CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-005976
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Carbamoyl-phosphate synthase [ammonia], mitochondrial 
Protein Synonyms/Alias
 Carbamoyl-phosphate synthetase I; CPSase I 
Gene Name
 CPS1 
Gene Synonyms/Alias
  
Created Date
 July 27, 2013 
Organism
 Homo sapiens (Human) 
NCBI Taxa ID
 9606 
Lysine Modification
Position
Peptide
Type
References
7*MKEENVKTVLMNPNubiquitination[1]
76LFKRGVLKEYGVKVLubiquitination[1]
109KLNEINEKIAPSFAVubiquitination[1]
300GIPLPEIKNVVSGKTubiquitination[1]
342SRIGSSMKSVGEVMAubiquitination[1]
360TFEESFQKALRMCHPubiquitination[1]
380TPRLPMNKEWPSNLDubiquitination[1]
405TRIYAIAKAIDDNMSubiquitination[1]
441LNMEKTLKGLNSESMubiquitination[1]
656KVAQAPWKAVNTLNEubiquitination[1]
717EHPVVLTKFVEGAREubiquitination[1]
732VEMDAVGKDGRVISHubiquitination[1]
818RSFPFVSKTLGVDFIubiquitination[1]
905AMLSTGFKIPQKGILubiquitination[1]
1020LTNFQVTKLFAEAVQubiquitination[1]
Reference
 [1] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661] 
Functional Description
 Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. 
Sequence Annotation
 DOMAIN 219 404 Glutamine amidotransferase type-1.
 DOMAIN 551 743 ATP-grasp 1.
 DOMAIN 1093 1284 ATP-grasp 2.
 REGION 39 218 Anthranilate phosphoribosyltransferase
 BINDING 1391 1391 Allosteric activator (Probable).
 BINDING 1394 1394 Allosteric activator (Probable).
 BINDING 1410 1410 Allosteric activator (Probable).
 BINDING 1437 1437 Allosteric activator (Probable).
 BINDING 1440 1440 Allosteric activator (Probable).
 BINDING 1449 1449 Allosteric activator (Probable).
 MOD_RES 55 55 N6-acetyllysine (By similarity).
 MOD_RES 119 119 N6-acetyllysine (By similarity).
 MOD_RES 287 287 N6-acetyllysine; alternate (By
 MOD_RES 287 287 N6-succinyllysine; alternate (By
 MOD_RES 527 527 N6-acetyllysine (By similarity).
 MOD_RES 841 841 N6-acetyllysine (By similarity).
 MOD_RES 892 892 N6-acetyllysine (By similarity).
 MOD_RES 898 898 Phosphoserine (By similarity).
 MOD_RES 1291 1291 N6-acetyllysine; alternate (By
 MOD_RES 1291 1291 N6-succinyllysine; alternate (By  
Keyword
 3D-structure; Acetylation; Allosteric enzyme; Alternative splicing; ATP-binding; Complete proteome; Disease mutation; Ligase; Mitochondrion; Nucleotide-binding; Nucleus; Phosphoprotein; Polymorphism; Reference proteome; Repeat; Transit peptide; Urea cycle. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 1500 AA 
Protein Sequence
MKEENVKTVL MNPNIASVQT NEVGLKQADT VYFLPITPQF VTEVIKAEQP DGLILGMGGQ 60
TALNCGVELF KRGVLKEYGV KVLGTSVESI MATEDRQLFS DKLNEINEKI APSFAVESIE 120
DALKAADTIG YPVMIRSAYA LGGLGSGICP NRETLMDLST KAFAMTNQIL VEKSVTGWKE 180
IEYEVVRDAD DNCVTVCNME NVDAMGVHTG DSVVVAPAQT LSNAEFQMLR RTSINVVRHL 240
GIVGECNIQF ALHPTSMEYC IIEVNARLSR SSALASKATG YPLAFIAAKI ALGIPLPEIK 300
NVVSGKTSAC FEPSLDYMVT KIPRWDLDRF HGTSSRIGSS MKSVGEVMAI GRTFEESFQK 360
ALRMCHPSIE GFTPRLPMNK EWPSNLDLRK ELSEPSSTRI YAIAKAIDDN MSLDEIEKLT 420
YIDKWFLYKM RDILNMEKTL KGLNSESMTE ETLKRAKEIG FSDKQISKCL GLTEAQTREL 480
RLKKNIHPWV KQIDTLAAEY PSVTNYLYVT YNGQEHDVNF DDHGMMVLGC GPYHIGSSVE 540
FDWCAVSSIR TLRQLGKKTV VVNCNPETVS TDFDECDKLY FEELSLERIL DIYHQEACGG 600
CIISVGGQIP NNLAVPLYKN GVKIMGTSPL QIDRAEDRSI FSAVLDELKV AQAPWKAVNT 660
LNEALEFAKS VDYPCLLRPS YVLSGSAMNV VFSEDEMKKF LEEATRVSQE HPVVLTKFVE 720
GAREVEMDAV GKDGRVISHA ISEHVEDAGV HSGDATLMLP TQTISQGAIE KVKDATRKIA 780
KAFAISGPFN VQFLVKGNDV LVIECNLRAS RSFPFVSKTL GVDFIDVATK VMIGENVDEK 840
HLPTLDHPII PADYVAIKAP MFSWPRLRDA DPILRCEMAS TGEVACFGEG IHTAFLKAML 900
STGFKIPQKG ILIGIQQSFR PRFLGVAEQL HNEGFKLFAT EATSDWLNAN NVPATPVAWP 960
SQEGQNPSLS SIRKLIRDGS IDLVINLPNN NTKFVHDNYV IRRTAVDSGI PLLTNFQVTK 1020
LFAEAVQKSR KVDSKSLFHY RQYSAGKAA 1049 
Gene Ontology
 GO:0042645; C:mitochondrial nucleoid; IDA:BHF-UCL.
 GO:0005730; C:nucleolus; IEA:UniProtKB-SubCell.
 GO:0005524; F:ATP binding; IEA:UniProtKB-KW.
 GO:0004087; F:carbamoyl-phosphate synthase (ammonia) activity; IMP:BHF-UCL.
 GO:0046872; F:metal ion binding; IEA:InterPro.
 GO:0072341; F:modified amino acid binding; IDA:UniProtKB.
 GO:0070409; P:carbamoyl phosphate biosynthetic process; IMP:UniProtKB.
 GO:0019240; P:citrulline biosynthetic process; NAS:BHF-UCL.
 GO:0006543; P:glutamine catabolic process; IEA:InterPro.
 GO:0005980; P:glycogen catabolic process; IMP:BHF-UCL.
 GO:0050667; P:homocysteine metabolic process; IDA:UniProtKB.
 GO:0046209; P:nitric oxide metabolic process; IMP:BHF-UCL.
 GO:0045909; P:positive regulation of vasodilation; IMP:BHF-UCL.
 GO:0032496; P:response to lipopolysaccharide; IDA:UniProtKB.
 GO:0019433; P:triglyceride catabolic process; IMP:BHF-UCL.
 GO:0000050; P:urea cycle; NAS:BHF-UCL. 
Interpro
 IPR011761; ATP-grasp.
 IPR013815; ATP_grasp_subdomain_1.
 IPR013816; ATP_grasp_subdomain_2.
 IPR006275; CarbamoylP_synth_lsu.
 IPR005481; CarbamoylP_synth_lsu_N.
 IPR005480; CarbamoylP_synth_lsu_oligo.
 IPR006274; CarbamoylP_synth_ssu.
 IPR002474; CarbamoylP_synth_ssu_N.
 IPR005479; CbamoylP_synth_lsu-like_ATP-bd.
 IPR005483; CbamoylP_synth_lsu_CPSase_dom.
 IPR017926; GATASE.
 IPR011607; MGS-like_dom.
 IPR016185; PreATP-grasp_dom. 
Pfam
 PF00289; CPSase_L_chain
 PF02786; CPSase_L_D2
 PF02787; CPSase_L_D3
 PF00988; CPSase_sm_chain
 PF00117; GATase
 PF02142; MGS 
SMART
 SM01096; CPSase_L_D3
 SM01097; CPSase_sm_chain
 SM00851; MGS 
PROSITE
 PS50975; ATP_GRASP
 PS00866; CPSASE_1
 PS00867; CPSASE_2
 PS51273; GATASE_TYPE_1 
PRINTS
 PR00098; CPSASE.