Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Acyl carrier protein, mitochondrial

Gene

NDUFAB1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Carrier of the growing fatty acid chain in fatty acid biosynthesis (By similarity). Accessory and non-catalytic subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), which functions in the transfer of electrons from NADH to the respiratory chain (PubMed:27626371).By similarity1 Publication

Miscellaneous

In contrast to other accessory subunits of complex I, NDUFAB1 is the only subunit that is essential for cell viability in HEK293T cells. Since knockout cells lack assembled complex I and die in galactose media, this suggests that the essential role of NDUFAB1 is independent of complex I.1 Publication

GO - Molecular functioni

  • acyl binding Source: GO_Central
  • acyl carrier activity Source: GO_Central
  • calcium ion binding Source: UniProtKB
  • fatty acid binding Source: UniProtKB
  • NADH dehydrogenase (ubiquinone) activity Source: UniProtKB
  • phosphopantetheine binding Source: GO_Central

GO - Biological processi

  • cellular nitrogen compound metabolic process Source: Reactome
  • fatty acid beta-oxidation Source: Reactome
  • fatty acid biosynthetic process Source: UniProtKB
  • lipid A biosynthetic process Source: GO_Central
  • mitochondrial electron transport, NADH to ubiquinone Source: Reactome
  • mitochondrial respiratory chain complex I assembly Source: UniProtKB
  • protein lipoylation Source: UniProtKB

Keywordsi

Biological processElectron transport, Fatty acid biosynthesis, Fatty acid metabolism, Lipid biosynthesis, Lipid metabolism, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiR-HSA-389661 Glyoxylate metabolism and glycine degradation
R-HSA-611105 Respiratory electron transport
R-HSA-6799198 Complex I biogenesis
R-HSA-77289 Mitochondrial Fatty Acid Beta-Oxidation

Names & Taxonomyi

Protein namesi
Recommended name:
Acyl carrier protein, mitochondrial
Short name:
ACP
Alternative name(s):
CI-SDAP
NADH-ubiquinone oxidoreductase 9.6 kDa subunit
Gene namesi
Name:NDUFAB1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

EuPathDBiHostDB:ENSG00000004779.9
HGNCiHGNC:7694 NDUFAB1
MIMi603836 gene
neXtProtiNX_O14561

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi4706
MalaCardsiNDUFAB1
OpenTargetsiENSG00000004779
PharmGKBiPA31500

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiNDUFAB1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 68MitochondrionCombined sources1 PublicationAdd BLAST68
ChainiPRO_000000056169 – 156Acyl carrier protein, mitochondrialAdd BLAST88

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei88N6-acetyllysineBy similarity1
Modified residuei112O-(pantetheine 4'-phosphoryl)serinePROSITE-ProRule annotation1

Keywords - PTMi

Acetylation, Phosphopantetheine, Phosphoprotein

Proteomic databases

EPDiO14561
MaxQBiO14561
PaxDbiO14561
PeptideAtlasiO14561
PRIDEiO14561
TopDownProteomicsiO14561

PTM databases

iPTMnetiO14561
PhosphoSitePlusiO14561
SwissPalmiO14561

Expressioni

Gene expression databases

BgeeiENSG00000004779
CleanExiHS_NDUFAB1
ExpressionAtlasiO14561 baseline and differential
GenevisibleiO14561 HS

Organism-specific databases

HPAiHPA054364

Interactioni

Subunit structurei

Mammalian complex I is composed of 45 different subunits (PubMed:12611891). Interacts with ETFRF1 (PubMed:27499296).2 Publications

Protein-protein interaction databases

BioGridi110786, 29 interactors
CORUMiO14561
IntActiO14561, 25 interactors
MINTiO14561
STRINGi9606.ENSP00000007516

Structurei

Secondary structure

1156
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi76 – 89Combined sources14
Beta strandi91 – 93Combined sources3
Turni95 – 97Combined sources3
Beta strandi99 – 101Combined sources3
Beta strandi104 – 108Combined sources5
Helixi112 – 123Combined sources12
Turni132 – 136Combined sources5
Helixi141 – 151Combined sources11

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2DNWNMR-A71-156[»]
5OOLelectron microscopy3.06w1-156[»]
5OOMelectron microscopy3.03w1-156[»]
5XTBelectron microscopy3.40G72-156[»]
5XTCelectron microscopy3.70X72-156[»]
5XTDelectron microscopy3.70G/X72-156[»]
5XTHelectron microscopy3.90G/X72-156[»]
5XTIelectron microscopy17.40BG/BX/G/X72-156[»]
ProteinModelPortaliO14561
SMRiO14561
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiO14561

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini77 – 152CarrierPROSITE-ProRule annotationAdd BLAST76

Sequence similaritiesi

Belongs to the acyl carrier protein (ACP) family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1748 Eukaryota
COG0236 LUCA
GeneTreeiENSGT00390000002127
HOGENOMiHOG000178184
HOVERGENiHBG024318
InParanoidiO14561
KOiK03955
OMAiVTHLCRQ
OrthoDBiEOG091G0UIC
PhylomeDBiO14561
TreeFamiTF314361

Family and domain databases

Gene3Di1.10.1200.10, 1 hit
HAMAPiMF_01217 Acyl_carrier, 1 hit
InterProiView protein in InterPro
IPR036736 ACP-like_sf
IPR003231 Acyl_carrier
IPR009081 PP-bd_ACP
IPR006162 Ppantetheine_attach_site
PfamiView protein in Pfam
PF00550 PP-binding, 1 hit
ProDomiView protein in ProDom or Entries sharing at least one domain
PD000887 PD000887, 1 hit
SUPFAMiSSF47336 SSF47336, 1 hit
TIGRFAMsiTIGR00517 acyl_carrier, 1 hit
PROSITEiView protein in PROSITE
PS50075 CARRIER, 1 hit
PS00012 PHOSPHOPANTETHEINE, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O14561-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MASRVLSAYV SRLPAAFAPL PRVRMLAVAR PLSTALCSAG TQTRLGTLQP
60 70 80 90 100
ALVLAQVPGR VTQLCRQYSD MPPLTLEGIQ DRVLYVLKLY DKIDPEKLSV
110 120 130 140 150
NSHFMKDLGL DSLDQVEIIM AMEDEFGFEI PDIDAEKLMC PQEIVDYIAD

KKDVYE
Length:156
Mass (Da):17,417
Last modified:July 11, 2002 - v3
Checksum:i0FCE3C3EC6D97AE2
GO

Sequence cautioni

The sequence AAC05814 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti132D → Y in AAH58920 (PubMed:15489334).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF087660 mRNA Translation: AAD23566.1
AK311877 mRNA Translation: BAG34818.1
AC002400 Genomic DNA Translation: AAC05814.1 Different initiation.
CH471145 Genomic DNA Translation: EAW55816.1
BC058920 mRNA Translation: AAH58920.1
CCDSiCCDS10614.1
PIRiT00741
RefSeqiNP_004994.1, NM_005003.2
UniGeneiHs.189716

Genome annotation databases

EnsembliENST00000007516; ENSP00000007516; ENSG00000004779
ENST00000570319; ENSP00000458770; ENSG00000004779
GeneIDi4706
KEGGihsa:4706
UCSCiuc002dlw.4 human

Similar proteinsi

Entry informationi

Entry nameiACPM_HUMAN
AccessioniPrimary (citable) accession number: O14561
Secondary accession number(s): B2R4M1, Q9UNV1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: July 11, 2002
Last modified: March 28, 2018
This is version 173 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health