CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-019594
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Twinkle protein, mitochondrial 
Protein Synonyms/Alias
 Progressive external ophthalmoplegia 1 protein; T7 gp4-like protein with intramitochondrial nucleoid localization; T7-like mitochondrial DNA helicase 
Gene Name
 PEO1 
Gene Synonyms/Alias
 C10orf2 
Created Date
 July 27, 2013 
Organism
 Homo sapiens (Human) 
NCBI Taxa ID
 9606 
Lysine Modification
Position
Peptide
Type
References
144REGFLLSKAPEFEDSubiquitination[1]
365TALPAWHKSIVSFRQubiquitination[2]
Reference
 [1] Systematic and quantitative assessment of the ubiquitin-modified proteome.
 Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
 Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983]
 [2] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961] 
Functional Description
 Involved in mitochondrial DNA (mtDNA) metabolism. Could function as an adenine nucleotide-dependent DNA helicase. Function inferred to be critical for lifetime maintenance of mtDNA integrity. In vitro, forms in combination with POLG, a processive replication machinery, which can use double-stranded DNA (dsDNA) as template to synthesize single-stranded DNA (ssDNA) molecules. May be a key regulator of mtDNA copy number in mammals. 
Sequence Annotation
 DOMAIN 384 635 SF4 helicase.
 NP_BIND 415 422 ATP (By similarity).  
Keyword
 Alternative splicing; ATP-binding; Complete proteome; Disease mutation; DNA replication; Helicase; Hydrolase; Mitochondrion; Mitochondrion nucleoid; Neurodegeneration; Neuropathy; Nucleotide-binding; Polymorphism; Progressive external ophthalmoplegia; Reference proteome; Transit peptide. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 684 AA 
Protein Sequence
MWVLLRSGYP LRILLPLRGE WMGRRGLPRN LAPGPPRRRY RKETLQALDM PVLPVTATEI 60
RQYLRGHGIP FQDGHSCLRA LSPFAESSQL KGQTGVTTSF SLFIDKTTGH FLCMTSLAEG 120
SWEDFQASVE GRGDGAREGF LLSKAPEFED SEEVRRIWNR AIPLWELPDQ EEVQLADTMF 180
GLTKVTDDTL KRFSVRYLRP ARSLVFPWFS PGGSGLRGLK LLEAKCQGDG VSYEETTIPR 240
PSAYHNLFGL PLISRRDAEV VLTSRELDSL ALNQSTGLPT LTLPRGTTCL PPALLPYLEQ 300
FRRIVFWLGD DLRSWEAAKL FARKLNPKRC FLVRPGDQQP RPLEALNGGF NLSRILRTAL 360
PAWHKSIVSF RQLREEVLGE LSNVEQAAGL RWSRFPDLNR ILKGHRKGEL TVFTGPTGSG 420
KTTFISEYAL DLCSQGVNTL WGSFEISNVR LARVMLTQFA EGRLEDQLDK YDHWADRFED 480
LPLYFMTFHG QQSIRTVIDT MQHAVYVYDI CHVIIDNLQF MMGHEQLSTD RIAAQDYIIG 540
VFRKFATDNN CHVTLVIHPR KEDDDKELQT ASIFGSAKAS QEADNVLILQ DRKLVTGPGK 600
RYLQVSKNRF DGDVGVFPLE FNKNSLTFSI PPKNKARLKK IKDDTGPVAK KPSSGKKGAT 660
TQNSEICSGQ APTPDQPDTS KRSK 684 
Gene Ontology
 GO:0042645; C:mitochondrial nucleoid; IDA:UniProtKB.
 GO:0043139; F:5'-3' DNA helicase activity; IDA:UniProtKB.
 GO:0005524; F:ATP binding; IEA:UniProtKB-KW.
 GO:0003697; F:single-stranded DNA binding; IDA:UniProtKB.
 GO:0008219; P:cell death; IEA:UniProtKB-KW.
 GO:0006268; P:DNA unwinding involved in replication; IEA:Compara.
 GO:0006264; P:mitochondrial DNA replication; IMP:UniProtKB.
 GO:0034214; P:protein hexamerization; IDA:UniProtKB.
 GO:0051260; P:protein homooligomerization; IDA:UniProtKB.
 GO:0006390; P:transcription from mitochondrial promoter; IMP:UniProtKB. 
Interpro
 IPR007694; DNA_helicase_DnaB-like_C.
 IPR027417; P-loop_NTPase.
 IPR027032; Twinkle. 
Pfam
  
SMART
  
PROSITE
 PS51199; SF4_HELICASE 
PRINTS