NDUFB11

Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

ESSS subunit of NADH:ubiquinone oxidoreductase (complex I)
ESSS subunit of NADH:ubiquinone oxidoreductase (complex I)
Identifiers
Symbol	ESSS
Pfam	PF10183
InterPro	IPR019329
Pfam
Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

NDUFB11
Identifiers
Aliases	NDUFB11, CI-ESSS, ESSS, NP17.3, Np15, P17.3, LSDMCA3, NADH:ubiquinone oxidoreductase subunit B11, MC1DN30
External IDs	OMIM: 300403; MGI: 1349919; HomoloGene: 10398; GeneCards: NDUFB11; OMA:NDUFB11 - orthologs
Gene location (Human)
Chr.	X chromosome (human)
End	47,145,466 bp
Gene location (Mouse)
Chr.	X chromosome (mouse)
End	20,483,858 bp
RNA expression pattern
	Top expressed in
	apex of heart; ; muscle of thigh; ; gastrocnemius muscle; ; mucosa of transverse colon; ; left ventricle; ; right adrenal gland; ; right auricle of heart; ; right adrenal cortex; ; ganglionic eminence; ; left adrenal gland;
	Top expressed in
	temporal muscle; ; digastric muscle; ; sternocleidomastoid muscle; ; interventricular septum; ; triceps brachii muscle; ; right ventricle; ; plantaris muscle; ; extensor digitorum longus muscle; ; extraocular muscle; ; cardiac muscle tissue of left ventricle;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	protein binding;
Cellular component	membrane; respirasome; mitochondrial respiratory chain complex I; integral component of membrane; mitochondrial inner membrane; mitochondrion;
Biological process	mitochondrial respiratory chain complex I assembly; mitochondrial electron transport, NADH to ubiquinone;
	Sources:Amigo / QuickGO
Orthologs
	54539
	104130
	ENSG00000147123
	ENSMUSG00000031059
	Q9NX14
	O09111
	NM_019056; NM_001135998
	NM_019435; NM_001358624
	NP_001129470; NP_061929
	NP_062308; NP_001345553
	Wikidata
View/Edit Human	View/Edit Mouse

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial (NADH-ubiquinone oxidoreductase ESSS subunit) is an enzyme that in humans is encoded by the NDUFB11 gene. NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11 is an accessory subunit of the NADH dehydrogenase (ubiquinone) complex, located in the mitochondrial inner membrane. It is also known as Complex I and is the largest of the five complexes of the electron transport chain. NDUFB11 mutations have been associated with linear skin defects with multiple congenital anomalies 3 and mitochondrial complex I deficiency.