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Protein Page:
Huntingtin (human)
p Phosphorylation
a Acetylation
m Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
u Ubiquitination
s Sumoylation
n Neddylation
gl O-GlcNAc
ga O-GalNAc
h Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage

Overview
Huntingtin may play a role in microtubule-mediated transport or vesicle function. Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation. Defects are the cause of Huntington's disease, a neurodegenerative disorder characterized by loss of striatal neurons. This is thought to be caused by an expanded, unstable trinucleotide repeat in the Huntingtin gene, which translates as a polyglutamine repeat in the protein product. The Huntingtin locus is large, spanning 180 kb and consisting of 67 exons. The Huntingtin gene is widely expressed and is required for normal development. It is expressed as 2 alternatively polyadenylated isoforms displaying different relative abundance in various fetal and adult tissues. Note: This description may include information from UniProtKB.
Protein type: Cytoskeletal protein
Cellular Component: Golgi apparatus; protein complex; cytoplasmic vesicle membrane; mitochondrion; endoplasmic reticulum; dendrite; autophagic vacuole; inclusion body; cytosol; axon; late endosome; cytoplasm; nucleus
Molecular Function: identical protein binding; protein binding; p53 binding; dynein intermediate chain binding; beta-tubulin binding; diazepam binding; transcription factor binding
Biological Process: ER to Golgi vesicle-mediated transport; citrulline metabolic process; paraxial mesoderm formation; regulation of protein phosphatase type 2A activity; regulation of synaptic plasticity; locomotory behavior; determination of adult life span; endosome transport; regulation of protein export from nucleus; anterior/posterior pattern formation; L-glutamate import; regulation of mitochondrial membrane potential; establishment of mitotic spindle orientation; protein import into nucleus; organ development; quinolinate biosynthetic process; vesicle transport along microtubule; retrograde vesicle-mediated transport, Golgi to ER; visual learning; negative regulation of neuron apoptosis; Golgi organization and biogenesis; endoplasmic reticulum organization and biogenesis; grooming behavior; striatum development; axon cargo transport; cell aging; olfactory lobe development; social behavior; lactate biosynthetic process from pyruvate; iron ion homeostasis; neuron apoptosis; insulin secretion; dopamine receptor signaling pathway; neuron development; hormone metabolic process; spermatogenesis; regulation of mitochondrial membrane permeability; response to calcium ion; neural plate formation; urea cycle
Reference #:  P42858 (UniProtKB)
Alt. Names/Synonyms: HD; HD protein; HTT; Huntingtin; Huntington disease protein; IT15
Gene Symbols: HTT
Molecular weight: 347,603 Da
Basal Isoelectric point: 5.81  Predict pI for various phosphorylation states
CST Pathways:  PI3K/Akt Signaling
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
Select Structure to View Below

Huntingtin

Protein Structure Not Found.


STRING  |  Scansite  |  Phospho.ELM  |  NetworKIN  |  Pfam  |  RCSB PDB  |  Phospho3D  |  DISEASE  |  Source  |  InnateDB  |  UCSD-Nature  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene


Sites Implicated In
apoptosis, inhibited: S419‑p
cytoskeletal reorganization: S419‑p
activity, induced: S419‑p
intracellular localization: S13‑p, S16‑p
molecular association, regulation: T3‑p, S13‑p, S16‑p, S419‑p
protein degradation: S429‑p
protein stabilization: S430‑p, S534‑p

Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
2 1 T3-p _____MAtLEkLMkA
0 1 K6-a __MAtLEkLMkAFEs
1 0 K6-s __MAtLEkLMkAFEs
2 0 K9-a AtLEkLMkAFEsLks
1 0 K9-s AtLEkLMkAFEsLks
6 1 S13-p kLMkAFEsLksFQQQ
1 0 K15-s MkAFEsLksFQQQQQ
7 0 S16-p kAFEsLksFQQQQQQ
1 1 S114-p CENIVAQsVRNsPEF
1 0 S118-p VAQsVRNsPEFQKLL
1 0 K176-a ELYKEIKkNGAPRSL
1 0 K234-a TLAAAVPkIMASFGN
0 1 T265 NLKSSSPTIRRtAAG
1 0 T269-p SSPTIRRtAAGSAVS
1 0 K343-a GSFGVTRkEMEVSPS
0 1 T405-p VGGIGQLtAAKEEsG
0 1 S411-p LtAAKEEsGGRsRsG
1 4 S415-p KEEsGGRsRsGsIVE
0 14 S417-p EsGGRsRsGsIVELI
8 27 S419-p GGRsRsGsIVELIAG
2 2 S429-p ELIAGGGssCsPVLs
1 6 S430-p LIAGGGssCsPVLsR
2 63 S432-p AGGGssCsPVLsRKQ
0 4 S436-p ssCsPVLsRKQKGkV
2 0 K442-a LsRKQKGkVLLGEEE
1 1 K442-u LsRKQKGkVLLGEEE
1 0 S455-p EEALEDDsEsRsDVs
1 0 S457-p ALEDDsEsRsDVsss
1 0 S459-p EDDsEsRsDVsssAL
1 0 S462-p sEsRsDVsssALTAS
1 0 S463-p EsRsDVsssALTASV
1 0 S464-p sRsDVsssALTASVK
1 0 S485-p LAASSGVstPGsAGH
1 1 T486-p AASSGVstPGsAGHD
1 0 S489-p SGVstPGsAGHDIIT
0 1 S534-p EDILSHSsSQVSAVP
0 13 S640-p MSHCRQPsDSsVDKF
0 3 S643-p CRQPsDSsVDKFVLR
0 2 G657 RDEATEPGDQENKPC
0 1 K813 DCIPLLRKTLKDESS
0 1 K941 LVPKLFYKCDQGQAD
0 1 S1177-p PSLTNPPsLsPIRRK
2 15 S1179-p LTNPPsLsPIRRKGK
2 10 S1199-p EQASVPLsPKKGSEA
0 1 K1238 YHLPSYLKLHDVLKA
0 2 K1262-u DLQNSTEkFGGFLRS
0 1 K1337 GLSSNPSKSQGRAQR
0 1 K1402-u QKVSTQLkTNLTSVT
0 1 K1410 TNLTSVTKNRADKNA
0 1 K1431 LFEPLVIKALKQYTT
0 1 K1730 LEEHSEGKQIKNLPE
0 1 T1857 WWAEVQQTPKRHsLs
0 3 S1862-p QQTPKRHsLsSTKLL
0 1 S1864-p TPKRHsLsSTKLLsP
0 3 S1870-p LsSTKLLsPQMsGEE
0 16 S1874-p KLLsPQMsGEEEDSD
0 1 S2074-p MQDSLSPsPPVSSHP
0 1 S2233-p AQYLVVVsKLPSHLH
0 1 K2417-u RVPPLVWkLGWSPKP
0 2 S2651-p ADAPAPSsPPTsPVN
0 3 S2655-p APSsPPTsPVNSRKH
0 1 S2764-p KAVAEPVsRLLESTL
0 1 S2888 GLERLLLSEQLsRLD
0 1 S2892-p LLLSEQLsRLDAESL
0 1 A2896 EQLsRLDAESLVKLS
0 1 S2898 LsRLDAESLVKLSVD
0 1 A2945 TSDPNPAAPDsEsVI
0 2 S2948-p PNPAAPDsEsVIVAM
0 2 S2950-p PAAPDsEsVIVAMER
0 1 S3039 VRDWVMLSLSNFTQR
0 1 S3041 DWVMLSLSNFTQRAP
0 1 T3044 MLSLSNFTQRAPVAM
0 1 T3065 CFFVSASTSPWVAAI
0 1 S3066 FFVSASTSPWVAAIL
0 1 A3070 ASTSPWVAAILPHVI
0 1 S3078 AILPHVISRMGKLEQ
  mouse

 
T3-p _____MAtLEKLMkA
K6 __MAtLEKLMkAFEs
K6 __MAtLEKLMkAFEs
K9-a AtLEKLMkAFEsLKs
K9 AtLEKLMKAFEsLKs
S13-p KLMkAFEsLKsFQQQ
K15 MkAFEsLKsFQQQQQ
S16-p kAFEsLKsFQQQQQQ
S93 CENIVAQSLRNSPEF
S97 VAQSLRNSPEFQKLL
K155 ELYKEIKKNGAPRSL
K213 TLAAAVPKIMASFGN
T244-p NLKSSSPtVRRTAAG
T248 SSPtVRRTAAGSAVS
K322 GSFGVTRKEMEVSPS
T384 PGGLGQLTLVQEEAR
A390 LTLVQEEARGRGRsG
G394 QEEARGRGRsGsIVE
S396-p EARGRGRsGsIVELL
S398-p RGRGRsGsIVELLAG
S408-p ELLAGGGssCsPVLs
S409-p LLAGGGssCsPVLsR
S411-p AGGGssCsPVLsRKQ
S415-p ssCsPVLsRKQKGKV
K421 LsRKQKGKVLLGEEE
K421 LsRKQKGKVLLGEEE
S434 EEALEDDSESRSDVS
S436 ALEDDSESRSDVSSS
S438 EDDSESRSDVSSSAF
S441 SESRSDVSSSAFAAS
S442 ESRSDVSSSAFAASV
S443 SRSDVSSSAFAASVK
S464 LAASSGVSTPGSVGH
T465 AASSGVSTPGSVGHD
S468 SGVSTPGSVGHDIIT
S513 EDILSHSSSQFSAVP
S620-p MGHSRQPsDSsIDKY
S623-p SRQPsDSsIDKYVTR
S637-p RDEVAEAsDPESKPC
K793-u DCIPLLQkTLKDESS
K921-u LVPKLFYkCDQGQAD
S1157 PSLTNPPSLsPIRRK
S1159-p LTNPPSLsPIRRKGK
S1179-p EQASTPMsPKKVGEA
K1218-u YHLPSYLkLHDVLKA
K1242-u DLQNSTEkFGGFLRS
K1317-u GLSSNPSkSQCRAQR
K1382 QKVSAQLKTNLTSVT
K1390-u TNLTSVTkNRADKNA
K1411-u LFEPLVIkALKQYTT
K1710-u LGECSEGkQKSLPED
T1836-p WWAEVQQtPKRHsLs
S1841-p QQtPKRHsLsCTKSL
S1843-p tPKRHsLsCTKSLNP
N1849 LsCTKSLNPQKsGEE
S1853-p KSLNPQKsGEEEDSG
L2053 VQDSLSPLPPVTSHP
S2212 AQYLVVLSKVPAHLH
K2394 RVPPLVWKLGWSPKP
S2628-p SDVPAPTsPPVsPVN
S2632-p APTsPPVsPVNSRKH
S2741 KTVAEPVSRLLESTL
S2865-p GLERLLLsEQLSRLD
S2869 LLLsEQLSRLDtEsL
T2873-p EQLSRLDtEsLVKLS
S2875-p LSRLDtEsLVKLSVD
T2922-p ASDPSPAtPDSESVI
S2925 PSPAtPDSESVIVAM
S2927 PAtPDSESVIVAMER
S3016-p VRDWVMLsLsNFtQR
S3018-p DWVMLsLsNFtQRTP
T3021-p MLsLsNFtQRTPVAM
T3042-p CFLVSAStsPWVsAI
S3043-p FLVSAStsPWVsAIL
S3047-p AStsPWVsAILPHVI
S3055-p AILPHVIsRMGKLEQ
  rat

 
- gap
- gap
- gap
- gap
K2 ______MKAFESLKS
S6 __MKAFESLKSFQQQ
K8 MKAFESLKSFQQQQQ
S9 KAFESLKSFQQQQQQ
S85 CENIVAQSLRNSPEF
S89 VAQSLRNSPEFQKLL
K146 ELYKEIKKNGAPRSL
K204 TLAAAVPKIMASFGN
T235 NLKSSSPTVRRTAAG
T239 SSPTVRRTAAGSAVS
K313 GSFGVTRKEMEVSPS
T375 PGGLGQLTLVREEAG
A381 LTLVREEAGGRGRsG
G385 REEAGGRGRsGsIVE
S387-p EAGGRGRsGsIVELL
S389-p GGRGRsGsIVELLAG
S399 ELLAGGGSSCsPVLS
S400 LLAGGGSSCsPVLSR
S402-p AGGGSSCsPVLSRKQ
S406 SSCsPVLSRKQKGKV
K412 LSRKQKGKVLLGEEE
K412 LSRKQKGKVLLGEEE
S425 EEALEDDSESRSDVS
S427 ALEDDSESRSDVSSS
S429 EDDSESRSDVSSSAF
S432 SESRSDVSSSAFAAS
S433 ESRSDVSSSAFAASV
S434 SRSDVSSSAFAASVK
S456 AASSSGVSTPGSVGH
T457 ASSSGVSTPGSVGHD
S460 SGVSTPGSVGHDIIT
S505 EDILSHSSSQFSAVP
S611 MGHSRQPSDSSVDKF
S614 SRQPSDSSVDKFVSK
G628 KDEVAEAGDPESKPC
K784 DCIPLLQKTLKDESS
K912 LVPKLFYKCDQGQAD
S1148 PSLTNPPSLsPIRRK
S1150-p LTNPPSLsPIRRKGK
S1170-p EQTSTPMsPKKGGEA
R1209 YHLPSYLRLHDVLKA
K1233 DLQNSTEKFGGFLRS
K1308 GLSSNPSKSQCRAQR
K1373 QKVSAQLKTNLTSVT
K1381 TNLTSVTKNRADKNA
K1402 LFEPLVIKALKQYTT
K1701 LGERSRGKQVKNLPE
T1828 WWAEVQQTPKRHSLS
S1833 QQTPKRHSLSCTKSL
S1835 TPKRHSLSCTKSLNP
N1841 LSCTKSLNPQISAEE
S1845 KSLNPQISAEEDSGS
L2044 VQDSLSPLPPVTSHP
S2203 AQYLVVLSKVPAPLH
K2385 RVPPLVWKLGWSPKP
S2619 ADAPAPTSPPVSPVN
S2623 APTSPPVSPVNSRKH
S2732 KTVAEPVSRLLESTL
S2856 GLERLLLSEQLSRLD
S2860 LLLSEQLSRLDTESL
T2864 EQLSRLDTESLVKLS
S2866 LSRLDTESLVKLSVD
T2913 ASDPSPATPDSESVI
S2916 PSPATPDSESVIVAM
S2918 PATPDSESVIVAMER
S3007 VRDWVMLSLSNFTQR
S3009 DWVMLSLSNFTQRTP
T3012 MLSLSNFTQRTPVAM
T3033 CFLVSASTSPWVSAI
S3034 FLVSASTSPWVSAIL
S3038 ASTSPWVSAILPHVI
S3046 AILPHVISRMGKLEQ
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