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Protein

Lysosomal acid phosphatase

Gene

ACP2

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

Functioni

Catalytic activityi

A phosphate monoester + H2O = an alcohol + phosphate.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei42NucleophileBy similarity1
Active sitei287Proton donorBy similarity1

GO - Molecular functioni

  • acid phosphatase activity Source: ProtInc

GO - Biological processi

Keywordsi

Molecular functionHydrolase

Names & Taxonomyi

Protein namesi
Recommended name:
Lysosomal acid phosphatase (EC:3.1.3.2)
Short name:
LAP
Gene namesi
Name:ACP2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 11

Organism-specific databases

EuPathDBiHostDB:ENSG00000134575.9
HGNCiHGNC:123 ACP2
MIMi171650 gene
neXtProtiNX_P11117

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini31 – 380LumenalSequence analysisAdd BLAST350
Transmembranei381 – 401HelicalSequence analysisAdd BLAST21
Topological domaini402 – 423CytoplasmicSequence analysisAdd BLAST22

Keywords - Cellular componenti

Lysosome, Membrane

Pathology & Biotechi

Involvement in diseasei

Lysosomal acid phosphatase has been shown to be deficient in cultured fibroblasts from patients manifesting intermittent vomiting, hypotonia, lethargy, opisthotonos, terminal bleeding and death in early infancy.1 Publication

Organism-specific databases

DisGeNETi53
MalaCardsiACP2
OpenTargetsiENSG00000134575
Orphaneti35121 Acid phosphatase deficiency
PharmGKBiPA24447

Polymorphism and mutation databases

BioMutaiACP2
DMDMi115502439

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 30Add BLAST30
ChainiPRO_000002396031 – 423Lysosomal acid phosphataseAdd BLAST393

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi92N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi133N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi159 ↔ 370By similarity
Glycosylationi167N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi177N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi191N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi212 ↔ 310By similarity
Glycosylationi267N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi322N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi331N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi345 ↔ 349By similarity

Post-translational modificationi

The membrane-bound form is converted to the soluble form by sequential proteolytic processing. First, the C-terminal cytoplasmic tail is removed. Cleavage by a lysosomal protease releases the soluble form in the lysosome lumen.2 Publications
N-glycosylated. The intermediates formed during enzymatic deglycosylation suggest that all eight predicted N-glycosylation sites are used.2 Publications

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

EPDiP11117
MaxQBiP11117
PaxDbiP11117
PeptideAtlasiP11117
PRIDEiP11117
ProteomicsDBi52693
TopDownProteomicsiP11117-1 [P11117-1]

PTM databases

DEPODiP11117
iPTMnetiP11117
PhosphoSitePlusiP11117

Expressioni

Gene expression databases

BgeeiENSG00000134575
CleanExiHS_ACP2
ExpressionAtlasiP11117 baseline and differential
GenevisibleiP11117 HS

Interactioni

Protein-protein interaction databases

BioGridi106569, 38 interactors
ELMiP11117
IntActiP11117, 2 interactors
STRINGi9606.ENSP00000256997

Structurei

3D structure databases

ProteinModelPortaliP11117
SMRiP11117
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the histidine acid phosphatase family.Curated

Keywords - Domaini

Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3720 Eukaryota
ENOG410ZVBQ LUCA
GeneTreeiENSGT00530000062956
HOGENOMiHOG000231439
HOVERGENiHBG002203
InParanoidiP11117
KOiK14410
OMAiFLHLTEP
OrthoDBiEOG091G09FA
PhylomeDBiP11117
TreeFamiTF312893

Family and domain databases

CDDicd07061 HP_HAP_like, 1 hit
Gene3Di3.40.50.1240, 1 hit
InterProiView protein in InterPro
IPR033379 Acid_Pase_AS
IPR000560 His_Pase_clade-2
IPR029033 His_PPase_superfam
PfamiView protein in Pfam
PF00328 His_Phos_2, 1 hit
SUPFAMiSSF53254 SSF53254, 1 hit
PROSITEiView protein in PROSITE
PS00616 HIS_ACID_PHOSPHAT_1, 1 hit
PS00778 HIS_ACID_PHOSPHAT_2, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

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

Isoform 1 (identifier: P11117-1) [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
MAGKRSGWSR AALLQLLLGV NLVVMPPTRA RSLRFVTLLY RHGDRSPVKT
60 70 80 90 100
YPKDPYQEEE WPQGFGQLTK EGMLQHWELG QALRQRYHGF LNTSYHRQEV
110 120 130 140 150
YVRSTDFDRT LMSAEANLAG LFPPNGMQRF NPNISWQPIP VHTVPITEDR
160 170 180 190 200
LLKFPLGPCP RYEQLQNETR QTPEYQNESS RNAQFLDMVA NETGLTDLTL
210 220 230 240 250
ETVWNVYDTL FCEQTHGLRL PPWASPQTMQ RLSRLKDFSF RFLFGIYQQA
260 270 280 290 300
EKARLQGGVL LAQIRKNLTL MATTSQLPKL LVYSAHDTTL VALQMALDVY
310 320 330 340 350
NGEQAPYASC HIFELYQEDS GNFSVEMYFR NESDKAPWPL SLPGCPHRCP
360 370 380 390 400
LQDFLRLTEP VVPKDWQQEC QLASGPADTE VIVALAVCGS ILFLLIVLLL
410 420
TVLFRMQAQP PGYRHVADGE DHA
Length:423
Mass (Da):48,344
Last modified:October 3, 2006 - v3
Checksum:i3431A30B83A1E2B4
GO
Isoform 2 (identifier: P11117-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     151-160: LLKFPLGPCP → VRVASPSLGW
     161-423: Missing.

Note: No experimental confirmation available.
Show »
Length:160
Mass (Da):18,417
Checksum:i18EA6F3F31278714
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02780129R → Q2 PublicationsCorresponds to variant dbSNP:rs2167079Ensembl.1
Natural variantiVAR_034394320S → F. Corresponds to variant dbSNP:rs34425282Ensembl.1
Natural variantiVAR_050519402V → I. Corresponds to variant dbSNP:rs4647764Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_045629151 – 160LLKFPLGPCP → VRVASPSLGW in isoform 2. 1 Publication10
Alternative sequenceiVSP_045630161 – 423Missing in isoform 2. 1 PublicationAdd BLAST263

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X12548 mRNA Translation: CAA31064.1
X15525
, X15526, X15527, X15528, X15529, X15530, X15531, X15532, X15533, X15534, X15535 Genomic DNA Translation: CAA33542.1
DA382854 mRNA No translation available.
AC018410 Genomic DNA No translation available.
BC003160 mRNA Translation: AAH03160.1
BC093010 mRNA Translation: AAH93010.1
CCDSiCCDS7928.1 [P11117-1]
PIRiS06167
RefSeqiNP_001289418.1, NM_001302489.1
NP_001289419.1, NM_001302490.1
NP_001289420.1, NM_001302491.1
NP_001289421.1, NM_001302492.1
NP_001601.1, NM_001610.3 [P11117-1]
UniGeneiHs.532492

Genome annotation databases

EnsembliENST00000256997; ENSP00000256997; ENSG00000134575 [P11117-1]
GeneIDi53
KEGGihsa:53
UCSCiuc001nei.3 human [P11117-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiPPAL_HUMAN
AccessioniPrimary (citable) accession number: P11117
Secondary accession number(s): E9PCI1, Q561W5, Q9BTU7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: October 3, 2006
Last modified: June 20, 2018
This is version 170 of the entry and version 3 of the sequence. See 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, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 11
    Human chromosome 11: 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. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

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