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

Aspartyl/asparaginyl beta-hydroxylase

Gene

Asph

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

Functioni

Isoform 1: specifically hydroxylates an Asp or Asn residue in certain epidermal growth factor-like (EGF) domains of a number of proteins.1 Publication

Catalytic activityi

Peptide L-aspartate + 2-oxoglutarate + O2 = peptide 3-hydroxy-L-aspartate + succinate + CO2.1 Publication

Cofactori

Fe cationBy similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei6082-oxoglutarateBy similarity1
Binding sitei6512-oxoglutarateBy similarity1
Metal bindingi662IronBy similarity1
Metal bindingi708IronBy similarity1
Binding sitei7182-oxoglutarateBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Calcium bindingi99 – 111By similarityAdd BLAST13

GO - Molecular functioni

  • metal ion binding Source: UniProtKB-KW
  • peptide-aspartate beta-dioxygenase activity Source: MGI

GO - Biological processi

  • activation of cysteine-type endopeptidase activity Source: MGI
  • face morphogenesis Source: MGI
  • limb morphogenesis Source: MGI
  • negative regulation of cell proliferation Source: MGI
  • palate development Source: MGI
  • pattern specification process Source: MGI
  • peptidyl-aspartic acid hydroxylation Source: MGI
  • positive regulation of proteolysis Source: MGI
  • regulation of protein depolymerization Source: BHF-UCL
  • regulation of protein stability Source: BHF-UCL
Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Ligandi

Calcium, Iron, Metal-binding

Enzyme and pathway databases

ReactomeiR-MMU-2672351. Stimuli-sensing channels.
R-MMU-5578775. Ion homeostasis.

Names & Taxonomyi

Protein namesi
Recommended name:
Aspartyl/asparaginyl beta-hydroxylase (EC:1.14.11.161 Publication)
Alternative name(s):
Aspartate beta-hydroxylase
Short name:
ASP beta-hydroxylase
Peptide-aspartate beta-dioxygenase
Gene namesi
Name:Asph
Synonyms:Bah
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 4

Organism-specific databases

MGIiMGI:1914186. Asph.

Subcellular locationi

Isoform 1 :
Isoform 2 :

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 62CytoplasmicSequence analysisAdd BLAST62
Transmembranei63 – 83Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST21
Topological domaini84 – 741LumenalSequence analysisAdd BLAST658

GO - Cellular componenti

  • cortical endoplasmic reticulum Source: MGI
  • cytoplasm Source: BHF-UCL
  • endoplasmic reticulum Source: MGI
  • integral component of membrane Source: MGI
  • plasma membrane Source: MGI
  • sarcoplasmic reticulum membrane Source: UniProtKB-SubCell
Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane, Sarcoplasmic reticulum

Pathology & Biotechi

Disruption phenotypei

Selective disruption of isoform 1 abolishes liver aspartyl beta-hydroxylase activity, but does not affect the expression of isoform 2. Mice lacking isoform 1 have normal blood chemistry, do not present blood coagulation defects and appear more or less normal, except for shorter snouts, mild defects of the palate ridges, syndactily due to fusion of soft tissues and reduced litter size from mutant females, while male fertiliy appears normal. Mice lacking isoform 2 show no visible phenotype.2 Publications

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002541611 – 741Aspartyl/asparaginyl beta-hydroxylaseAdd BLAST741

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei15PhosphoserineBy similarity1
Glycosylationi453N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi624 ↔ 631By similarity
Glycosylationi689N-linked (GlcNAc...)Sequence analysis1

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein

Proteomic databases

EPDiQ8BSY0.
PaxDbiQ8BSY0.
PRIDEiQ8BSY0.

PTM databases

iPTMnetiQ8BSY0.
PhosphoSitePlusiQ8BSY0.

Expressioni

Tissue specificityi

Isoform 1 is detected in heart, liver and ovary (at protein level). Detected in heart ventricle. Isoform 1 is widely expressed. Isoform 2 is detected in heart and skeletal muscle.3 Publications

Developmental stagei

Strongly expressed in the snout, limbs and eye of embryonic day 11.5 (E11.5) and E12. Strong localization of the protein in the lens of the developing eye at all three stages.1 Publication

Gene expression databases

BgeeiENSMUSG00000028207.
CleanExiMM_ASPH.
ExpressionAtlasiQ8BSY0. baseline and differential.
GenevisibleiQ8BSY0. MM.

Interactioni

Subunit structurei

Monomer. Isoform 2 interacts with CASQ2.By similarity

Protein-protein interaction databases

BioGridi211167. 2 interactors.
IntActiQ8BSY0. 8 interactors.
MINTiMINT-4112262.
STRINGi10090.ENSMUSP00000077273.

Structurei

3D structure databases

ProteinModelPortaliQ8BSY0.
SMRiQ8BSY0.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati324 – 357TPR 1Add BLAST34
Repeati365 – 398TPR 2Add BLAST34
Repeati437 – 470TPR 3Add BLAST34
Repeati472 – 504TPR 4Add BLAST33
Repeati508 – 540TPR 5Add BLAST33

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni671 – 6732-oxoglutarate bindingBy similarity3

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi9 – 61Gly-richAdd BLAST53
Compositional biasi14 – 38Ser-richAdd BLAST25
Compositional biasi310 – 315Poly-Lys6

Sequence similaritiesi

Contains 5 TPR repeats.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Signal-anchor, TPR repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3696. Eukaryota.
COG3555. LUCA.
GeneTreeiENSGT00530000063281.
HOGENOMiHOG000231625.
HOVERGENiHBG004290.
InParanoidiQ8BSY0.
KOiK00476.
OMAiRNENACK.
OrthoDBiEOG091G0H5R.
PhylomeDBiQ8BSY0.
TreeFamiTF312799.

Family and domain databases

Gene3Di1.25.40.10. 1 hit.
2.60.120.330. 1 hit.
InterProiIPR007943. Asp-B-hydro/Triadin_dom.
IPR007803. Asp/Arg/Pro-Hydrxlase.
IPR027443. IPNS-like.
IPR013026. TPR-contain_dom.
IPR011990. TPR-like_helical_dom.
IPR019734. TPR_repeat.
[Graphical view]
PfamiPF05279. Asp-B-Hydro_N. 1 hit.
PF05118. Asp_Arg_Hydrox. 1 hit.
[Graphical view]
SMARTiSM00028. TPR. 2 hits.
[Graphical view]
SUPFAMiSSF48452. SSF48452. 1 hit.
PROSITEiPS50005. TPR. 2 hits.
PS50293. TPR_REGION. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q8BSY0-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAPRKNAKGG GGNSSSSGSG SGSGSGSPST GSSGSSSSPG ARREAKHGGH
60 70 80 90 100
KNGRRGGISG GSFFTWFMVI ALLGVWTSVA VVWFDLVDYE EVLGKLGVYD
110 120 130 140 150
ADGDGDFDVD DAKVLLGLKE RSPSERTFPP EEEAETHAEL EEQAPEGADI
160 170 180 190 200
QNVEDEVKEQ IQSLLQESVH TDHDLEADGL AGEPQPEVED FLTVTDSDDR
210 220 230 240 250
FEDLEPGTVH EEIEDTYHVE DTASQNHPND MEEMTNEQEN SDPSEAVTDA
260 270 280 290 300
GVLLPHAEEV RHQDYDEPVY EPSEHEGVAI SDNTIDDSSI ISEEINVASV
310 320 330 340 350
EEQQDTPPVK KKKPKLLNKF DKTIKAELDA AEKLRKRGKI EEAVNAFEEL
360 370 380 390 400
VRKYPQSPRA RYGKAQCEDD LAEKQRSNEV LRRAIETYQE AADLPDAPTD
410 420 430 440 450
LVKLSLKRRS ERQQFLGHMR GSLLTLQRLV QLFPSDTTLK NDLGVGYLLL
460 470 480 490 500
GDNDSAKKVY EEVLNVTPND GFAKVHYGFI LKAQNKISES IPYLKEGIES
510 520 530 540 550
GDPGTDDGRF YFHLGDAMQR VGNKEAYKWY ELGHKRGHFA SVWQRSLYNV
560 570 580 590 600
NGLKAQPWWT PRETGYTELV KSLERNWKLI RDEGLMVMDK AKGLFLPEDE
610 620 630 640 650
NLREKGDWSQ FTLWQQGRKN ENACKGAPKT CALLEKFSET TGCRRGQIKY
660 670 680 690 700
SIMHPGTHVW PHTGPTNCRL RMHLGLVIPK EGCKIRCANE TRTWEEGKVL
710 720 730 740
IFDDSFEHEV WQDASSFRLI FIVDVWHPEL TPQQRRSLPA I
Length:741
Mass (Da):83,042
Last modified:March 1, 2003 - v1
Checksum:i0660A6A5E34418C8
GO
Isoform 2 (identifier: Q8BSY0-2) [UniParc]FASTAAdd to basket
Also known as: Junctin

The sequence of this isoform differs from the canonical sequence as follows:
     1-43: MAPRKNAKGGGGNSSSSGSGSGSGSGSPSTGSSGSSSSPGARR → MAEDK
     117-245: GLKERSPSER...NEQENSDPSE → EGPGGLAKRK...SKENGQKRKN
     246-741: Missing.

Show »
Length:207
Mass (Da):22,757
Checksum:i37A13D30E49CD67C
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti131 – 132Missing in AAG40808 (PubMed:10956665).Curated2
Sequence conflicti131 – 132Missing in AAG40809 (PubMed:10956665).Curated2
Sequence conflicti279A → E in AAG40808 (PubMed:10956665).Curated1
Sequence conflicti279A → E in AAG40809 (PubMed:10956665).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0567921 – 43MAPRK…PGARR → MAEDK in isoform 2. CuratedAdd BLAST43
Alternative sequenceiVSP_056793117 – 245GLKER…SDPSE → EGPGGLAKRKTKAKAKEPIK EELKKERGKAVPSKNEERRQ GKKEQEDRGKGRKKPDSDTS QKASAAGKRDRDKEKASSDK SSKSKESWKKAVETKAVSSK VAARDKDRRGRSSSGHAHVS KENGQKRKN in isoform 2. CuratedAdd BLAST129
Alternative sequenceiVSP_056794246 – 741Missing in isoform 2. CuratedAdd BLAST496

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF221854 mRNA. Translation: AAK00614.1.
AF289486 mRNA. Translation: AAG40808.1.
AF289487 mRNA. Translation: AAG40809.1.
AF289490 mRNA. Translation: AAG40812.1.
AF223413 mRNA. Translation: AAN87549.1.
AK030293 mRNA. Translation: BAC26882.1.
BC128299 mRNA. Translation: AAI28300.1.
BC152365 mRNA. Translation: AAI52366.1.
CCDSiCCDS17959.1. [Q8BSY0-2]
CCDS38690.1. [Q8BSY0-1]
RefSeqiNP_001171321.1. NM_001177850.1.
NP_001171323.1. NM_001177852.1.
NP_001171324.1. NM_001177853.1.
NP_001171325.1. NM_001177854.1.
NP_001171326.1. NM_001177855.1.
NP_001277296.1. NM_001290367.1.
NP_075553.2. NM_023066.3. [Q8BSY0-1]
UniGeneiMm.222206.
Mm.412008.

Genome annotation databases

EnsembliENSMUST00000078139; ENSMUSP00000077273; ENSMUSG00000028207. [Q8BSY0-1]
GeneIDi65973.
KEGGimmu:65973.
UCSCiuc008ryf.2. mouse. [Q8BSY0-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF221854 mRNA. Translation: AAK00614.1.
AF289486 mRNA. Translation: AAG40808.1.
AF289487 mRNA. Translation: AAG40809.1.
AF289490 mRNA. Translation: AAG40812.1.
AF223413 mRNA. Translation: AAN87549.1.
AK030293 mRNA. Translation: BAC26882.1.
BC128299 mRNA. Translation: AAI28300.1.
BC152365 mRNA. Translation: AAI52366.1.
CCDSiCCDS17959.1. [Q8BSY0-2]
CCDS38690.1. [Q8BSY0-1]
RefSeqiNP_001171321.1. NM_001177850.1.
NP_001171323.1. NM_001177852.1.
NP_001171324.1. NM_001177853.1.
NP_001171325.1. NM_001177854.1.
NP_001171326.1. NM_001177855.1.
NP_001277296.1. NM_001290367.1.
NP_075553.2. NM_023066.3. [Q8BSY0-1]
UniGeneiMm.222206.
Mm.412008.

3D structure databases

ProteinModelPortaliQ8BSY0.
SMRiQ8BSY0.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi211167. 2 interactors.
IntActiQ8BSY0. 8 interactors.
MINTiMINT-4112262.
STRINGi10090.ENSMUSP00000077273.

PTM databases

iPTMnetiQ8BSY0.
PhosphoSitePlusiQ8BSY0.

Proteomic databases

EPDiQ8BSY0.
PaxDbiQ8BSY0.
PRIDEiQ8BSY0.

Protocols and materials databases

DNASUi65973.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000078139; ENSMUSP00000077273; ENSMUSG00000028207. [Q8BSY0-1]
GeneIDi65973.
KEGGimmu:65973.
UCSCiuc008ryf.2. mouse. [Q8BSY0-1]

Organism-specific databases

CTDi444.
MGIiMGI:1914186. Asph.

Phylogenomic databases

eggNOGiKOG3696. Eukaryota.
COG3555. LUCA.
GeneTreeiENSGT00530000063281.
HOGENOMiHOG000231625.
HOVERGENiHBG004290.
InParanoidiQ8BSY0.
KOiK00476.
OMAiRNENACK.
OrthoDBiEOG091G0H5R.
PhylomeDBiQ8BSY0.
TreeFamiTF312799.

Enzyme and pathway databases

ReactomeiR-MMU-2672351. Stimuli-sensing channels.
R-MMU-5578775. Ion homeostasis.

Miscellaneous databases

PROiQ8BSY0.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000028207.
CleanExiMM_ASPH.
ExpressionAtlasiQ8BSY0. baseline and differential.
GenevisibleiQ8BSY0. MM.

Family and domain databases

Gene3Di1.25.40.10. 1 hit.
2.60.120.330. 1 hit.
InterProiIPR007943. Asp-B-hydro/Triadin_dom.
IPR007803. Asp/Arg/Pro-Hydrxlase.
IPR027443. IPNS-like.
IPR013026. TPR-contain_dom.
IPR011990. TPR-like_helical_dom.
IPR019734. TPR_repeat.
[Graphical view]
PfamiPF05279. Asp-B-Hydro_N. 1 hit.
PF05118. Asp_Arg_Hydrox. 1 hit.
[Graphical view]
SMARTiSM00028. TPR. 2 hits.
[Graphical view]
SUPFAMiSSF48452. SSF48452. 1 hit.
PROSITEiPS50005. TPR. 2 hits.
PS50293. TPR_REGION. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiASPH_MOUSE
AccessioniPrimary (citable) accession number: Q8BSY0
Secondary accession number(s): Q9EPA6, Q9EQ64
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 31, 2006
Last sequence update: March 1, 2003
Last modified: November 2, 2016
This is version 126 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, 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.