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P51111

- HD_RAT

UniProt

P51111 - HD_RAT

Protein

Huntingtin

Gene

Htt

Organism
Rattus norvegicus (Rat)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 104 (01 Oct 2014)
      Sequence version 1 (01 Oct 1996)
      Previous versions | rss
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    Functioni

    May play a role in microtubule-mediated transport or vesicle function.

    GO - Molecular functioni

    1. protein binding Source: RGD
    2. receptor binding Source: RGD
    3. transcription factor binding Source: RefGenome

    GO - Biological processi

    1. central nervous system development Source: RGD
    2. mRNA transport Source: RGD
    3. negative regulation of cysteine-type endopeptidase activity Source: RGD
    4. organ development Source: RefGenome

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Huntingtin
    Alternative name(s):
    Huntington disease protein homolog
    Short name:
    HD protein homolog
    Gene namesi
    Name:Htt
    Synonyms:Hd, Hdh
    OrganismiRattus norvegicus (Rat)
    Taxonomic identifieri10116 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
    ProteomesiUP000002494: Unplaced

    Organism-specific databases

    RGDi68337. Htt.

    Subcellular locationi

    Cytoplasm By similarity. Nucleus By similarity
    Note: Shuttles between cytoplasm and nucleus in a Ran GTPase-independent manner.

    GO - Cellular componenti

    1. axon Source: RGD
    2. clathrin-coated vesicle Source: RGD
    3. cytoplasm Source: RefGenome
    4. cytosol Source: RGD
    5. dendrite Source: RGD
    6. neuronal cell body Source: RGD
    7. nucleus Source: RefGenome
    8. postsynaptic density Source: RGD

    Keywords - Cellular componenti

    Cytoplasm, Nucleus

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 31103110HuntingtinPRO_0000083944Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei2 – 21N6-acetyllysineBy similarity
    Modified residuei146 – 1461N6-acetyllysineBy similarity
    Modified residuei204 – 2041N6-acetyllysineBy similarity
    Modified residuei313 – 3131N6-acetyllysineBy similarity
    Modified residuei402 – 4021PhosphoserineBy similarity
    Modified residuei412 – 4121N6-acetyllysineBy similarity
    Modified residuei1150 – 11501Phosphoserine; by CDK5By similarity
    Modified residuei1170 – 11701Phosphoserine; by CDK5By similarity
    Modified residuei1845 – 18451PhosphoserineBy similarity

    Post-translational modificationi

    Phosphorylation at Ser-1150 and Ser-1170 by CDK5 in response to DNA damage in nuclei of neurons protects neurons against polyglutamine expansion as well as DNA damage mediated toxicity.

    Keywords - PTMi

    Acetylation, Phosphoprotein

    Proteomic databases

    PaxDbiP51111.
    PRIDEiP51111.

    PTM databases

    PhosphoSiteiP51111.

    Expressioni

    Tissue specificityi

    Expressed to a high degree in all the regions of the brain of adults and in meiotic cells of the testis. In addition, very low levels are detected in various non-neuronal tissues (heart, muscle, liver, lung and kidney).

    Developmental stagei

    Identified at high levels in neuronal tissues of embryos as early as day 14.5. This expression remains constant in all further development stages (up to the adult). On the other hand the expression in non-neuronal tissues is down-regulated from stage 17.5 day old embryos.

    Gene expression databases

    GenevestigatoriP51111.

    Interactioni

    Subunit structurei

    Interacts with PQBP1 and SETD2. Binds SH3GLB1 By similarity. Interacts with PFN1. Interacts with TPR; the interaction is inhibited by forms of Huntingtin with expanded polyglutamine stretch By similarity.By similarity

    Protein-protein interaction databases

    MINTiMINT-3375769.
    STRINGi10116.ENSRNOP00000054971.

    Structurei

    3D structure databases

    ProteinModelPortaliP51111.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Repeati174 – 21138HEAT 1Add
    BLAST
    Repeati216 – 25338HEAT 2Add
    BLAST
    Repeati773 – 81038HEAT 3Add
    BLAST
    Repeati873 – 91139HEAT 4Add
    BLAST
    Repeati1395 – 143238HEAT 5Add
    BLAST

    Motif

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Motifi2363 – 237210Nuclear export signalBy similarity

    Compositional bias

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Compositional biasi11 – 188Poly-Gln
    Compositional biasi19 – 3820Poly-ProAdd
    BLAST
    Compositional biasi42 – 5110Poly-Pro
    Compositional biasi1408 – 14114Poly-Thr
    Compositional biasi2606 – 26116Poly-Glu

    Domaini

    The N-terminal Gln-rich and Pro-rich domain has great conformational flexibility and is likely to exist in a fluctuating equilibrium of alpha-helical, random coil, and extended conformations.By similarity

    Sequence similaritiesi

    Belongs to the huntingtin family.Curated
    Contains 5 HEAT repeats.Curated

    Keywords - Domaini

    Repeat

    Phylogenomic databases

    eggNOGiNOG82191.
    HOGENOMiHOG000082472.
    HOVERGENiHBG005953.
    InParanoidiP51111.
    PhylomeDBiP51111.

    Family and domain databases

    Gene3Di1.25.10.10. 4 hits.
    InterProiIPR011989. ARM-like.
    IPR016024. ARM-type_fold.
    IPR000091. Huntingtin.
    IPR028426. Huntingtin_fam.
    IPR024613. Huntingtin_middle-repeat.
    [Graphical view]
    PANTHERiPTHR10170. PTHR10170. 1 hit.
    PfamiPF12372. DUF3652. 2 hits.
    [Graphical view]
    PRINTSiPR00375. HUNTINGTIN.
    SUPFAMiSSF48371. SSF48371. 6 hits.

    Sequencei

    Sequence statusi: Complete.

    P51111-1 [UniParc]FASTAAdd to Basket

    « Hide

    MKAFESLKSF QQQQQQQQPP PQPPPPPPPP PQPPQPPPQG QPPPPPPLPG     50
    PAEEPLHRPK KELSATKKDR VNHCLTICEN IVAQSLRNSP EFQKLLGIAM 100
    ELFLLCSDDA SRRRMVADEC LNKVIKALMD SNLPRLQLEL YKEIKKNGAP 150
    RSLRAALWRF AELAHLVRPQ KCRPYLVNLL PCLTRTSKRP EESVQETLAA 200
    AVPKIMASFG NFANDNEIKV LLKAFIANLK SSSPTVRRTA AGSAVSICQH 250
    SRRTQYFYNW LLNVLLGLLV PMEEDHPTLL ILGVLLTLRC LVPLLQQQVK 300
    DTSLKGSFGV TRKEMEVSPS AEQLVQVYEL TLHHTQHQDH NVVTGALELL 350
    QQLFRTPPPE LLQALTTPGG LGQLTLVREE AGGRGRSGSI VELLAGGGSS 400
    CSPVLSRKQK GKVLLGEEEA LEDDSESRSD VSSSAFAASV KSEIGGELAA 450
    SSSGVSTPGS VGHDIITEQP RSQHTLQADS VDLSGCDLTS AATDGDEEDI 500
    LSHSSSQFSA VPSDPAMDLN DGTQASSPIS DSSQTTTEGP DSAVTPSDSS 550
    EIVLDGADSQ YLGVQIGQPQ EEDREAAGVL SGEVSDVFRN SSLALQQAHL 600
    LERMGHSRQP SDSSVDKFVS KDEVAEAGDP ESKPCRIKGD IGQPNDDDSA 650
    PLVHCVRLLS ASFLLTGEKK ALVPDRDVRV SVKALALSCI GAAVALHPES 700
    FFSKLYKVPL STMESTEEQY VSDILNYIDH GDPQVRGATA ILCGTLVYSI 750
    LSRSRLRVGD WLGTIRALTG NTFSLVDCIP LLQKTLKDES SVTCKLACTA 800
    VRHCVLSLCS SSYSDLGLQL LIDMLPLKNS SYWLVRTELL ETLAEIDFRL 850
    VSFLEAKAES LHRGPHHYTG FLKLQERVLN NVVIYLLGDE DPRVRHVAAT 900
    TLTRLVPKLF YKCDQGQADP VEAVARDQSS VYLKLLMHET QPPSHFSVST 950
    ITRIYRGYSL LPSVTDVTME NNLSRVVAAV SHELITSTTR ALTFGCCEAL 1000
    CVLSAAFPVC TWSLGWHCGV PPLSASDESR KSCTVGMASM ILTLLSSAWF 1050
    PLDLSAIQDA LILAGNLLAA SAPKSLRSSW ASEEEGSSAA TRQEEIWPAL 1100
    GDRTLVPMVE QLFSHLLKVI NICAHVLDDE TPGPAIKAAL PSLTNPPSLS 1150
    PIRRKGKEKE PGEQTSTPMS PKKGGEASTA SRQSDTSGPV TASKSSSLGS 1200
    FYHLPSYLRL HDVLKATHAN YKVTLDLQNS TEKFGGFLRS ALDVLSQILE 1250
    LATLQDIGKC VEEVLGYLKS CFSREPMMAT VCVQQLLKTL FGTNLASQFD 1300
    GLSSNPSKSQ CRAQRLGSSS VRPGLYHYCF MAPYTHFTQA LADASLRNMV 1350
    QADQEHDASG WFDVLQKVSA QLKTNLTSVT KNRADKNAIH NHIRLFEPLV 1400
    IKALKQYTTT TSVQLQKQVL DLLAQLVQLR VNYCLLDSDQ VFIGFVLKQF 1450
    EYIEVGQFRE SEAIIPNIFF FLVLLSYERY HSKQIIGIPK IIQLCDGIMA 1500
    SGRKAVTHAI PALQPIVHDL FVLRGTNKAD AGKELETQKE VVVSMLLRLI 1550
    QYHQVLEMFI LVLQQCHKEN EDKWKRLSRQ VADIILPMLA KQQMHIDSHE 1600
    ALGVLNTLFE ILAPSSLRPV DMLLRSMFIT PSTMASVSTV QLWISGILAI 1650
    LRVLISQSTE DIVLSRIQEL SFSPYLISCP VINRLRDGDS NPTLGERSRG 1700
    KQVKNLPEDT FSRFLLQLVG ILLEDIVTKQ LKVDMSEQQH TFYCQELGTL 1750
    LMCLIHIFKS GMFRRITAAA TRLFTSDGCE GSFYTLDSLN ARVRAMVPTH 1800
    PALVLLWCQI LLLINHTDHR WWAEVQQTPK RHSLSCTKSL NPQISAEEDS 1850
    GSAAQLGMCN REIVRRGALI LFCDYVCQNL HDSEHLTWLI VNHIQDLISL 1900
    SHEPPVQDFI SAIHRNSAAS GLFIQAIQSR CENLSTPTTL KKTLQCLEGI 1950
    HLSQSGAVLT LYVDRLLGTP FRALARMVDT LACRRVEMLL AANLQSSMAQ 2000
    LPEEELNRIQ EHLQNTGLAQ RHQRLYSLLD RFRLSTVQDS LSPLPPVTSH 2050
    PLDGDGHTSL ETVNPDKDWY LQLVRSQCWT RSDSALLEGA ELVNRIPAED 2100
    MSDFMMSSEF NLSLFAPCLS LGMSEIAGSQ KSPLFEAARR VTLDRVTNVV 2150
    QQLPAVHQVF QPFLPTEPTA YWSKLNDLFG DTTSYQSLTT LARALAQYLV 2200
    VLSKVPAPLH LPPEKEGHTV KFVVMTLEAL SWHLIHEQIP LSLDLQAGLD 2250
    CCCLALQVPG LWGVLSSPEY VTHTCSLIHC VRFILEAIAV QPGDQLLGPE 2300
    SRSHTPRAVR KEEVDSDIQN LSHITSACEM VADMVESLQS VLALGHKRNS 2350
    TLPSFLTAVL KNIVVSLARL PLVNSYTRVP PLVWKLGWSP KPGGDFGTVF 2400
    PEIPVEFLQE KEVLKEFIYR INTLGWTSRT QFEETWATLL GVLVTQPLVM 2450
    EQEESPPEED TERTQIHVLA VQAITSLVLS AMAVPVAGNP AVSCLEQQPR 2500
    NKPLKALDTR FGRKLSMIRG IVEQEIQEMV SQRENTATHH SHQAWDPVPS 2550
    LLPATTGALI SHDKLLLQIN SEREPGNMSY KLGQVSIHSV WLGNNITPLR 2600
    EEEWDEEEEE EADAPAPTSP PVSPVNSRKH RAGVDIHSCS QFLLELYSRW 2650
    ILPSSAARRT PVILISEVVR SLLVVSDLFT DVPQFEMMYL TLTELRRVHP 2700
    SEDEILIQYL VPATCKAAAV LGMDKTVAEP VSRLLESTLR STHLPSQIGA 2750
    LHGILYVLEC DLLDDTVKQL IPVVSDYLLS NLKGIAHCVN IHSQQHVLVM 2800
    CATAFYLMEN YPLDVGPEFS ASVIQMCGVM LSGSEESTPS VIYHCALRGL 2850
    ERLLLSEQLS RLDTESLVKL SVDRVNVQSP HRAMAALGLM LTCMYTGKEK 2900
    ASPGRASDPS PATPDSESVI VAMERVSVLF DRIRKGFPCE ARVVARILPQ 2950
    FLDDFFPPQD VMNKVIGEFL SNQQPYPQFM ATVVYKVFQT LHSAGQSSMV 3000
    RDWVMLSLSN FTQRTPVAMA MWSLSCFLVS ASTSPWVSAI LPHVISRMGK 3050
    LEQVDVNLFC LVATDFYRHQ IEEEFDRRAF QSVFEVVAAP GSPYHRLLAC 3100
    LQNVHKVTAC 3110
    Length:3,110
    Mass (Da):343,762
    Last modified:October 1, 1996 - v1
    Checksum:i33C357E8FC141550
    GO

    Polymorphismi

    The poly-Gln region does not appear to be polymorphic, explaining the absence of a rodent HD-like disorder.

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    U18650 mRNA. Translation: AAA90987.1. Sequence problems.
    U01022 mRNA. Translation: AAC52133.1.
    PIRiS40522.
    UniGeneiRn.11193.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    U18650 mRNA. Translation: AAA90987.1 . Sequence problems.
    U01022 mRNA. Translation: AAC52133.1 .
    PIRi S40522.
    UniGenei Rn.11193.

    3D structure databases

    ProteinModelPortali P51111.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    MINTi MINT-3375769.
    STRINGi 10116.ENSRNOP00000054971.

    Chemistry

    ChEMBLi CHEMBL2439943.

    PTM databases

    PhosphoSitei P51111.

    Proteomic databases

    PaxDbi P51111.
    PRIDEi P51111.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Organism-specific databases

    RGDi 68337. Htt.

    Phylogenomic databases

    eggNOGi NOG82191.
    HOGENOMi HOG000082472.
    HOVERGENi HBG005953.
    InParanoidi P51111.
    PhylomeDBi P51111.

    Miscellaneous databases

    PROi P51111.

    Gene expression databases

    Genevestigatori P51111.

    Family and domain databases

    Gene3Di 1.25.10.10. 4 hits.
    InterProi IPR011989. ARM-like.
    IPR016024. ARM-type_fold.
    IPR000091. Huntingtin.
    IPR028426. Huntingtin_fam.
    IPR024613. Huntingtin_middle-repeat.
    [Graphical view ]
    PANTHERi PTHR10170. PTHR10170. 1 hit.
    Pfami PF12372. DUF3652. 2 hits.
    [Graphical view ]
    PRINTSi PR00375. HUNTINGTIN.
    SUPFAMi SSF48371. SSF48371. 6 hits.
    ProtoNeti Search...

    Publicationsi

    1. "Expression of the Huntington disease gene in rodents: cloning the rat homologue and evidence for downregulation in non-neuronal tissues during development."
      Schmitt I., Baechner D., Megow D., Henklein P., Boulter J., Hameister H., Epplen J.T., Riess O.
      Hum. Mol. Genet. 4:1173-1182(1995) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
      Tissue: Brain.
    2. Lubec G., Kang S.U., Lubec S.
      Submitted (SEP-2007) to UniProtKB
      Cited for: PROTEIN SEQUENCE OF 356-384; 429-441; 767-795; 1760-1772; 1832-1838 AND 2875-2898, IDENTIFICATION BY MASS SPECTROMETRY.
      Strain: Sprague-Dawley.
      Tissue: Brain.
    3. "Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues."
      Strong T.V., Tagle D.A., Valdes J.M., Elmer L.W., Boehm K., Swaroop M., Kaatz K.W., Collins F.S., Albin R.L.
      Nat. Genet. 5:259-265(1993) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 1773-1926.
      Tissue: Brain.

    Entry informationi

    Entry nameiHD_RAT
    AccessioniPrimary (citable) accession number: P51111
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: October 1, 1996
    Last sequence update: October 1, 1996
    Last modified: October 1, 2014
    This is version 104 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3