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Protein

2-phosphoxylose phosphatase 1

Gene

PXYLP1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Responsible for the 2-O-dephosphorylation of xylose in the glycosaminoglycan-protein linkage region of proteoglycans thereby regulating the amount of mature glycosaminoglycan (GAG) chains. Sulfated glycosaminoglycans (GAGs), including heparan sulfate and chondroitin sulfate, are synthesized on the so-called common GAG-protein linkage region (GlcUAbeta1-3Galbeta1-3Galbeta1-4Xylbeta1-O-Ser) of core proteins, which is formed by the stepwise addition of monosaccharide residues by the respective specific glycosyltransferases. Xylose 2-O-dephosphorylation during completion of linkage region formation is a prerequisite for the initiation and efficient elongation of the repeating disaccharide region of GAG chains.1 Publication

pH dependencei

Optimum pH is 5.8.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei97NucleophileBy similarity1
Active sitei379Proton donorBy similarity1

GO - Molecular functioni

  • acid phosphatase activity Source: InterPro
  • phosphatase activity Source: UniProtKB

GO - Biological processi

  • chondroitin sulfate proteoglycan biosynthetic process Source: UniProtKB
  • glycosaminoglycan biosynthetic process Source: UniProtKB
  • positive regulation of heparan sulfate proteoglycan biosynthetic process Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Enzyme and pathway databases

BioCyciZFISH:HS08078-MONOMER.

Names & Taxonomyi

Protein namesi
Recommended name:
2-phosphoxylose phosphatase 11 Publication (EC:3.1.3.-1 Publication)
Alternative name(s):
Acid phosphatase-like protein 2
Xylosyl phosphatase1 PublicationImported
epididymis luminal protein 1241 PublicationImported
Gene namesi
Name:PXYLP1Imported
Synonyms:ACPL2, HEL1241 PublicationImported, XYLP1 Publication
ORF Names:UNQ370/PRO706
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

HGNCiHGNC:26303. PXYLP1.

Subcellular locationi

  • Golgi apparatus membrane 1 Publication; Single-pass type II membrane protein Sequence analysis

  • Note: Colocalizes to Golgi apparatus in a B3GAT3-dependent manner.1 Publication

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 6CytoplasmicSequence analysis6
Transmembranei7 – 27Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST21
Topological domaini28 – 480LumenalSequence analysisAdd BLAST453

GO - Cellular componenti

  • Golgi apparatus Source: UniProtKB
  • Golgi membrane Source: UniProtKB-SubCell
  • integral component of membrane Source: UniProtKB-KW
Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi92370.
OpenTargetsiENSG00000155893.
PharmGKBiPA134964387.

Polymorphism and mutation databases

BioMutaiACPL2.
DMDMi74730610.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003149241 – 4802-phosphoxylose phosphatase 1Add BLAST480

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi305N-linked (GlcNAc...)Sequence analysis1
Glycosylationi354N-linked (GlcNAc...)PROSITE-ProRule annotation1

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiQ8TE99.
PaxDbiQ8TE99.
PeptideAtlasiQ8TE99.
PRIDEiQ8TE99.

PTM databases

DEPODiQ8TE99.
iPTMnetiQ8TE99.
PhosphoSitePlusiQ8TE99.

Expressioni

Tissue specificityi

Widely expressed. Strongly expressed in spleen, fetal liver, moderately in placenta, pancreas, kidney, thymus and colon.1 Publication

Gene expression databases

BgeeiENSG00000155893.
CleanExiHS_ACPL2.
ExpressionAtlasiQ8TE99. baseline and differential.
GenevisibleiQ8TE99. HS.

Organism-specific databases

HPAiHPA017243.

Interactioni

Subunit structurei

Interacts with B3GAT3; the interaction increases the 2-phosphoxylose phosphatase activity of PXYLP1 during completion of linkage region formation in a B3GAT3-mediated manner.1 Publication

Protein-protein interaction databases

BioGridi124942. 8 interactors.
STRINGi9606.ENSP00000286353.

Structurei

3D structure databases

ProteinModelPortaliQ8TE99.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the histidine acid phosphatase family.Curated

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3672. Eukaryota.
ENOG410YGWX. LUCA.
GeneTreeiENSGT00390000016324.
HOGENOMiHOG000033787.
HOVERGENiHBG106056.
InParanoidiQ8TE99.
OMAiPATERGC.
OrthoDBiEOG091G05TQ.
PhylomeDBiQ8TE99.
TreeFamiTF318821.

Family and domain databases

CDDicd07061. HP_HAP_like. 1 hit.
Gene3Di3.40.50.1240. 2 hits.
InterProiIPR000560. His_Pase_clade-2.
IPR029033. His_PPase_superfam.
[Graphical view]
PfamiPF00328. His_Phos_2. 1 hit.
[Graphical view]
SUPFAMiSSF53254. SSF53254. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

Isoform 1 (identifier: Q8TE99-1) [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
MLFRNRFLLL LALAALLAFV SLSLQFFHLI PVSTPKNGMS SKSRKRIMPD
60 70 80 90 100
PVTEPPVTDP VYEALLYCNI PSVAERSMEG HAPHHFKLVS VHVFIRHGDR
110 120 130 140 150
YPLYVIPKTK RPEIDCTLVA NRKPYHPKLE AFISHMSKGS GASFESPLNS
160 170 180 190 200
LPLYPNHPLC EMGELTQTGV VQHLQNGQLL RDIYLKKHKL LPNDWSADQL
210 220 230 240 250
YLETTGKSRT LQSGLALLYG FLPDFDWKKI YFRHQPSALF CSGSCYCPVR
260 270 280 290 300
NQYLEKEQRR QYLLRLKNSQ LEKTYGEMAK IVDVPTKQLR AANPIDSMLC
310 320 330 340 350
HFCHNVSFPC TRNGCVDMEH FKVIKTHQIE DERERREKKL YFGYSLLGAH
360 370 380 390 400
PILNQTIGRM QRATEGRKEE LFALYSAHDV TLSPVLSALG LSEARFPRFA
410 420 430 440 450
ARLIFELWQD REKPSEHSVR ILYNGVDVTF HTSFCQDHHK RSPKPMCPLE
460 470 480
NLVRFVKRDM FVALGGSGTN YYDACHREGF
Length:480
Mass (Da):55,240
Last modified:June 1, 2002 - v1
Checksum:iC1A8BEC2B68ECA62
GO
Isoform 2 (identifier: Q8TE99-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-27: MLFRNRFLLLLALAALLAFVSLSLQFF → MEHSVCPSSAV

Note: No experimental confirmation available.
Show »
Length:464
Mass (Da):53,257
Checksum:i334FF1A12565DADA
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0304321 – 27MLFRN…SLQFF → MEHSVCPSSAV in isoform 2. 1 PublicationAdd BLAST27

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB827640 mRNA. Translation: BAO45795.1.
EU794677 mRNA. Translation: ACJ13731.1.
AY358460 mRNA. Translation: AAQ88825.1.
AK074331 mRNA. Translation: BAB85053.1.
CH471052 Genomic DNA. Translation: EAW79007.1.
CH471052 Genomic DNA. Translation: EAW79009.1.
BC035834 mRNA. Translation: AAH35834.1.
BC036701 mRNA. Translation: AAH36701.1.
CCDSiCCDS3116.1. [Q8TE99-1]
RefSeqiNP_001032249.1. NM_001037172.2. [Q8TE99-1]
NP_001269657.1. NM_001282728.1.
NP_689495.1. NM_152282.4. [Q8TE99-1]
XP_016862999.1. XM_017007510.1. [Q8TE99-1]
UniGeneiHs.657887.

Genome annotation databases

EnsembliENST00000286353; ENSP00000286353; ENSG00000155893. [Q8TE99-1]
ENST00000393010; ENSP00000376733; ENSG00000155893. [Q8TE99-1]
GeneIDi92370.
KEGGihsa:92370.
UCSCiuc003etu.5. human. [Q8TE99-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB827640 mRNA. Translation: BAO45795.1.
EU794677 mRNA. Translation: ACJ13731.1.
AY358460 mRNA. Translation: AAQ88825.1.
AK074331 mRNA. Translation: BAB85053.1.
CH471052 Genomic DNA. Translation: EAW79007.1.
CH471052 Genomic DNA. Translation: EAW79009.1.
BC035834 mRNA. Translation: AAH35834.1.
BC036701 mRNA. Translation: AAH36701.1.
CCDSiCCDS3116.1. [Q8TE99-1]
RefSeqiNP_001032249.1. NM_001037172.2. [Q8TE99-1]
NP_001269657.1. NM_001282728.1.
NP_689495.1. NM_152282.4. [Q8TE99-1]
XP_016862999.1. XM_017007510.1. [Q8TE99-1]
UniGeneiHs.657887.

3D structure databases

ProteinModelPortaliQ8TE99.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124942. 8 interactors.
STRINGi9606.ENSP00000286353.

PTM databases

DEPODiQ8TE99.
iPTMnetiQ8TE99.
PhosphoSitePlusiQ8TE99.

Polymorphism and mutation databases

BioMutaiACPL2.
DMDMi74730610.

Proteomic databases

EPDiQ8TE99.
PaxDbiQ8TE99.
PeptideAtlasiQ8TE99.
PRIDEiQ8TE99.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000286353; ENSP00000286353; ENSG00000155893. [Q8TE99-1]
ENST00000393010; ENSP00000376733; ENSG00000155893. [Q8TE99-1]
GeneIDi92370.
KEGGihsa:92370.
UCSCiuc003etu.5. human. [Q8TE99-1]

Organism-specific databases

CTDi92370.
DisGeNETi92370.
GeneCardsiPXYLP1.
HGNCiHGNC:26303. PXYLP1.
HPAiHPA017243.
neXtProtiNX_Q8TE99.
OpenTargetsiENSG00000155893.
PharmGKBiPA134964387.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3672. Eukaryota.
ENOG410YGWX. LUCA.
GeneTreeiENSGT00390000016324.
HOGENOMiHOG000033787.
HOVERGENiHBG106056.
InParanoidiQ8TE99.
OMAiPATERGC.
OrthoDBiEOG091G05TQ.
PhylomeDBiQ8TE99.
TreeFamiTF318821.

Enzyme and pathway databases

BioCyciZFISH:HS08078-MONOMER.

Miscellaneous databases

GenomeRNAii92370.
PROiQ8TE99.

Gene expression databases

BgeeiENSG00000155893.
CleanExiHS_ACPL2.
ExpressionAtlasiQ8TE99. baseline and differential.
GenevisibleiQ8TE99. HS.

Family and domain databases

CDDicd07061. HP_HAP_like. 1 hit.
Gene3Di3.40.50.1240. 2 hits.
InterProiIPR000560. His_Pase_clade-2.
IPR029033. His_PPase_superfam.
[Graphical view]
PfamiPF00328. His_Phos_2. 1 hit.
[Graphical view]
SUPFAMiSSF53254. SSF53254. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiPXYP1_HUMAN
AccessioniPrimary (citable) accession number: Q8TE99
Secondary accession number(s): D3DNF5, Q49AJ2, W0TR04
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 15, 2008
Last sequence update: June 1, 2002
Last modified: November 2, 2016
This is version 109 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  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.