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Q99643 (C560_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 149. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (4) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Web links·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended 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 names
Name:SDHC
Synonyms:CYB560, SDH3
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length169 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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).

Cofactor

Heme group. The heme is bound between the two transmembrane subunits SDHC and SDHD By similarity.

Pathway

Carbohydrate metabolism; tricarboxylic acid cycle.

Subunit structure

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.

Subcellular location

Mitochondrion inner membrane; Multi-pass membrane protein.

Involvement in disease

Paragangliomas 3 (PGL3) [MIM:605373]: A 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.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.10

Paraganglioma and gastric stromal sarcoma (PGGSS) [MIM:606864]: Gastrointestinal 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.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.11

Sequence similarities

Belongs to the cytochrome b560 family.

Ontologies

Keywords
   Biological processElectron transport
Transport
Tricarboxylic acid cycle
   Cellular componentMembrane
Mitochondrion
Mitochondrion inner membrane
   Coding sequence diversityAlternative splicing
   DomainTransit peptide
Transmembrane
Transmembrane helix
   LigandHeme
Iron
Metal-binding
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processaerobic respiration

Traceable author statement Ref.2. Source: UniProtKB

cellular metabolic process

Traceable author statement. Source: Reactome

oxidation-reduction process

Traceable author statement Ref.2. Source: UniProtKB

respiratory electron transport chain

Traceable author statement. Source: Reactome

small molecule metabolic process

Traceable author statement. Source: Reactome

tricarboxylic acid cycle

Traceable author statement Ref.2. Source: UniProtKB

   Cellular_componentintegral component of membrane

Traceable author statement Ref.2. Source: UniProtKB

mitochondrial inner membrane

Inferred from sequence or structural similarity. Source: UniProtKB

mitochondrial respiratory chain complex II

Inferred from sequence or structural similarity. Source: UniProtKB

mitochondrion

Traceable author statement PubMed 2302193. Source: UniProtKB

respiratory chain complex II

Traceable author statement Ref.2. Source: UniProtKB

   Molecular_functionelectron carrier activity

Inferred from electronic annotation. Source: InterPro

heme binding

Inferred from sequence or structural similarity. Source: UniProtKB

metal ion binding

Inferred from electronic annotation. Source: UniProtKB-KW

succinate dehydrogenase activity

Inferred from electronic annotation. Source: InterPro

Complete GO annotation...

Alternative products

This entry describes 5 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q99643-1)

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.
Isoform 2 (identifier: Q99643-2)

The sequence of this isoform differs from the canonical sequence as follows:
     81-169: GVSLFGMSAL...VLSSMGLAAM → DVGPRKRPED...PAWEKFSLFV
Isoform 3 (identifier: Q99643-3)

Also known as: CII-3b;

The sequence of this isoform differs from the canonical sequence as follows:
     27-60: Missing.
Isoform 4 (identifier: Q99643-4)

The sequence of this isoform differs from the canonical sequence as follows:
     27-60: Missing.
     81-169: GVSLFGMSAL...VLSSMGLAAM → DVGPRKRPED...PAWEKFSLFV
Isoform 5 (identifier: Q99643-5)

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

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 2929Mitochondrion By similarity
Chain30 – 169140Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
PRO_0000003634

Regions

Topological domain30 – 6233Mitochondrial matrix By similarity
Transmembrane63 – 9230Helical; By similarity
Topological domain93 – 11220Mitochondrial intermembrane By similarity
Transmembrane113 – 13725Helical; By similarity
Topological domain138 – 1447Mitochondrial matrix By similarity
Transmembrane145 – 16622Helical; By similarity
Topological domain167 – 1693Mitochondrial intermembrane By similarity

Sites

Metal binding1271Iron (heme axial ligand); shared with SDHD By similarity

Natural variations

Alternative sequence7 – 5953Missing in isoform 5.
VSP_041381
Alternative sequence27 – 6034Missing in isoform 3 and isoform 4.
VSP_041382
Alternative sequence81 – 16989GVSLF…GLAAM → DVGPRKRPEDSPAIPVWSGC PGSYCVVLYGAGSHVKKGGS QHHLPTHYYIHPSFCLSFLS PAWEKFSLFV in isoform 2 and isoform 4.
VSP_041383

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified May 1, 1997. Version 1.
Checksum: DF7CBD6D0CD49500

FASTA16918,610
        10         20         30         40         50         60 
MAALLLRHVG RHCLRAHFSP QLCIRNAVPL GTTAKEEMER FWNKNIGSNR PLSPHITIYS 

        70         80         90        100        110        120 
WSLPMAMSIC HRGTGIALSA GVSLFGMSAL LLPGNFESYL ELVKSLCLGP ALIHTAKFAL 

       130        140        150        160 
VFPLMYHTWN GIRHLMWDLG KGLKIPQLYQ SGVVVLVLTV LSSMGLAAM 

« Hide

Isoform 2 [UniParc].

Checksum: 971579A96F795D90
Show »

FASTA15016,650
Isoform 3 (CII-3b) [UniParc].

Checksum: 5F7A8BC812112D75
Show »

FASTA13514,770
Isoform 4 [UniParc].

Checksum: 36DFFD8CC2E6D4E8
Show »

FASTA11612,810
Isoform 5 [UniParc].

Checksum: AC702AE33DD1F118
Show »

FASTA11612,474

References

« Hide 'large scale' references
[1]"The cDNA sequence of human CII-3, an integral membrane protein subunit of complex II of the mitochondria electron transport chain."
Au H.C., Raval P.J., Scheffler I.E.
Submitted (MAY-1996) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
[2]"Cytochrome b in human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the components in liver mitochondria and chromosome assignment of the genes for the large (SDHC) and small (SDHD) subunits to 1q21 and 11q23."
Hirawake H., Taniwaki M., Tamura A., Kojima S., Kita K.
Cytogenet. Cell Genet. 79:132-138(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
Tissue: Liver.
[3]"Characterization of the human SDHC gene encoding one of the integral membrane proteins of succinate-quinone oxidoreductase in mitochondria."
Elbehti-Green A., Au H.C., Mascarello J.T., Ream-Robinson D., Scheffler I.E.
Gene 213:133-140(1998) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[4]"A human succinate-ubiquinone oxidoreductase CII-3 subunit gene ending in a polymorphic dinucleotide repeat is located within the sulfonylurea receptor (SUR) gene."
Wohllk N., Thomas P.M., Huang E., Cote G.J.
Mol. Genet. Metab. 65:187-190(1998) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 3).
Tissue: Peripheral blood.
[5]"Homo sapiens succinate dehydrogenase complex, subunit C mRNA, alternative splicing variants."
Hiatomi H., Kitano S., Kawano K., Hibi N.
Submitted (OCT-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS 2; 3; 4 AND 5).
[6]"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. expand/collapse author list , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
Tissue: Amygdala.
[7]"The DNA sequence and biological annotation of human chromosome 1."
Gregory S.G., Barlow K.F., McLay K.E., Kaul R., Swarbreck D., Dunham A., Scott C.E., Howe K.L., Woodfine K., Spencer C.C.A., Jones M.C., Gillson C., Searle S., Zhou Y., Kokocinski F., McDonald L., Evans R., Phillips K. expand/collapse author list , Atkinson A., Cooper R., Jones C., Hall R.E., Andrews T.D., Lloyd C., Ainscough R., Almeida J.P., Ambrose K.D., Anderson F., Andrew R.W., Ashwell R.I.S., Aubin K., Babbage A.K., Bagguley C.L., Bailey J., Beasley H., Bethel G., Bird C.P., Bray-Allen S., Brown J.Y., Brown A.J., Buckley D., Burton J., Bye J., Carder C., Chapman J.C., Clark S.Y., Clarke G., Clee C., Cobley V., Collier R.E., Corby N., Coville G.J., Davies J., Deadman R., Dunn M., Earthrowl M., Ellington A.G., Errington H., Frankish A., Frankland J., French L., Garner P., Garnett J., Gay L., Ghori M.R.J., Gibson R., Gilby L.M., Gillett W., Glithero R.J., Grafham D.V., Griffiths C., Griffiths-Jones S., Grocock R., Hammond S., Harrison E.S.I., Hart E., Haugen E., Heath P.D., Holmes S., Holt K., Howden P.J., Hunt A.R., Hunt S.E., Hunter G., Isherwood J., James R., Johnson C., Johnson D., Joy A., Kay M., Kershaw J.K., Kibukawa M., Kimberley A.M., King A., Knights A.J., Lad H., Laird G., Lawlor S., Leongamornlert D.A., Lloyd D.M., Loveland J., Lovell J., Lush M.J., Lyne R., Martin S., Mashreghi-Mohammadi M., Matthews L., Matthews N.S.W., McLaren S., Milne S., Mistry S., Moore M.J.F., Nickerson T., O'Dell C.N., Oliver K., Palmeiri A., Palmer S.A., Parker A., Patel D., Pearce A.V., Peck A.I., Pelan S., Phelps K., Phillimore B.J., Plumb R., Rajan J., Raymond C., Rouse G., Saenphimmachak C., Sehra H.K., Sheridan E., Shownkeen R., Sims S., Skuce C.D., Smith M., Steward C., Subramanian S., Sycamore N., Tracey A., Tromans A., Van Helmond Z., Wall M., Wallis J.M., White S., Whitehead S.L., Wilkinson J.E., Willey D.L., Williams H., Wilming L., Wray P.W., Wu Z., Coulson A., Vaudin M., Sulston J.E., Durbin R.M., Hubbard T., Wooster R., Dunham I., Carter N.P., McVean G., Ross M.T., Harrow J., Olson M.V., Beck S., Rogers J., Bentley D.R.
Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[8]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[9]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
Tissue: Brain, Mammary gland and Skeletal muscle.
[10]"Mutations in SDHC cause autosomal dominant paraganglioma, type 3."
Niemann S., Mueller U.
Nat. Genet. 26:268-270(2000) [PubMed] [Europe PMC] [Abstract]
Cited for: INVOLVEMENT IN PGL3.
[11]"Familial gastrointestinal stromal tumors and germ-line mutations."
McWhinney S.R., Pasini B., Stratakis C.A.
N. Engl. J. Med. 357:1054-1056(2007) [PubMed] [Europe PMC] [Abstract]
Cited for: INVOLVEMENT IN PGGSS.
[12]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U57877 mRNA. Translation: AAB41838.1.
D49737 mRNA. Translation: BAA31998.1.
AF039594 expand/collapse EMBL AC list , 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. Translation: CAH70271.1.
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.
CCDSCCDS1230.1. [Q99643-1]
CCDS41431.1. [Q99643-2]
CCDS41432.1. [Q99643-5]
CCDS44263.1. [Q99643-3]
CCDS60330.1. [Q99643-4]
RefSeqNP_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]
UniGeneHs.444472.

3D structure databases

ProteinModelPortalQ99643.
SMRQ99643. Positions 32-169.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid112292. 4 interactions.
IntActQ99643. 3 interactions.
MINTMINT-3059494.
STRING9606.ENSP00000356953.

Chemistry

DrugBankDB00139. Succinic acid.

PTM databases

PhosphoSiteQ99643.

Polymorphism databases

DMDM5915811.

Proteomic databases

MaxQBQ99643.
PaxDbQ99643.
PRIDEQ99643.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000342751; ENSP00000356952; ENSG00000143252. [Q99643-2]
ENST00000367975; ENSP00000356953; ENSG00000143252. [Q99643-1]
ENST00000392169; ENSP00000376009; ENSG00000143252. [Q99643-5]
ENST00000432287; ENSP00000390558; ENSG00000143252. [Q99643-3]
ENST00000513009; ENSP00000423260; ENSG00000143252. [Q99643-4]
GeneID6391.
KEGGhsa:6391.
UCSCuc001gag.3. human. [Q99643-1]
uc001gah.3. human. [Q99643-3]
uc001gai.3. human. [Q99643-2]
uc001gaj.3. human. [Q99643-5]
uc001gak.3. human. [Q99643-4]

Organism-specific databases

CTD6391.
GeneCardsGC01P161285.
GeneReviewsSDHC.
HGNCHGNC:10682. SDHC.
MIM602413. gene.
605373. phenotype.
606864. phenotype.
neXtProtNX_Q99643.
Orphanet97286. Carney-Stratakis syndrome.
201. Cowden syndrome.
44890. Gastrointestinal stromal tumor.
29072. Hereditary pheochromocytoma-paraganglioma.
PharmGKBPA35607.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG2009.
HOGENOMHOG000160253.
HOVERGENHBG003187.
InParanoidQ99643.
KOK00236.
OMALTLKGAY.
OrthoDBEOG7SV0WV.
PhylomeDBQ99643.
TreeFamTF313317.

Enzyme and pathway databases

BioCycMetaCyc:HS07014-MONOMER.
ReactomeREACT_111217. Metabolism.
UniPathwayUPA00223.

Gene expression databases

ArrayExpressQ99643.
BgeeQ99643.
CleanExHS_SDHC.
GenevestigatorQ99643.

Family and domain databases

InterProIPR018495. Succ_DH_cyt_bsu_CS.
IPR014314. Succ_DH_cytb556.
IPR014361. Succ_DH_cytb560.
IPR000701. Succ_DH_Fumarate_Rdtase_TM-su.
[Graphical view]
PfamPF01127. Sdh_cyt. 1 hit.
[Graphical view]
PIRSFPIRSF000178. SDH_cyt_b560. 1 hit.
TIGRFAMsTIGR02970. succ_dehyd_cytB. 1 hit.
PROSITEPS01000. SDH_CYT_1. 1 hit.
PS01001. SDH_CYT_2. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

GeneWikiSuccinate_dehydrogenase_complex_subunit_C.
GenomeRNAi6391.
NextBio24828.
PROQ99643.
SOURCESearch...

Entry information

Entry nameC560_HUMAN
AccessionPrimary (citable) accession number: Q99643
Secondary accession number(s): O75609 expand/collapse secondary AC list , Q3C259, Q3C2D8, Q3C2H4, Q5VTH3
Entry history
Integrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: May 1, 1997
Last modified: July 9, 2014
This is version 149 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

PATHWAY comments

Index of metabolic and biosynthesis pathways

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human chromosome 1

Human chromosome 1: entries, gene names and cross-references to MIM