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3.D.4.11.1
Cytochrome oxidase (Cox or CcO).  Reversible hydration-level changes of the cavity can be a key factor that regulates the branching of proton transfer events and therefore contributes to the vectorial efficiency of proton transport (Son et al. 2017). Cox16 is required for the assembly of the mitochondrial cytochrome c oxidase (respiratory chain complex IV (CIV)), possibly by promoting the insertion of copper into the active site of cytochrome c oxidase subunit II (MT-CO2/COX2) (Cerqua et al. 2018; Aich et al. 2018). Lipid composition affects the efficiency of the functional reconstitution of the cytochrome c oxidase (Hugentobler et al. 2020). The DeepCys program has been used to predict the functions of cysteine residues in Cox2 (Nallapareddy et al. 2021).

Accession Number:P00395
Protein Name:CoxI aka MT-CO1
Length:513
Molecular Weight:57041.00
Species:Homo sapiens (Human) [9606]
Number of TMSs:12
Location1 / Topology2 / Orientation3: Mitochondrion inner membrane1 / Multi-pass membrane protein2
Substrate hydron

Cross database links:

RefSeq: YP_003024028.1   
Entrez Gene ID: 4512   
Pfam: PF00115   
OMIM: 220110  phenotype
516030  gene
535000  phenotype
550500  phenotype
KEGG: hsa:4512   

Gene Ontology

GO:0016021 C:integral to membrane
GO:0005743 C:mitochondrial inner membrane
GO:0070469 C:respiratory chain
GO:0004129 F:cytochrome-c oxidase activity
GO:0009055 F:electron carrier activity
GO:0020037 F:heme binding
GO:0005515 F:protein binding
GO:0009060 P:aerobic respiration
GO:0006123 P:mitochondrial electron transport, cytochrom...
GO:0006810 P:transport

References (14)

[1] “Sequence and organization of the human mitochondrial genome.”  Anderson S.et.al.   7219534
[2] “Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs.”  Horai S.et.al.   7530363
[3] “Lineage-specific selection in human mtDNA: lack of polymorphisms in a segment of MTND5 gene in haplogroup J.”  Moilanen J.S.et.al.   12949126
[4] “Mitochondrial genome variation and the origin of modern humans.”  Ingman M.et.al.   11130070
[5] “Mitochondrial genome variation and evolutionary history of Australian and New Guinean aborigines.”  Ingman M.et.al.   12840039
[6] “Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in Caucasians.”  Coble M.D.et.al.   14760490
[7] “Cloning in single-stranded bacteriophage as an aid to rapid DNA sequencing.”  Sanger F.et.al.   6260957
[8] “Normal variants of human mitochondrial DNA and translation products: the building of a reference data base.”  Marzuki S.et.al.   1757091
[9] “A mitochondrial DNA variant, identified in Leber hereditary optic neuropathy patients, which extends the amino acid sequence of cytochrome c oxidase subunit I.”  Brown M.D.et.al.   1322638
[10] “Heteroplasmic point mutations of mitochondrial DNA affecting subunit I of cytochrome c oxidase in two patients with acquired idiopathic sideroblastic anemia.”  Gattermann N.et.al.   9389715
[11] “MtDNA mutations associated with sideroblastic anaemia cause a defect of mitochondrial cytochrome c oxidase.”  Broker S.et.al.   9851701
[12] “Recurrent myoglobinuria due to a nonsense mutation in the COX I gene of mitochondrial DNA.”  Karadimas C.L.et.al.   10980727
[13] “Metabolic consequences of a novel missense mutation of the mtDNA CO I gene.”  Varlamov D.A.et.al.   12140182
[14] “Introducing a novel human mtDNA mutation into the Paracoccus denitrificans COX I gene explains functional deficits in a patient.”  Lucioli S.et.al.   16284789
Structure:
5Z62     

External Searches:

Analyze:

Predict TMSs (Predict number of transmembrane segments)
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FASTA formatted sequence
1:	MFADRWLFST NHKDIGTLYL LFGAWAGVLG TALSLLIRAE LGQPGNLLGN DHIYNVIVTA 
61:	HAFVMIFFMV MPIMIGGFGN WLVPLMIGAP DMAFPRMNNM SFWLLPPSLL LLLASAMVEA 
121:	GAGTGWTVYP PLAGNYSHPG ASVDLTIFSL HLAGVSSILG AINFITTIIN MKPPAMTQYQ 
181:	TPLFVWSVLI TAVLLLLSLP VLAAGITMLL TDRNLNTTFF DPAGGGDPIL YQHLFWFFGH 
241:	PEVYILILPG FGMISHIVTY YSGKKEPFGY MGMVWAMMSI GFLGFIVWAH HMFTVGMDVD 
301:	TRAYFTSATM IIAIPTGVKV FSWLATLHGS NMKWSAAVLW ALGFIFLFTV GGLTGIVLAN 
361:	SSLDIVLHDT YYVVAHFHYV LSMGAVFAIM GGFIHWFPLF SGYTLDQTYA KIHFTIMFIG 
421:	VNLTFFPQHF LGLSGMPRRY SDYPDAYTTW NILSSVGSFI SLTAVMLMIF MIWEAFASKR 
481:	KVLMVEEPSM NLEWLYGCPP PYHTFEEPVY MKS