Hmdb loader
Identification
HMDB Protein ID HMDBP09268
Secondary Accession Numbers
  • 15096
Name E3 ubiquitin-protein ligase UBR1
Synonyms
  1. N-recognin-1
  2. Ubiquitin-protein ligase E3-alpha-1
  3. Ubiquitin-protein ligase E3-alpha-I
Gene Name UBR1
Protein Type Enzyme
Biological Properties
General Function Involved in protein catabolic process
Specific Function E3 ubiquitin-protein ligase which is a component of the N-end rule pathway. Recognizes and binds to proteins bearing specific N-terminal residues that are destabilizing according to the N-end rule, leading to their ubiquitination and subsequent degradation. May be involved in pancreatic homeostasis. Binds leucine and is a negative regulator of the leucine-mTOR signaling pathway, thereby controlling cell growth
Pathways Not Available
Reactions Not Available
GO Classification
Function
ion binding
cation binding
metal ion binding
binding
catalytic activity
small conjugating protein ligase activity
transition metal ion binding
zinc ion binding
ligase activity
ligase activity, forming carbon-nitrogen bonds
acid-amino acid ligase activity
ubiquitin-protein ligase activity
Process
metabolic process
catabolic process
macromolecule catabolic process
protein catabolic process
Cellular Location
  1. Cytoplasm
  2. cytosol
Gene Properties
Chromosome Location Chromosome:1
Locus 15q13
SNPs UBR1
Gene Sequence
>5250 bp
ATGGCGGACGAGGAGGCTGGAGGTACTGAGAGGATGGAAATCAGCGCGGAGTTACCCCAG
ACCCCTCAGCGTCTGGCATCTTGGTGGGATCAGCAAGTTGATTTTTATACTGCTTTCTTG
CATCATTTGGCACAATTGGTGCCAGAAATTTACTTTGCTGAAATGGACCCAGACTTGGAA
AAGCAGGAGGAAAGTGTACAAATGTCAATATTCACTCCACTGGAATGGTACTTATTTGGA
GAAGATCCAGATATTTGCTTAGAGAAATTGAAGCACAGTGGAGCATTTCAGCTTTGTGGG
AGGGTTTTCAAAAGTGGAGAGACAACCTATTCTTGCAGGGATTGTGCAATTGATCCAACA
TGTGTACTCTGTATGGACTGCTTCCAGGACAGTGTTCATAAAAATCATCGTTACAAGATG
CATACTTCTACTGGAGGAGGGTTCTGTGACTGTGGAGACACAGAGGCATGGAAAACTGGC
CCTTTTTGTGTAAATCATGAACCTGGAAGAGCAGGTACTATAAAAGAGAATTCACGCTGT
CCGTTGAATGAAGAGGTAATTGTCCAAGCCAGGAAAATATTTCCTTCAGTGATAAAATAT
GTCGTAGAAATGACTATATGGGAAGAGGAAAAAGAACTGCCTCCTGAACTCCAGATAAGG
GAGAAAAATGAAAGATACTATTGTGTCCTTTTCAATGATGAACACCATTCATATGACCAC
GTCATATACAGCCTACAAAGAGCTCTTGACTGTGAGCTCGCAGAGGCCCAGTTGCATACC
ACTGCCATTGACAAAGAGGGTCGTCGGGCTGTTAAAGCGGGAGCTTATGCTGCTTGCCAG
GAAGCAAAGGAAGATATAAAGAGTCATTCAGAAAATGTCTCTCAACATCCACTTCATGTA
GAAGTATTACACTCAGAGATTATGGCTCATCAGAAATTTGCTTTGCGTCTTGGTTCCTGG
ATGAACAAAATTATGAGCTATTCAAGTGACTTTAGGCAGATCTTTTGCCAAGCATGCCTT
AGAGAAGAACCTGACTCGGAGAATCCCTGTCTCATAAGCAGGTTAATGCTTTGGGATGCA
AAGCTTTATAAAGGTGCCCGTAAGATCCTTCATGAATTGATCTTCAGCAGTTTTTTTATG
GAGATGGAATACAAAAAACTCTTTGCTATGGAATTTGTGAAGTATTATAAACAACTGCAG
AAAGAATATATCAGTGATGATCATGACAGAAGTATCTCTATAACTGCACTTTCAGTTCAG
ATGTTTACTGTTCCTACTCTGGCTCGACATCTTATTGAAGAGCAGAATGTTATCTCTGTC
ATTACTGAAACTCTGCTAGAAGTTTTACCTGAGTACTTGGACAGGAACAATAAATTCAAC
TTCCAGGGTTATAGCCAGGACAAATTGGGAAGAGTATATGCAGTAATATGTGACCTAAAG
TATATCCTGATCAGCAAACCCACAATATGGACAGAAAGATTAAGAATGCAGTTCCTTGAA
GGTTTTCGATCTTTTTTGAAGATTCTTACCTGTATGCAGGGAATGGAAGAAATCCGAAGA
CAGGTTGGGCAACACATTGAAGTGGATCCTGATTGGGAGGCTGCCATTGCTATACAGATG
CAATTGAAGAATATTTTACTCATGTTCCAAGAGTGGTGTGCTTGTGATGAAGAACTCTTA
CTTGTGGCTTATAAAGAATGTCACAAAGCTGTGATGAGGTGCAGTACCAGTTTCATATCT
AGTAGCAAGACAGTAGTACAATCGTGTGGACATAGTTTGGAAACAAAGTCCTACAGAGTA
TCTGAGGATCTTGTAAGCATACATCTGCCACTCTCTAGGACCCTTGCTGGTCTTCATGTA
CGTTTAAGCAGGCTGGGTGCTGTTTCAAGACTGCATGAATTTGTGTCTTTTGAGGACTTT
CAAGTAGAGGTACTAGTGGAATATCCTTTACGTTGTCTGGTGTTGGTTGCCCAGGTTGTT
GCTGAGATGTGGCGAAGAAATGGACTGTCTCTTATTAGCCAGGTGTTTTATTACCAAGAT
GTTAAGTGCAGAGAAGAAATGTATGATAAAGATATCATCATGCTTCAGATTGGTGCATCT
TTAATGGATCCCAATAAGTTCTTGTTACTGGTACTTCAGAGGTATGAACTTGCCGAGGCT
TTTAACAAGACCATATCTACAAAAGACCAGGATTTGATTAAACAATATAATACACTAATA
GAAGAAATGCTTCAGGTCCTCATCTATATTGTGGGTGAGCGTTATGTACCTGGAGTGGGA
AATGTGACCAAAGAAGAGGTCACAATGAGAGAAATCATTCACTTGCTTTGCATTGAACCC
ATGCCACACAGTGCCATTGCCAAAAATTTACCTGAGAATGAAAATAATGAAACTGGCTTA
GAGAATGTCATAAACAAAGTGGCCACATTTAAGAAACCAGGTGTATCAGGCCATGGAGTT
TATGAACTAAAAGATGAATCACTGAAAGACTTCAATATGTACTTTTATCATTACTCCAAA
ACCCAGCATAGCAAGGCTGAACATATGCAGAAGAAAAGGAGAAAACAAGAAAACAAAGAT
GAAGCATTGCCGCCACCACCACCTCCTGAATTCTGCCCTGCTTTCAGCAAAGTGATTAAC
CTTCTCAACTGTGATATCATGATGTACATTCTCAGGACCGTATTTGAGCGGGCAATAGAC
ACAGATTCTAACTTGTGGACCGAAGGGATGCTCCAAATGGCTTTTCATATTCTGGCATTG
GGTTTACTAGAAGAGAAGCAACAGCTTCAAAAAGCTCCTGAAGAAGAAGTAACATTTGAC
TTTTATCATAAGGCTTCAAGATTGGGAAGTTCAGCCATGAATATACAAATGCTTTTGGAA
AAACTCAAAGGAATTCCCCAGTTAGAAGGCCAGAAGGACATGATAACGTGGATACTTCAG
ATGTTTGACACAGTGAAGCGATTAAGAGAAAAATCTTGTTTAATTGTAGCAACCACATCA
GGATCGGAATCTATTAAGAATGATGAGATTACTCATGATAAAGAAAAAGCAGAACGAAAA
AGAAAAGCTGAAGCTGCTAGGCTACATCGCCAGAAGATCATGGCTCAGATGTCTGCCTTA
CAGAAAAACTTCATTGAAACTCATAAACTCATGTATGACAATACATCAGAAATGCCTGGG
AAAGAAGATTCCATTATGGAGGAAGAGAGCACCCCAGCAGTCAGTGACTACTCTAGAATT
GCTTTGGGTCCTAAACGGGGTCCATCTGTTACTGAAAAGGAGGTGCTGACGTGCATCCTT
TGCCAAGAAGAACAGGAGGTGAAAATAGAAAATAATGCCATGGTATTATCGGCCTGTGTC
CAGAAATCTACTGCCTTAACCCAGCACAGGGGAAAACCCATAGAACTCTCAGGAGAAGCC
CTAGACCCACTTTTCATGGATCCAGACTTGGCATATGGAACTTATACAGGAAGCTGTGGT
CATGTAATGCACGCAGTGTGCTGGCAGAAGTATTTTGAAGCTGTACAGCTGAGCTCTCAG
CAGCGCATTCATGTTGACCTTTTTGACTTGGAAAGTGGAGAATATCTTTGCCCTCTTTGC
AAATCTCTGTGCAATACTGTGATCCCCATTATTCCTTTGCAACCTCAAAAGATAAACAGT
GAGAATGCAGATGCTCTTGCTCAACTTTTGACCCTGGCACGGTGGATACAGACTGTTCTG
GCCAGAATATCAGGTTATAATATAAGACATGCTAAAGGAGAAAACCCAATTCCTATTTTC
TTTAATCAAGGAATGGGAGATTCTACTTTGGAGTTCCATTCCATCCTGAGTTTTGGCGTT
GAGTCTTCGATTAAATATTCAAATAGCATCAAGGAAATGGTTATTCTCTTTGCCACAACA
ATTTATAGAATTGGATTGAAAGTGCCACCTGATGAAAGGGATCCTCGAGTCCCCATGCTG
ACCTGGAGCACCTGCGCTTTCACTATCCAGGCAATTGAAAATCTATTGGGAGATGAAGGA
AAACCTCTGTTTGGAGCACTTCAAAATAGGCAGCATAATGGTCTGAAAGCATTAATGCAG
TTTGCAGTTGCACAGAGGATTACCTGTCCTCAGGTCCTGATACAGAAACATCTGGTTCGT
CTTCTATCAGTTGTTCTTCCTAACATAAAATCAGAAGATACACCATGCCTTCTGTCTATA
GATCTGTTTCATGTTTTGGTGGGTGCTGTGTTAGCATTCCCATCCTTGTATTGGGATGAC
CCTGTTGATCTGCAGCCTTCTTCAGTTAGTTCTTCCTATAACCACCTTTATCTCTTCCAT
TTGATCACCATGGCACACATGCTTCAGATACTACTTACAGTAGACACAGGCCTACCCCTT
GCTCAGGTTCAAGAAGACAGTGAAGAGGCTCATTCCGCATCTTCTTTCTTTGCAGAAATT
TCTCAATATACAAGTGGCTCCATTGGGTGTGATATTCCTGGCTGGTATTTGTGGGTCTCA
CTGAAGAATGGCATCACCCCTTATCTTCGCTGTGCTGCATTGTTTTTCCACTATTTACTT
GGGGTAACTCCGCCTGAGGAACTGCATACCAATTCTGCAGAAGGAGAGTACAGTGCACTC
TGTAGCTATCTATCTTTACCTACAAATTTGTTCCTGCTCTTCCAGGAATATTGGGATACT
GTAAGGCCCTTGCTCCAGAGGTGGTGTGCAGATCCTGCCTTACTAAACTGTTTGAAGCAA
AAAAACACCGTGGTCAGGTACCCTAGAAAAAGAAATAGTTTGATAGAGCTTCCTGATGAC
TATAGCTGCCTCCTGAATCAAGCTTCTCATTTCAGGTGCCCACGGTCTGCAGATGATGAG
CGAAAGCATCCTGTCCTCTGCCTTTTCTGTGGGGCTATACTATGTTCTCAGAACATTTGC
TGCCAGGAAATTGTGAACGGGGAAGAGGTTGGAGCTTGCATTTTTCACGCACTTCACTGT
GGAGCCGGAGTCTGCATTTTCCTAAAAATCAGAGAATGCCGAGTGGTCCTGGTTGAAGGT
AAAGCCAGAGGCTGTGCCTATCCAGCTCCTTACTTGGATGAATATGGAGAAACAGACCCT
GGCCTGAAGAGGGGCAACCCCCTTCATTTATCTCGTGAGCGGTATCGGAAGCTCCATTTG
GTCTGGCAACAACACTGCATTATAGAAGAGATTGCTAGGAGCCAAGAGACTAATCAGATG
TTATTTGGATTCAACTGGCAGTTACTGTGA
Protein Properties
Number of Residues 1749
Molecular Weight 200208.8
Theoretical pI 5.91
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • None
Protein Sequence
>E3 ubiquitin-protein ligase UBR1
MADEEAGGTERMEISAELPQTPQRLASWWDQQVDFYTAFLHHLAQLVPEIYFAEMDPDLE
KQEESVQMSIFTPLEWYLFGEDPDICLEKLKHSGAFQLCGRVFKSGETTYSCRDCAIDPT
CVLCMDCFQDSVHKNHRYKMHTSTGGGFCDCGDTEAWKTGPFCVNHEPGRAGTIKENSRC
PLNEEVIVQARKIFPSVIKYVVEMTIWEEEKELPPELQIREKNERYYCVLFNDEHHSYDH
VIYSLQRALDCELAEAQLHTTAIDKEGRRAVKAGAYAACQEAKEDIKSHSENVSQHPLHV
EVLHSEIMAHQKFALRLGSWMNKIMSYSSDFRQIFCQACLREEPDSENPCLISRLMLWDA
KLYKGARKILHELIFSSFFMEMEYKKLFAMEFVKYYKQLQKEYISDDHDRSISITALSVQ
MFTVPTLARHLIEEQNVISVITETLLEVLPEYLDRNNKFNFQGYSQDKLGRVYAVICDLK
YILISKPTIWTERLRMQFLEGFRSFLKILTCMQGMEEIRRQVGQHIEVDPDWEAAIAIQM
QLKNILLMFQEWCACDEELLLVAYKECHKAVMRCSTSFISSSKTVVQSCGHSLETKSYRV
SEDLVSIHLPLSRTLAGLHVRLSRLGAVSRLHEFVSFEDFQVEVLVEYPLRCLVLVAQVV
AEMWRRNGLSLISQVFYYQDVKCREEMYDKDIIMLQIGASLMDPNKFLLLVLQRYELAEA
FNKTISTKDQDLIKQYNTLIEEMLQVLIYIVGERYVPGVGNVTKEEVTMREIIHLLCIEP
MPHSAIAKNLPENENNETGLENVINKVATFKKPGVSGHGVYELKDESLKDFNMYFYHYSK
TQHSKAEHMQKKRRKQENKDEALPPPPPPEFCPAFSKVINLLNCDIMMYILRTVFERAID
TDSNLWTEGMLQMAFHILALGLLEEKQQLQKAPEEEVTFDFYHKASRLGSSAMNIQMLLE
KLKGIPQLEGQKDMITWILQMFDTVKRLREKSCLIVATTSGSESIKNDEITHDKEKAERK
RKAEAARLHRQKIMAQMSALQKNFIETHKLMYDNTSEMPGKEDSIMEEESTPAVSDYSRI
ALGPKRGPSVTEKEVLTCILCQEEQEVKIENNAMVLSACVQKSTALTQHRGKPIELSGEA
LDPLFMDPDLAYGTYTGSCGHVMHAVCWQKYFEAVQLSSQQRIHVDLFDLESGEYLCPLC
KSLCNTVIPIIPLQPQKINSENADALAQLLTLARWIQTVLARISGYNIRHAKGENPIPIF
FNQGMGDSTLEFHSILSFGVESSIKYSNSIKEMVILFATTIYRIGLKVPPDERDPRVPML
TWSTCAFTIQAIENLLGDEGKPLFGALQNRQHNGLKALMQFAVAQRITCPQVLIQKHLVR
LLSVVLPNIKSEDTPCLLSIDLFHVLVGAVLAFPSLYWDDPVDLQPSSVSSSYNHLYLFH
LITMAHMLQILLTVDTGLPLAQVQEDSEEAHSASSFFAEISQYTSGSIGCDIPGWYLWVS
LKNGITPYLRCAALFFHYLLGVTPPEELHTNSAEGEYSALCSYLSLPTNLFLLFQEYWDT
VRPLLQRWCADPALLNCLKQKNTVVRYPRKRNSLIELPDDYSCLLNQASHFRCPRSADDE
RKHPVLCLFCGAILCSQNICCQEIVNGEEVGACIFHALHCGAGVCIFLKIRECRVVLVEG
KARGCAYPAPYLDEYGETDPGLKRGNPLHLSRERYRKLHLVWQQHCIIEEIARSQETNQM
LFGFNWQLL
GenBank ID Protein 28372497
UniProtKB/Swiss-Prot ID Q8IWV7
UniProtKB/Swiss-Prot Entry Name UBR1_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_174916.2
GeneCard ID UBR1
GenAtlas ID UBR1
HGNC ID HGNC:16808
References
General References
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  3. Mayya V, Lundgren DH, Hwang SI, Rezaul K, Wu L, Eng JK, Rodionov V, Han DK: Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions. Sci Signal. 2009 Aug 18;2(84):ra46. doi: 10.1126/scisignal.2000007. [PubMed:19690332 ]
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  8. Bechtel S, Rosenfelder H, Duda A, Schmidt CP, Ernst U, Wellenreuther R, Mehrle A, Schuster C, Bahr A, Blocker H, Heubner D, Hoerlein A, Michel G, Wedler H, Kohrer K, Ottenwalder B, Poustka A, Wiemann S, Schupp I: The full-ORF clone resource of the German cDNA Consortium. BMC Genomics. 2007 Oct 31;8:399. [PubMed:17974005 ]
  9. Kwak KS, Zhou X, Solomon V, Baracos VE, Davis J, Bannon AW, Boyle WJ, Lacey DL, Han HQ: Regulation of protein catabolism by muscle-specific and cytokine-inducible ubiquitin ligase E3alpha-II during cancer cachexia. Cancer Res. 2004 Nov 15;64(22):8193-8. [PubMed:15548684 ]
  10. Dgany O, Avidan N, Delaunay J, Krasnov T, Shalmon L, Shalev H, Eidelitz-Markus T, Kapelushnik J, Cattan D, Pariente A, Tulliez M, Cretien A, Schischmanoff PO, Iolascon A, Fibach E, Koren A, Rossler J, Le Merrer M, Yaniv I, Zaizov R, Ben-Asher E, Olender T, Lancet D, Beckmann JS, Tamary H: Congenital dyserythropoietic anemia type I is caused by mutations in codanin-1. Am J Hum Genet. 2002 Dec;71(6):1467-74. Epub 2002 Nov 14. [PubMed:12434312 ]
  11. Kwon YT, Reiss Y, Fried VA, Hershko A, Yoon JK, Gonda DK, Sangan P, Copeland NG, Jenkins NA, Varshavsky A: The mouse and human genes encoding the recognition component of the N-end rule pathway. Proc Natl Acad Sci U S A. 1998 Jul 7;95(14):7898-903. [PubMed:9653112 ]
  12. Yin J, Kwon YT, Varshavsky A, Wang W: RECQL4, mutated in the Rothmund-Thomson and RAPADILINO syndromes, interacts with ubiquitin ligases UBR1 and UBR2 of the N-end rule pathway. Hum Mol Genet. 2004 Oct 15;13(20):2421-30. Epub 2004 Aug 18. [PubMed:15317757 ]
  13. Kume K, Iizumi Y, Shimada M, Ito Y, Kishi T, Yamaguchi Y, Handa H: Role of N-end rule ubiquitin ligases UBR1 and UBR2 in regulating the leucine-mTOR signaling pathway. Genes Cells. 2010 Apr 1;15(4):339-49. doi: 10.1111/j.1365-2443.2010.01385.x. Epub 2010 Mar 16. [PubMed:20298436 ]
  14. Zenker M, Mayerle J, Lerch MM, Tagariello A, Zerres K, Durie PR, Beier M, Hulskamp G, Guzman C, Rehder H, Beemer FA, Hamel B, Vanlieferinghen P, Gershoni-Baruch R, Vieira MW, Dumic M, Auslender R, Gil-da-Silva-Lopes VL, Steinlicht S, Rauh M, Shalev SA, Thiel C, Ekici AB, Winterpacht A, Kwon YT, Varshavsky A, Reis A: Deficiency of UBR1, a ubiquitin ligase of the N-end rule pathway, causes pancreatic dysfunction, malformations and mental retardation (Johanson-Blizzard syndrome). Nat Genet. 2005 Dec;37(12):1345-50. Epub 2005 Nov 20. [PubMed:16311597 ]