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Protein

Phospholysine phosphohistidine inorganic pyrophosphate phosphatase

Gene

LHPP

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

Functioni

Phosphatase that hydrolyzes imidodiphosphate, 3-phosphohistidine and 6-phospholysine. Has broad substrate specificity and can also hydrolyze inorganic diphosphate, but with lower efficiency (By similarity).By similarity

Catalytic activityi

Diphosphate + H2O = 2 phosphate.1 Publication

Cofactori

Mg2+2 PublicationsNote: Binds 1 Mg2+ ion per subunit.2 Publications

pH dependencei

Optimum pH is 7.0 for PNP, and 5.5 for PPi.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi17Magnesium1
Metal bindingi19Magnesium; via carbonyl oxygen1
Binding sitei189Substrate1
Metal bindingi214Magnesium1

GO - Molecular functioni

  • inorganic diphosphatase activity Source: UniProtKB
  • magnesium ion binding Source: UniProtKB
  • phosphohistidine phosphatase activity Source: UniProtKB
  • protein homodimerization activity Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Keywords - Ligandi

Magnesium, Metal-binding

Enzyme and pathway databases

BioCyciZFISH:HS03044-MONOMER.
ReactomeiR-HSA-73817. Purine ribonucleoside monophosphate biosynthesis.

Names & Taxonomyi

Protein namesi
Recommended name:
Phospholysine phosphohistidine inorganic pyrophosphate phosphatase (EC:3.1.3.-, EC:3.6.1.1)
Short name:
hLHPP
Gene namesi
Name:LHPP
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 10

Organism-specific databases

HGNCiHGNC:30042. LHPP.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: HPA
  • cytosol Source: UniProtKB
  • nucleoplasm Source: HPA
  • nucleus Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Nucleus

Pathology & Biotechi

Organism-specific databases

DisGeNETi64077.
OpenTargetsiENSG00000107902.
PharmGKBiPA165548763.

Polymorphism and mutation databases

BioMutaiLHPP.
DMDMi158705883.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003050741 – 270Phospholysine phosphohistidine inorganic pyrophosphate phosphataseAdd BLAST270

Proteomic databases

EPDiQ9H008.
MaxQBiQ9H008.
PaxDbiQ9H008.
PeptideAtlasiQ9H008.
PRIDEiQ9H008.

PTM databases

DEPODiQ9H008.
iPTMnetiQ9H008.
PhosphoSitePlusiQ9H008.

Expressioni

Tissue specificityi

Expressed in brain, and at lower levels in liver and kidney. Detected in thyroid (at protein level). Expressed in liver, kidney and moderately in brain.2 Publications

Gene expression databases

BgeeiENSG00000107902.
ExpressionAtlasiQ9H008. baseline and differential.
GenevisibleiQ9H008. HS.

Organism-specific databases

HPAiHPA009163.
HPA009269.

Interactioni

Subunit structurei

Homodimer.2 Publications

GO - Molecular functioni

  • protein homodimerization activity Source: UniProtKB

Protein-protein interaction databases

BioGridi122044. 2 interactors.
STRINGi9606.ENSP00000357835.

Structurei

Secondary structure

1270
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi4 – 7Combined sources4
Turni8 – 10Combined sources3
Beta strandi13 – 16Combined sources4
Turni19 – 21Combined sources3
Beta strandi22 – 24Combined sources3
Turni27 – 29Combined sources3
Helixi36 – 45Combined sources10
Beta strandi46 – 53Combined sources8
Helixi61 – 70Combined sources10
Helixi77 – 79Combined sources3
Helixi83 – 94Combined sources12
Beta strandi98 – 101Combined sources4
Helixi104 – 110Combined sources7
Beta strandi119 – 123Combined sources5
Helixi127 – 129Combined sources3
Helixi132 – 144Combined sources13
Beta strandi150 – 153Combined sources4
Beta strandi157 – 161Combined sources5
Beta strandi164 – 167Combined sources4
Helixi169 – 180Combined sources12
Beta strandi185 – 188Combined sources4
Helixi192 – 202Combined sources11
Helixi206 – 208Combined sources3
Beta strandi209 – 214Combined sources6
Turni216 – 219Combined sources4
Helixi220 – 225Combined sources6
Beta strandi229 – 235Combined sources7
Helixi240 – 244Combined sources5
Beta strandi251 – 256Combined sources6
Helixi257 – 267Combined sources11

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2X4DX-ray1.92A/B1-270[»]
ProteinModelPortaliQ9H008.
SMRiQ9H008.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni17 – 19Substrate binding3
Regioni54 – 55Substrate binding2

Sequence similaritiesi

Belongs to the HAD-like hydrolase superfamily.Curated

Phylogenomic databases

eggNOGiKOG3040. Eukaryota.
COG0647. LUCA.
GeneTreeiENSGT00510000046678.
HOGENOMiHOG000068106.
HOVERGENiHBG075146.
InParanoidiQ9H008.
KOiK11725.
OMAiRPYLLIH.
OrthoDBiEOG091G191B.
PhylomeDBiQ9H008.
TreeFamiTF314344.

Family and domain databases

Gene3Di3.40.50.1000. 2 hits.
3.40.50.10410. 1 hit.
InterProiIPR023214. HAD-like_dom.
IPR006357. HAD-SF_hydro_IIA.
IPR006355. HAD-SF_hydro_IIA_hyp2.
IPR023215. NPhePase-like_dom.
[Graphical view]
PfamiPF13344. Hydrolase_6. 1 hit.
[Graphical view]
SUPFAMiSSF56784. SSF56784. 1 hit.
TIGRFAMsiTIGR01460. HAD-SF-IIA. 1 hit.
TIGR01458. HAD-SF-IIA-hyp3. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

Isoform 1 (identifier: Q9H008-1) [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
MAPWGKRLAG VRGVLLDISG VLYDSGAGGG TAIAGSVEAV ARLKRSRLKV
60 70 80 90 100
RFCTNESQKS RAELVGQLQR LGFDISEQEV TAPAPAACQI LKEQGLRPYL
110 120 130 140 150
LIHDGVRSEF DQIDTSNPNC VVIADAGESF SYQNMNNAFQ VLMELEKPVL
160 170 180 190 200
ISLGKGRYYK ETSGLMLDVG PYMKALEYAC GIKAEVVGKP SPEFFKSALQ
210 220 230 240 250
AIGVEAHQAV MIGDDIVGDV GGAQRCGMRA LQVRTGKFRP SDEHHPEVKA
260 270
DGYVDNLAEA VDLLLQHADK
Length:270
Mass (Da):29,165
Last modified:October 2, 2007 - v2
Checksum:i09C301584453E79D
GO
Isoform 2 (identifier: Q9H008-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     210-210: V → Q
     211-270: Missing.

Note: No experimental confirmation available.
Show »
Length:210
Mass (Da):22,696
Checksum:i93F0D37C2D348195
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti155K → R in BAG51532 (PubMed:14702039).Curated1
Sequence conflicti170G → C in AAI10345 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03516394Q → R.3 PublicationsCorresponds to variant rs6597801dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_041685210V → Q in isoform 2. 1 Publication1
Alternative sequenceiVSP_041686211 – 270Missing in isoform 2. 1 PublicationAdd BLAST60

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB049629 mRNA. Translation: BAB16411.1.
AK055532 mRNA. Translation: BAG51532.1.
AL513190, AL445237 Genomic DNA. Translation: CAH70377.1.
AL513190, AL391708, AL445237 Genomic DNA. Translation: CAH70378.1.
AL391708, AL445237, AL513190 Genomic DNA. Translation: CAH74011.1.
AL445237, AL513190 Genomic DNA. Translation: CAI17295.1.
AL445237, AL391708, AL513190 Genomic DNA. Translation: CAI17296.1.
BC110344 mRNA. Translation: AAI10345.1.
BC113629 mRNA. Translation: AAI13630.1.
BC113631 mRNA. Translation: AAI13632.1.
CCDSiCCDS53587.1. [Q9H008-2]
CCDS7640.1. [Q9H008-1]
RefSeqiNP_001161352.1. NM_001167880.1. [Q9H008-2]
NP_001305260.1. NM_001318331.1.
NP_001305261.1. NM_001318332.1.
NP_071409.3. NM_022126.3. [Q9H008-1]
UniGeneiHs.527748.

Genome annotation databases

EnsembliENST00000368839; ENSP00000357832; ENSG00000107902. [Q9H008-2]
ENST00000368842; ENSP00000357835; ENSG00000107902. [Q9H008-1]
GeneIDi64077.
KEGGihsa:64077.
UCSCiuc001lhs.3. human. [Q9H008-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB049629 mRNA. Translation: BAB16411.1.
AK055532 mRNA. Translation: BAG51532.1.
AL513190, AL445237 Genomic DNA. Translation: CAH70377.1.
AL513190, AL391708, AL445237 Genomic DNA. Translation: CAH70378.1.
AL391708, AL445237, AL513190 Genomic DNA. Translation: CAH74011.1.
AL445237, AL513190 Genomic DNA. Translation: CAI17295.1.
AL445237, AL391708, AL513190 Genomic DNA. Translation: CAI17296.1.
BC110344 mRNA. Translation: AAI10345.1.
BC113629 mRNA. Translation: AAI13630.1.
BC113631 mRNA. Translation: AAI13632.1.
CCDSiCCDS53587.1. [Q9H008-2]
CCDS7640.1. [Q9H008-1]
RefSeqiNP_001161352.1. NM_001167880.1. [Q9H008-2]
NP_001305260.1. NM_001318331.1.
NP_001305261.1. NM_001318332.1.
NP_071409.3. NM_022126.3. [Q9H008-1]
UniGeneiHs.527748.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2X4DX-ray1.92A/B1-270[»]
ProteinModelPortaliQ9H008.
SMRiQ9H008.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi122044. 2 interactors.
STRINGi9606.ENSP00000357835.

PTM databases

DEPODiQ9H008.
iPTMnetiQ9H008.
PhosphoSitePlusiQ9H008.

Polymorphism and mutation databases

BioMutaiLHPP.
DMDMi158705883.

Proteomic databases

EPDiQ9H008.
MaxQBiQ9H008.
PaxDbiQ9H008.
PeptideAtlasiQ9H008.
PRIDEiQ9H008.

Protocols and materials databases

DNASUi64077.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000368839; ENSP00000357832; ENSG00000107902. [Q9H008-2]
ENST00000368842; ENSP00000357835; ENSG00000107902. [Q9H008-1]
GeneIDi64077.
KEGGihsa:64077.
UCSCiuc001lhs.3. human. [Q9H008-1]

Organism-specific databases

CTDi64077.
DisGeNETi64077.
GeneCardsiLHPP.
HGNCiHGNC:30042. LHPP.
HPAiHPA009163.
HPA009269.
neXtProtiNX_Q9H008.
OpenTargetsiENSG00000107902.
PharmGKBiPA165548763.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3040. Eukaryota.
COG0647. LUCA.
GeneTreeiENSGT00510000046678.
HOGENOMiHOG000068106.
HOVERGENiHBG075146.
InParanoidiQ9H008.
KOiK11725.
OMAiRPYLLIH.
OrthoDBiEOG091G191B.
PhylomeDBiQ9H008.
TreeFamiTF314344.

Enzyme and pathway databases

BioCyciZFISH:HS03044-MONOMER.
ReactomeiR-HSA-73817. Purine ribonucleoside monophosphate biosynthesis.

Miscellaneous databases

ChiTaRSiLHPP. human.
GenomeRNAii64077.
PROiQ9H008.

Gene expression databases

BgeeiENSG00000107902.
ExpressionAtlasiQ9H008. baseline and differential.
GenevisibleiQ9H008. HS.

Family and domain databases

Gene3Di3.40.50.1000. 2 hits.
3.40.50.10410. 1 hit.
InterProiIPR023214. HAD-like_dom.
IPR006357. HAD-SF_hydro_IIA.
IPR006355. HAD-SF_hydro_IIA_hyp2.
IPR023215. NPhePase-like_dom.
[Graphical view]
PfamiPF13344. Hydrolase_6. 1 hit.
[Graphical view]
SUPFAMiSSF56784. SSF56784. 1 hit.
TIGRFAMsiTIGR01460. HAD-SF-IIA. 1 hit.
TIGR01458. HAD-SF-IIA-hyp3. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiLHPP_HUMAN
AccessioniPrimary (citable) accession number: Q9H008
Secondary accession number(s): B3KP20
, Q2TBE9, Q5VUV9, Q5VUW0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 2, 2007
Last sequence update: October 2, 2007
Last modified: November 2, 2016
This is version 116 of the entry and version 2 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 10
    Human chromosome 10: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  5. 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.