Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Ataxin-1-like

Gene

Atxn1l

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression (By similarity). Can suppress the cytotoxicity of ATXN1 in spinocerebellar ataxia type 1 (SCA1).By similarity1 Publication

GO - Molecular functioni

GO - Biological processi

  • extracellular matrix organization Source: MGI
  • lung alveolus development Source: MGI
  • negative regulation of transcription from RNA polymerase II promoter Source: MGI
  • positive regulation of hematopoietic stem cell proliferation Source: MGI
  • transcription, DNA-templated Source: UniProtKB-KW
Complete GO annotation...

Keywords - Molecular functioni

Repressor

Keywords - Biological processi

Transcription, Transcription regulation

Keywords - Ligandi

DNA-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Ataxin-1-like
Alternative name(s):
Brother of ataxin-1
Gene namesi
Name:Atxn1l
Synonyms:Boat
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 8

Organism-specific databases

MGIiMGI:3694797. Atxn1l.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cell projection, Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 687687Ataxin-1-likePRO_0000343710Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei282 – 2821PhosphoserineCombined sources
Modified residuei328 – 3281PhosphothreonineBy similarity
Modified residuei359 – 3591PhosphoserineCombined sources

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP0C7T6.
MaxQBiP0C7T6.
PaxDbiP0C7T6.
PeptideAtlasiP0C7T6.
PRIDEiP0C7T6.

PTM databases

iPTMnetiP0C7T6.
PhosphoSiteiP0C7T6.

Expressioni

Tissue specificityi

Expressed in neuronal cells. Highly expressed in Purkinje cells of cerebellum.1 Publication

Gene expression databases

BgeeiP0C7T6.
GenevisibleiP0C7T6. MM.

Interactioni

Subunit structurei

Homodimer. Interacts (via AXH domain) with NCOR2 (By similarity). Interacts with ATXN1 and CIC. Directly interacts with RBPJ; this interaction is disrupted in the presence of Notch intracellular domain. Competes with ATXN1 for RBPJ-binding (By similarity).By similarity

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000090850.

Structurei

3D structure databases

ProteinModelPortaliP0C7T6.
SMRiP0C7T6. Positions 466-580.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini455 – 586132AXHPROSITE-ProRule annotationAdd
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni20 – 197178Interaction with NCOR2 and ATXN1By similarityAdd
BLAST
Regioni20 – 197178Self-associationBy similarityAdd
BLAST

Sequence similaritiesi

Belongs to the ATXN1 family.Curated
Contains 1 AXH domain.PROSITE-ProRule annotation

Phylogenomic databases

eggNOGiKOG4053. Eukaryota.
ENOG410XSNX. LUCA.
GeneTreeiENSGT00390000005939.
HOGENOMiHOG000034225.
HOVERGENiHBG100955.
InParanoidiP0C7T6.
OMAiTSCSTNH.
OrthoDBiEOG7PGDQ2.
PhylomeDBiP0C7T6.
TreeFamiTF350643.

Family and domain databases

Gene3Di2.170.16.10. 1 hit.
InterProiIPR003652. Ataxin_AXH_dom.
IPR028992. Hedgehog/Intein_dom.
[Graphical view]
PfamiPF08517. AXH. 1 hit.
[Graphical view]
SMARTiSM00536. AXH. 1 hit.
[Graphical view]
SUPFAMiSSF102031. SSF102031. 1 hit.
PROSITEiPS51148. AXH. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

P0C7T6-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKPVHERSQE CLPPKKRDLP VTSEDMGRTT SCSTNHTPSS DASEWSRGVV
60 70 80 90 100
VAGQSQTGAR VSLGGDGTEA ITGLTVDQYG MLYKVAVPPA TFSPTGLPSV
110 120 130 140 150
VNMSPLPPTF NVASSLIQHP GIHYPPVHYA QLPSTSLQFI GSPYSLPYAV
160 170 180 190 200
PPNFLPSPLL SPSANLATTH LPHFVPYASL LAEEATPPPQ AASPAQSFNK
210 220 230 240 250
SSSATSPPGQ LPHHSNTQPL DLAPGRMPIY YQMSRLPAGY TLHETSTAGA
260 270 280 290 300
SPILTPQEGQ SALEAAAANG QRQRERNVRR ESEALDSASS KGESQGLVPV
310 320 330 340 350
VECMADGQLF SGSQTPRVEV AAPAHRGTPD TDLEVQRVVG ALASQDYRVV
360 370 380 390 400
AAQRKDEPSP LNLSHHNLDH QGEGRGSARN PTELVEKSQA RVFYPQSHQE
410 420 430 440 450
PVKHRPLPKA MVVANGNLVP TGTDPSLLPV GSEILVASSL DLQARATFPD
460 470 480 490 500
KEPTPPPVTS SHLPSHFMKG AIIQLATGEL KRVEDLQTQD FVRSAEVSGG
510 520 530 540 550
LKIDSSTVVD IQESQWPGFV MLHFVVGEQQ SKVSIEVPPE HPFFVYGQGW
560 570 580 590 600
SSCSPGRTAQ LFSLPCHRLQ VGDVCISISL QSLNSNSVSQ ASCAPPGQLG
610 620 630 640 650
TPRERPERTV LGPRDLCDSE GKNQPSGEGS RVGEPSQPEP GAQACWPAPS
660 670 680
FQRFSMQGEE ARAAMLRPSF IPQEVKLSIE GRSNAGK
Length:687
Mass (Da):73,374
Last modified:July 22, 2008 - v1
Checksum:i952C394FFB6B8300
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC132138 Genomic DNA. No translation available.
CCDSiCCDS40474.1.
RefSeqiNP_001074399.1. NM_001080930.1.
XP_011246741.1. XM_011248439.1.
XP_011246742.1. XM_011248440.1.
XP_011246743.1. XM_011248441.1.
UniGeneiMm.276770.

Genome annotation databases

EnsembliENSMUST00000093162; ENSMUSP00000090850; ENSMUSG00000069895.
GeneIDi52335.
KEGGimmu:52335.
UCSCiuc009njh.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC132138 Genomic DNA. No translation available.
CCDSiCCDS40474.1.
RefSeqiNP_001074399.1. NM_001080930.1.
XP_011246741.1. XM_011248439.1.
XP_011246742.1. XM_011248440.1.
XP_011246743.1. XM_011248441.1.
UniGeneiMm.276770.

3D structure databases

ProteinModelPortaliP0C7T6.
SMRiP0C7T6. Positions 466-580.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000090850.

PTM databases

iPTMnetiP0C7T6.
PhosphoSiteiP0C7T6.

Proteomic databases

EPDiP0C7T6.
MaxQBiP0C7T6.
PaxDbiP0C7T6.
PeptideAtlasiP0C7T6.
PRIDEiP0C7T6.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000093162; ENSMUSP00000090850; ENSMUSG00000069895.
GeneIDi52335.
KEGGimmu:52335.
UCSCiuc009njh.1. mouse.

Organism-specific databases

CTDi342371.
MGIiMGI:3694797. Atxn1l.

Phylogenomic databases

eggNOGiKOG4053. Eukaryota.
ENOG410XSNX. LUCA.
GeneTreeiENSGT00390000005939.
HOGENOMiHOG000034225.
HOVERGENiHBG100955.
InParanoidiP0C7T6.
OMAiTSCSTNH.
OrthoDBiEOG7PGDQ2.
PhylomeDBiP0C7T6.
TreeFamiTF350643.

Miscellaneous databases

ChiTaRSiAtxn1l. mouse.
PROiP0C7T6.
SOURCEiSearch...

Gene expression databases

BgeeiP0C7T6.
GenevisibleiP0C7T6. MM.

Family and domain databases

Gene3Di2.170.16.10. 1 hit.
InterProiIPR003652. Ataxin_AXH_dom.
IPR028992. Hedgehog/Intein_dom.
[Graphical view]
PfamiPF08517. AXH. 1 hit.
[Graphical view]
SMARTiSM00536. AXH. 1 hit.
[Graphical view]
SUPFAMiSSF102031. SSF102031. 1 hit.
PROSITEiPS51148. AXH. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    Strain: C57BL/6J.
  2. "Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1."
    Mizutani A., Wang L., Rajan H., Vig P.J.S., Alaynick W.A., Thaler J.P., Tsai C.-C.
    EMBO J. 24:3339-3351(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, SUBCELLULAR LOCATION, TISSUE SPECIFICITY.
  3. "Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes."
    Bowman A.B., Lam Y.C., Jafar-Nejad P., Chen H.-K., Richman R., Samaco R.C., Fryer J.D., Kahle J.J., Orr H.T., Zoghbi H.Y.
    Nat. Genet. 39:373-379(2007) [PubMed] [Europe PMC] [Abstract]
    Cited for: INTERACTION WITH ATXN1 AND CIC.
  4. Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-282 AND SER-359, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Brain, Heart, Kidney, Lung and Spleen.

Entry informationi

Entry nameiATX1L_MOUSE
AccessioniPrimary (citable) accession number: P0C7T6
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 22, 2008
Last sequence update: July 22, 2008
Last modified: July 6, 2016
This is version 57 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Miscellaneous

Its overexpression suppresses the ataxia caused by polyglutamine-expanded Atxn1. Competes with mutant Atxn1 and wild-type Atxn1 for association with CIC. Decreased association of mutant Atxn1 into its CIC-containing complexes decreases the levels of mutant Atxn1-containing CIC complexes, suppressing pathology, while promoting aggregation and thus increasing nuclear inclusions.

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.