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P51111 (HD_RAT) Reviewed, UniProtKB/Swiss-Prot

Last modified June 11, 2014. Version 103. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (1) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Huntingtin
Alternative name(s):
Huntington disease protein homolog
Short name=HD protein homolog
Gene names
Name:Htt
Synonyms:Hd, Hdh
OrganismRattus norvegicus (Rat) [Reference proteome]
Taxonomic identifier10116 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus

Protein attributes

Sequence length3110 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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

Subunit structure

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.

Subcellular location

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

Tissue specificity

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 stage

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.

Domain

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.

Post-translational modification

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.

Polymorphism

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

Sequence similarities

Belongs to the huntingtin family.

Contains 5 HEAT repeats.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 31103110Huntingtin
PRO_0000083944

Regions

Repeat174 – 21138HEAT 1
Repeat216 – 25338HEAT 2
Repeat773 – 81038HEAT 3
Repeat873 – 91139HEAT 4
Repeat1395 – 143238HEAT 5
Motif2363 – 237210Nuclear export signal By similarity
Compositional bias11 – 188Poly-Gln
Compositional bias19 – 3820Poly-Pro
Compositional bias42 – 5110Poly-Pro
Compositional bias1408 – 14114Poly-Thr
Compositional bias2606 – 26116Poly-Glu

Amino acid modifications

Modified residue21N6-acetyllysine By similarity
Modified residue1461N6-acetyllysine By similarity
Modified residue2041N6-acetyllysine By similarity
Modified residue3131N6-acetyllysine By similarity
Modified residue4021Phosphoserine By similarity
Modified residue4121N6-acetyllysine By similarity
Modified residue11501Phosphoserine; by CDK5 By similarity
Modified residue11701Phosphoserine; by CDK5 By similarity
Modified residue18451Phosphoserine By similarity

Sequences

Sequence LengthMass (Da)Tools
P51111 [UniParc].

Last modified October 1, 1996. Version 1.
Checksum: 33C357E8FC141550

FASTA3,110343,762
        10         20         30         40         50         60 
MKAFESLKSF QQQQQQQQPP PQPPPPPPPP PQPPQPPPQG QPPPPPPLPG PAEEPLHRPK 

        70         80         90        100        110        120 
KELSATKKDR VNHCLTICEN IVAQSLRNSP EFQKLLGIAM ELFLLCSDDA SRRRMVADEC 

       130        140        150        160        170        180 
LNKVIKALMD SNLPRLQLEL YKEIKKNGAP RSLRAALWRF AELAHLVRPQ KCRPYLVNLL 

       190        200        210        220        230        240 
PCLTRTSKRP EESVQETLAA AVPKIMASFG NFANDNEIKV LLKAFIANLK SSSPTVRRTA 

       250        260        270        280        290        300 
AGSAVSICQH SRRTQYFYNW LLNVLLGLLV PMEEDHPTLL ILGVLLTLRC LVPLLQQQVK 

       310        320        330        340        350        360 
DTSLKGSFGV TRKEMEVSPS AEQLVQVYEL TLHHTQHQDH NVVTGALELL QQLFRTPPPE 

       370        380        390        400        410        420 
LLQALTTPGG LGQLTLVREE AGGRGRSGSI VELLAGGGSS CSPVLSRKQK GKVLLGEEEA 

       430        440        450        460        470        480 
LEDDSESRSD VSSSAFAASV KSEIGGELAA SSSGVSTPGS VGHDIITEQP RSQHTLQADS 

       490        500        510        520        530        540 
VDLSGCDLTS AATDGDEEDI LSHSSSQFSA VPSDPAMDLN DGTQASSPIS DSSQTTTEGP 

       550        560        570        580        590        600 
DSAVTPSDSS EIVLDGADSQ YLGVQIGQPQ EEDREAAGVL SGEVSDVFRN SSLALQQAHL 

       610        620        630        640        650        660 
LERMGHSRQP SDSSVDKFVS KDEVAEAGDP ESKPCRIKGD IGQPNDDDSA PLVHCVRLLS 

       670        680        690        700        710        720 
ASFLLTGEKK ALVPDRDVRV SVKALALSCI GAAVALHPES FFSKLYKVPL STMESTEEQY 

       730        740        750        760        770        780 
VSDILNYIDH GDPQVRGATA ILCGTLVYSI LSRSRLRVGD WLGTIRALTG NTFSLVDCIP 

       790        800        810        820        830        840 
LLQKTLKDES SVTCKLACTA VRHCVLSLCS SSYSDLGLQL LIDMLPLKNS SYWLVRTELL 

       850        860        870        880        890        900 
ETLAEIDFRL VSFLEAKAES LHRGPHHYTG FLKLQERVLN NVVIYLLGDE DPRVRHVAAT 

       910        920        930        940        950        960 
TLTRLVPKLF YKCDQGQADP VEAVARDQSS VYLKLLMHET QPPSHFSVST ITRIYRGYSL 

       970        980        990       1000       1010       1020 
LPSVTDVTME NNLSRVVAAV SHELITSTTR ALTFGCCEAL CVLSAAFPVC TWSLGWHCGV 

      1030       1040       1050       1060       1070       1080 
PPLSASDESR KSCTVGMASM ILTLLSSAWF PLDLSAIQDA LILAGNLLAA SAPKSLRSSW 

      1090       1100       1110       1120       1130       1140 
ASEEEGSSAA TRQEEIWPAL GDRTLVPMVE QLFSHLLKVI NICAHVLDDE TPGPAIKAAL 

      1150       1160       1170       1180       1190       1200 
PSLTNPPSLS PIRRKGKEKE PGEQTSTPMS PKKGGEASTA SRQSDTSGPV TASKSSSLGS 

      1210       1220       1230       1240       1250       1260 
FYHLPSYLRL HDVLKATHAN YKVTLDLQNS TEKFGGFLRS ALDVLSQILE LATLQDIGKC 

      1270       1280       1290       1300       1310       1320 
VEEVLGYLKS CFSREPMMAT VCVQQLLKTL FGTNLASQFD GLSSNPSKSQ CRAQRLGSSS 

      1330       1340       1350       1360       1370       1380 
VRPGLYHYCF MAPYTHFTQA LADASLRNMV QADQEHDASG WFDVLQKVSA QLKTNLTSVT 

      1390       1400       1410       1420       1430       1440 
KNRADKNAIH NHIRLFEPLV IKALKQYTTT TSVQLQKQVL DLLAQLVQLR VNYCLLDSDQ 

      1450       1460       1470       1480       1490       1500 
VFIGFVLKQF EYIEVGQFRE SEAIIPNIFF FLVLLSYERY HSKQIIGIPK IIQLCDGIMA 

      1510       1520       1530       1540       1550       1560 
SGRKAVTHAI PALQPIVHDL FVLRGTNKAD AGKELETQKE VVVSMLLRLI QYHQVLEMFI 

      1570       1580       1590       1600       1610       1620 
LVLQQCHKEN EDKWKRLSRQ VADIILPMLA KQQMHIDSHE ALGVLNTLFE ILAPSSLRPV 

      1630       1640       1650       1660       1670       1680 
DMLLRSMFIT PSTMASVSTV QLWISGILAI LRVLISQSTE DIVLSRIQEL SFSPYLISCP 

      1690       1700       1710       1720       1730       1740 
VINRLRDGDS NPTLGERSRG KQVKNLPEDT FSRFLLQLVG ILLEDIVTKQ LKVDMSEQQH 

      1750       1760       1770       1780       1790       1800 
TFYCQELGTL LMCLIHIFKS GMFRRITAAA TRLFTSDGCE GSFYTLDSLN ARVRAMVPTH 

      1810       1820       1830       1840       1850       1860 
PALVLLWCQI LLLINHTDHR WWAEVQQTPK RHSLSCTKSL NPQISAEEDS GSAAQLGMCN 

      1870       1880       1890       1900       1910       1920 
REIVRRGALI LFCDYVCQNL HDSEHLTWLI VNHIQDLISL SHEPPVQDFI SAIHRNSAAS 

      1930       1940       1950       1960       1970       1980 
GLFIQAIQSR CENLSTPTTL KKTLQCLEGI HLSQSGAVLT LYVDRLLGTP FRALARMVDT 

      1990       2000       2010       2020       2030       2040 
LACRRVEMLL AANLQSSMAQ LPEEELNRIQ EHLQNTGLAQ RHQRLYSLLD RFRLSTVQDS 

      2050       2060       2070       2080       2090       2100 
LSPLPPVTSH PLDGDGHTSL ETVNPDKDWY LQLVRSQCWT RSDSALLEGA ELVNRIPAED 

      2110       2120       2130       2140       2150       2160 
MSDFMMSSEF NLSLFAPCLS LGMSEIAGSQ KSPLFEAARR VTLDRVTNVV QQLPAVHQVF 

      2170       2180       2190       2200       2210       2220 
QPFLPTEPTA YWSKLNDLFG DTTSYQSLTT LARALAQYLV VLSKVPAPLH LPPEKEGHTV 

      2230       2240       2250       2260       2270       2280 
KFVVMTLEAL SWHLIHEQIP LSLDLQAGLD CCCLALQVPG LWGVLSSPEY VTHTCSLIHC 

      2290       2300       2310       2320       2330       2340 
VRFILEAIAV QPGDQLLGPE SRSHTPRAVR KEEVDSDIQN LSHITSACEM VADMVESLQS 

      2350       2360       2370       2380       2390       2400 
VLALGHKRNS TLPSFLTAVL KNIVVSLARL PLVNSYTRVP PLVWKLGWSP KPGGDFGTVF 

      2410       2420       2430       2440       2450       2460 
PEIPVEFLQE KEVLKEFIYR INTLGWTSRT QFEETWATLL GVLVTQPLVM EQEESPPEED 

      2470       2480       2490       2500       2510       2520 
TERTQIHVLA VQAITSLVLS AMAVPVAGNP AVSCLEQQPR NKPLKALDTR FGRKLSMIRG 

      2530       2540       2550       2560       2570       2580 
IVEQEIQEMV SQRENTATHH SHQAWDPVPS LLPATTGALI SHDKLLLQIN SEREPGNMSY 

      2590       2600       2610       2620       2630       2640 
KLGQVSIHSV WLGNNITPLR EEEWDEEEEE EADAPAPTSP PVSPVNSRKH RAGVDIHSCS 

      2650       2660       2670       2680       2690       2700 
QFLLELYSRW ILPSSAARRT PVILISEVVR SLLVVSDLFT DVPQFEMMYL TLTELRRVHP 

      2710       2720       2730       2740       2750       2760 
SEDEILIQYL VPATCKAAAV LGMDKTVAEP VSRLLESTLR STHLPSQIGA LHGILYVLEC 

      2770       2780       2790       2800       2810       2820 
DLLDDTVKQL IPVVSDYLLS NLKGIAHCVN IHSQQHVLVM CATAFYLMEN YPLDVGPEFS 

      2830       2840       2850       2860       2870       2880 
ASVIQMCGVM LSGSEESTPS VIYHCALRGL ERLLLSEQLS RLDTESLVKL SVDRVNVQSP 

      2890       2900       2910       2920       2930       2940 
HRAMAALGLM LTCMYTGKEK ASPGRASDPS PATPDSESVI VAMERVSVLF DRIRKGFPCE 

      2950       2960       2970       2980       2990       3000 
ARVVARILPQ FLDDFFPPQD VMNKVIGEFL SNQQPYPQFM ATVVYKVFQT LHSAGQSSMV 

      3010       3020       3030       3040       3050       3060 
RDWVMLSLSN FTQRTPVAMA MWSLSCFLVS ASTSPWVSAI LPHVISRMGK LEQVDVNLFC 

      3070       3080       3090       3100       3110 
LVATDFYRHQ IEEEFDRRAF QSVFEVVAAP GSPYHRLLAC LQNVHKVTAC 

« Hide

References

[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.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U18650 mRNA. Translation: AAA90987.1. Sequence problems.
U01022 mRNA. Translation: AAC52133.1.
PIRS40522.
UniGeneRn.11193.

3D structure databases

ProteinModelPortalP51111.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

MINTMINT-3375769.
STRING10116.ENSRNOP00000054971.

Chemistry

ChEMBLCHEMBL2439943.

PTM databases

PhosphoSiteP51111.

Proteomic databases

PaxDbP51111.
PRIDEP51111.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Organism-specific databases

RGD68337. Htt.

Phylogenomic databases

eggNOGNOG82191.
HOGENOMHOG000082472.
HOVERGENHBG005953.
InParanoidP51111.
PhylomeDBP51111.

Gene expression databases

GenevestigatorP51111.

Family and domain databases

Gene3D1.25.10.10. 4 hits.
InterProIPR011989. ARM-like.
IPR016024. ARM-type_fold.
IPR000091. Huntingtin.
IPR028426. Huntingtin_fam.
IPR024613. Huntingtin_middle-repeat.
[Graphical view]
PANTHERPTHR10170. PTHR10170. 1 hit.
PfamPF12372. DUF3652. 2 hits.
[Graphical view]
PRINTSPR00375. HUNTINGTIN.
SUPFAMSSF48371. SSF48371. 6 hits.
ProtoNetSearch...

Other

PROP51111.

Entry information

Entry nameHD_RAT
AccessionPrimary (citable) accession number: P51111
Entry history
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: June 11, 2014
This is version 103 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families