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Protein

Succinate dehydrogenase cytochrome b560 subunit, mitochondrial

Gene

SDHC

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

Functioni

Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).

Cofactori

hemeBy similarityNote: The heme is bound between the two transmembrane subunits SDHC and SDHD.By similarity

Pathwayi: tricarboxylic acid cycle

This protein is involved in the pathway tricarboxylic acid cycle, which is part of Carbohydrate metabolism.
View all proteins of this organism that are known to be involved in the pathway tricarboxylic acid cycle and in Carbohydrate metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi127Iron (heme axial ligand); shared with SDHDBy similarity1

GO - Molecular functioni

GO - Biological processi

  • aerobic respiration Source: UniProtKB
  • mitochondrial electron transport, succinate to ubiquinone Source: GO_Central
  • oxidation-reduction process Source: UniProtKB
  • tricarboxylic acid cycle Source: UniProtKB

Keywordsi

Biological processElectron transport, Transport, Tricarboxylic acid cycle
LigandHeme, Iron, Metal-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS07014-MONOMER
BRENDAi1.3.5.1 2681
ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-71403 Citric acid cycle (TCA cycle)
SIGNORiQ99643
UniPathwayiUPA00223

Names & Taxonomyi

Protein namesi
Recommended name:
Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
Alternative name(s):
Integral membrane protein CII-3
QPs-1
Short name:
QPs1
Succinate dehydrogenase complex subunit C
Succinate-ubiquinone oxidoreductase cytochrome B large subunit
Short name:
CYBL
Gene namesi
Name:SDHC
Synonyms:CYB560, SDH3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

EuPathDBiHostDB:ENSG00000143252.14
HGNCiHGNC:10682 SDHC
MIMi602413 gene
neXtProtiNX_Q99643

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

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini30 – 62Mitochondrial matrixBy similarityAdd BLAST33
Transmembranei63 – 92HelicalBy similarityAdd BLAST30
Topological domaini93 – 112Mitochondrial intermembraneBy similarityAdd BLAST20
Transmembranei113 – 137HelicalBy similarityAdd BLAST25
Topological domaini138 – 144Mitochondrial matrixBy similarity7
Transmembranei145 – 166HelicalBy similarityAdd BLAST22
Topological domaini167 – 169Mitochondrial intermembraneBy similarity3

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Paragangliomas 3 (PGL3)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas can develop at various body sites, including the head, neck, thorax and abdomen. Most commonly, they are located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear.
See also OMIM:605373
Paraganglioma and gastric stromal sarcoma (PGGSS)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionGastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance.
See also OMIM:606864

Organism-specific databases

DisGeNETi6391
GeneReviewsiSDHC
MalaCardsiSDHC
MIMi605373 phenotype
606864 phenotype
OpenTargetsiENSG00000143252
Orphaneti97286 Carney-Stratakis syndrome
201 Cowden syndrome
44890 Gastrointestinal stromal tumor
29072 Hereditary pheochromocytoma-paraganglioma
PharmGKBiPA35607

Chemistry databases

DrugBankiDB00139 Succinic acid
DB08689 UBIQUINONE-1

Polymorphism and mutation databases

BioMutaiSDHC
DMDMi5915811

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 29MitochondrionBy similarityAdd BLAST29
ChainiPRO_000000363430 – 169Succinate dehydrogenase cytochrome b560 subunit, mitochondrialAdd BLAST140

Proteomic databases

EPDiQ99643
MaxQBiQ99643
PaxDbiQ99643
PeptideAtlasiQ99643
PRIDEiQ99643
ProteomicsDBi78373
78374 [Q99643-2]
78375 [Q99643-3]
78376 [Q99643-4]
78377 [Q99643-5]
TopDownProteomicsiQ99643-1 [Q99643-1]

PTM databases

iPTMnetiQ99643
PhosphoSitePlusiQ99643
SwissPalmiQ99643

Expressioni

Gene expression databases

BgeeiENSG00000143252
CleanExiHS_SDHC
ExpressionAtlasiQ99643 baseline and differential
GenevisibleiQ99643 HS

Interactioni

Subunit structurei

Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD.

Protein-protein interaction databases

BioGridi112292, 14 interactors
ComplexPortaliCPX-561 Mitochondrial respiratory chain complex II
IntActiQ99643, 9 interactors
MINTiQ99643
STRINGi9606.ENSP00000356953

Structurei

3D structure databases

ProteinModelPortaliQ99643
SMRiQ99643
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the cytochrome b560 family.Curated

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0449 Eukaryota
COG2009 LUCA
GeneTreeiENSGT00390000000566
HOGENOMiHOG000160253
HOVERGENiHBG003187
InParanoidiQ99643
KOiK00236
OMAiIPGGIPC
OrthoDBiEOG091G0SR6
PhylomeDBiQ99643
TreeFamiTF313317

Family and domain databases

Gene3Di1.20.1300.10, 1 hit
InterProiView protein in InterPro
IPR034804 SQR/QFR_C/D
IPR018495 Succ_DH_cyt_bsu_CS
IPR014314 Succ_DH_cytb556
IPR000701 SuccDH_FuR_B_TM-su
PfamiView protein in Pfam
PF01127 Sdh_cyt, 1 hit
PIRSFiPIRSF000178 SDH_cyt_b560, 1 hit
SUPFAMiSSF81343 SSF81343, 1 hit
TIGRFAMsiTIGR02970 succ_dehyd_cytB, 1 hit
PROSITEiView protein in PROSITE
PS01000 SDH_CYT_1, 1 hit
PS01001 SDH_CYT_2, 1 hit

Sequences (5)i

Sequence statusi: Complete.

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

This entry describes 5 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q99643-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAALLLRHVG RHCLRAHFSP QLCIRNAVPL GTTAKEEMER FWNKNIGSNR
60 70 80 90 100
PLSPHITIYS WSLPMAMSIC HRGTGIALSA GVSLFGMSAL LLPGNFESYL
110 120 130 140 150
ELVKSLCLGP ALIHTAKFAL VFPLMYHTWN GIRHLMWDLG KGLKIPQLYQ
160
SGVVVLVLTV LSSMGLAAM
Length:169
Mass (Da):18,610
Last modified:May 1, 1997 - v1
Checksum:iDF7CBD6D0CD49500
GO
Isoform 2 (identifier: Q99643-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     81-169: GVSLFGMSAL...VLSSMGLAAM → DVGPRKRPED...PAWEKFSLFV

Show »
Length:150
Mass (Da):16,650
Checksum:i971579A96F795D90
GO
Isoform 3 (identifier: Q99643-3) [UniParc]FASTAAdd to basket
Also known as: CII-3b

The sequence of this isoform differs from the canonical sequence as follows:
     27-60: Missing.

Show »
Length:135
Mass (Da):14,770
Checksum:i5F7A8BC812112D75
GO
Isoform 4 (identifier: Q99643-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     27-60: Missing.
     81-169: GVSLFGMSAL...VLSSMGLAAM → DVGPRKRPED...PAWEKFSLFV

Show »
Length:116
Mass (Da):12,810
Checksum:i36DFFD8CC2E6D4E8
GO
Isoform 5 (identifier: Q99643-5) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     7-59: Missing.

Show »
Length:116
Mass (Da):12,474
Checksum:iAC702AE33DD1F118
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0413817 – 59Missing in isoform 5. 1 PublicationAdd BLAST53
Alternative sequenceiVSP_04138227 – 60Missing in isoform 3 and isoform 4. 2 PublicationsAdd BLAST34
Alternative sequenceiVSP_04138381 – 169GVSLF…GLAAM → DVGPRKRPEDSPAIPVWSGC PGSYCVVLYGAGSHVKKGGS QHHLPTHYYIHPSFCLSFLS PAWEKFSLFV in isoform 2 and isoform 4. 2 PublicationsAdd BLAST89

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U57877 mRNA Translation: AAB41838.1
D49737 mRNA Translation: BAA31998.1
AF039594
, AF039589, AF039590, AF039591, AF039592, AF039593 Genomic DNA Translation: AAC27993.1
AF081495 Genomic DNA Translation: AAC31940.1
AB201252 mRNA Translation: BAE46977.1
AB211234 mRNA Translation: BAE46978.1
AB211235 mRNA Translation: BAE46979.1
AB212048 mRNA Translation: BAE46980.1
AK294305 mRNA Translation: BAG57586.1
AL592295 Genomic DNA No translation available.
CH471121 Genomic DNA Translation: EAW52600.1
CH471121 Genomic DNA Translation: EAW52604.1
BC020808 mRNA Translation: AAH20808.1
BC033626 mRNA Translation: AAH33626.1
BC066329 mRNA Translation: AAH66329.1
CCDSiCCDS1230.1 [Q99643-1]
CCDS41431.1 [Q99643-2]
CCDS41432.1 [Q99643-5]
CCDS44263.1 [Q99643-3]
CCDS60330.1 [Q99643-4]
RefSeqiNP_001030588.1, NM_001035511.1 [Q99643-2]
NP_001030589.1, NM_001035512.1 [Q99643-3]
NP_001030590.1, NM_001035513.1 [Q99643-5]
NP_001265101.1, NM_001278172.1 [Q99643-4]
NP_002992.1, NM_003001.3 [Q99643-1]
UniGeneiHs.444472

Genome annotation databases

EnsembliENST00000342751; ENSP00000356952; ENSG00000143252 [Q99643-2]
ENST00000367975; ENSP00000356953; ENSG00000143252 [Q99643-1]
ENST00000392169; ENSP00000376009; ENSG00000143252 [Q99643-5]
ENST00000432287; ENSP00000390558; ENSG00000143252 [Q99643-3]
ENST00000513009; ENSP00000423260; ENSG00000143252 [Q99643-4]
GeneIDi6391
KEGGihsa:6391
UCSCiuc001gag.4 human [Q99643-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiC560_HUMAN
AccessioniPrimary (citable) accession number: Q99643
Secondary accession number(s): O75609
, Q3C259, Q3C2D8, Q3C2H4, Q5VTH3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: May 1, 1997
Last modified: June 20, 2018
This is version 185 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 1
    Human chromosome 1: 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. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. SIMILARITY comments
    Index of protein domains and families

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