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Protein

Succinate dehydrogenase assembly factor 3, mitochondrial

Gene

SDHAF3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 3 out of 5-Experimental evidence at protein leveli

Functioni

Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF1.By similarity

GO - Biological processi

Keywordsi

Molecular functionChaperone

Names & Taxonomyi

Protein namesi
Recommended name:
Succinate dehydrogenase assembly factor 3, mitochondrialBy similarityImported
Short name:
SDH assembly factor 3By similarity
Short name:
SDHAF3By similarity
Gene namesi
Name:SDHAF3Imported
Synonyms:ACN9Imported
ORF Names:DC11
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 7

Organism-specific databases

EuPathDBiHostDB:ENSG00000196636.7.
HGNCiHGNC:21752. SDHAF3.

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

DisGeNETi57001.
OpenTargetsiENSG00000196636.
PharmGKBiPA134976380.

Polymorphism and mutation databases

BioMutaiACN9.
DMDMi74734325.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 30MitochondrionSequence analysisAdd BLAST30
ChainiPRO_000004265331 – 125Succinate dehydrogenase assembly factor 3, mitochondrialAdd BLAST95

Proteomic databases

EPDiQ9NRP4.
MaxQBiQ9NRP4.
PaxDbiQ9NRP4.
PeptideAtlasiQ9NRP4.
PRIDEiQ9NRP4.

PTM databases

iPTMnetiQ9NRP4.
PhosphoSitePlusiQ9NRP4.

Expressioni

Gene expression databases

BgeeiENSG00000196636.
CleanExiHS_ACN9.
ExpressionAtlasiQ9NRP4. baseline and differential.
GenevisibleiQ9NRP4. HS.

Organism-specific databases

HPAiHPA020523.

Interactioni

Subunit structurei

Interacts with SDHB within an SDHA-SDHB subcomplex.By similarity

Protein-protein interaction databases

BioGridi121316. 12 interactors.
STRINGi9606.ENSP00000414066.

Structurei

3D structure databases

ProteinModelPortaliQ9NRP4.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I LYR family. SDHAF3 subfamily.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG4100. Eukaryota.
ENOG4111Y90. LUCA.
GeneTreeiENSGT00390000010029.
HOGENOMiHOG000039506.
HOVERGENiHBG080818.
InParanoidiQ9NRP4.
OMAiQLYELMH.
OrthoDBiEOG091G0TG1.
PhylomeDBiQ9NRP4.
TreeFamiTF105635.

Family and domain databases

InterProiView protein in InterPro
IPR008381. ACN9.
PANTHERiPTHR13137. PTHR13137. 1 hit.

Sequencei

Sequence statusi: Complete.

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

Q9NRP4-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MPGRHVSRVR ALYKRVLQLH RVLPPDLKSL GDQYVKDEFR RHKTVGSDEA
60 70 80 90 100
QRFLQEWEVY ATALLQQANE NRQNSTGKAC FGTFLPEEKL NDFRDEQIGQ
110 120
LQELMQEATK PNRQFSISES MKPKF
Length:125
Mass (Da):14,652
Last modified:October 1, 2000 - v1
Checksum:i8C6897D7815966FA
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF201933 mRNA. Translation: AAF86869.1.
AC073421 Genomic DNA. No translation available.
AC073900 Genomic DNA. Translation: AAS07570.1.
CH236949 Genomic DNA. Translation: EAL24120.1.
CH471091 Genomic DNA. Translation: EAW76740.1.
BC020621 mRNA. Translation: AAH20621.1.
BC022858 mRNA. Translation: AAH22858.1.
CCDSiCCDS5648.1.
RefSeqiNP_064571.1. NM_020186.2.
UniGeneiHs.592269.

Genome annotation databases

EnsembliENST00000432641; ENSP00000414066; ENSG00000196636.
GeneIDi57001.
KEGGihsa:57001.
UCSCiuc003uoo.5. human.

Similar proteinsi

Entry informationi

Entry nameiSDHF3_HUMAN
AccessioniPrimary (citable) accession number: Q9NRP4
Secondary accession number(s): A4D1J3, Q75MD6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 25, 2005
Last sequence update: October 1, 2000
Last modified: September 27, 2017
This is version 97 of the entry and version 1 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 7
    Human chromosome 7: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families