP17.3 subunit
complex I-ESSS
neuronal protein 17.3

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_052579.1 RefSeqGene

    Range
    5509..7995
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001135998.3NP_001129470.1  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform 2

    See identical proteins and their annotated locations for NP_001129470.1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (2) uses an alternate in_frame splice site in the 3' coding region, compared to variant 1. The encoded isoform (2) has a shorter C-terminus compared to isoform 1.
    Source sequence(s)
    AY359056
    Consensus CDS
    CCDS48100.1
    UniProtKB/Swiss-Prot
    Q5JRR3, Q5JRR4, Q6IAB6, Q8WZ96, Q9BXX9, Q9NX14
    UniProtKB/TrEMBL
    Q7Z4X2
    Related
    ENSP00000367042.3, ENST00000377811.4
    Conserved Domains (1) summary
    pfam10183
    Location:22141
    ESSS; ESSS subunit of NADH:ubiquinone oxidoreductase (complex I)
  2. NM_019056.7NP_061929.2  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform 1

    See identical proteins and their annotated locations for NP_061929.2

    Status: REVIEWED

    Description
    Transcript Variant: This variant (1) represents the longer transcript and encodes the longer isoform (1).
    Source sequence(s)
    AF044213, AF251063, AL513366, AY359056
    Consensus CDS
    CCDS14273.1
    UniProtKB/TrEMBL
    Q7Z4X2
    Related
    ENSP00000509334.1, ENST00000687244.1
    Conserved Domains (1) summary
    pfam10183
    Location:30143
    ESSS; ESSS subunit of NADH:ubiquinone oxidoreductase (complex I)

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2024_08

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p14 Primary Assembly

Genomic

  1. NC_000023.11 Reference GRCh38.p14 Primary Assembly

    Range
    47142216..47145491 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060947.1 Alternate T2T-CHM13v2.0

    Range
    46551936..46555216 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)