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UniProtKB - P11117 (PPAL_HUMAN)
Protein
Lysosomal acid phosphatase
Gene
ACP2
Organism
Homo sapiens (Human)
Status
Functioni
Catalytic activityi
- EC:3.1.3.2
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Active sitei | 42 | NucleophileBy similarity | 1 | |
Active sitei | 287 | Proton donorBy similarity | 1 |
GO - Molecular functioni
- acid phosphatase activity Source: GO_Central
- phosphatase activity Source: GO_Central
GO - Biological processi
- dephosphorylation Source: GO_Central
- lysosome organization Source: GO_Central
Keywordsi
Molecular function | Hydrolase |
Enzyme and pathway databases
PathwayCommonsi | P11117 |
SignaLinki | P11117 |
Names & Taxonomyi
Protein namesi | |
Gene namesi | Name:ACP2 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:123, ACP2 |
MIMi | 171650, gene |
neXtProti | NX_P11117 |
VEuPathDBi | HostDB:ENSG00000134575 |
Subcellular locationi
Lysosome
- Lysosome membrane 2 Publications; Single-pass membrane protein Sequence analysis; Lumenal side 2 Publications
- Lysosome lumen
Note: The soluble form arises by proteolytic processing of the membrane-bound form.2 Publications
Extracellular region or secreted
- extracellular exosome Source: UniProtKB
Lysosome
- lysosomal lumen Source: UniProtKB-SubCell
- lysosomal membrane Source: UniProtKB-SubCell
- lysosome Source: MGI
Other locations
- integral component of membrane Source: ProtInc
- membrane Source: UniProtKB
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 31 – 380 | LumenalSequence analysisAdd BLAST | 350 | |
Transmembranei | 381 – 401 | HelicalSequence analysisAdd BLAST | 21 | |
Topological domaini | 402 – 423 | CytoplasmicSequence analysisAdd BLAST | 22 |
Keywords - Cellular componenti
Lysosome, MembranePathology & 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
DisGeNETi | 53 |
MalaCardsi | ACP2 |
NIAGADSi | ENSG00000134575 |
OpenTargetsi | ENSG00000134575 |
PharmGKBi | PA24447 |
Miscellaneous databases
Pharosi | P11117, Tbio |
Genetic variation databases
BioMutai | ACP2 |
DMDMi | 115502439 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 30 | Add BLAST | 30 | |
ChainiPRO_0000023960 | 31 – 423 | Lysosomal acid phosphataseAdd BLAST | 393 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Glycosylationi | 92 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Glycosylationi | 133 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Disulfide bondi | 159 ↔ 370 | By similarity | ||
Glycosylationi | 167 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Glycosylationi | 177 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Glycosylationi | 191 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Disulfide bondi | 212 ↔ 310 | By similarity | ||
Glycosylationi | 267 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Glycosylationi | 322 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Glycosylationi | 331 | N-linked (GlcNAc...) asparagine1 Publication | 1 | |
Disulfide bondi | 345 ↔ 349 | By 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, GlycoproteinProteomic databases
CPTACi | CPTAC-1309 |
EPDi | P11117 |
jPOSTi | P11117 |
MassIVEi | P11117 |
MaxQBi | P11117 |
PaxDbi | P11117 |
PeptideAtlasi | P11117 |
PRIDEi | P11117 |
ProteomicsDBi | 19451 52693 [P11117-1] |
TopDownProteomicsi | P11117-1 [P11117-1] |
PTM databases
DEPODi | ACP2 |
GlyConnecti | 1476, 13 N-Linked glycans (5 sites), 1 O-Linked glycan (1 site) |
GlyGeni | P11117, 9 sites, 13 N-linked glycans (5 sites), 1 O-linked glycan (1 site) |
iPTMneti | P11117 |
PhosphoSitePlusi | P11117 |
Expressioni
Gene expression databases
Bgeei | ENSG00000134575, Expressed in right lobe of liver and 233 other tissues |
ExpressionAtlasi | P11117, baseline and differential |
Genevisiblei | P11117, HS |
Organism-specific databases
HPAi | ENSG00000134575, Low tissue specificity |
Interactioni
Binary interactionsi
P11117
With | #Exp. | IntAct |
---|---|---|
ATN1 [Q86V38] | 3 | EBI-2907070,EBI-11954292 |
GAB2 [Q9UQC2] | 3 | EBI-2907070,EBI-975200 |
KLK6 [Q92876] | 3 | EBI-2907070,EBI-2432309 |
PAK1 [Q13153] | 3 | EBI-2907070,EBI-1307 |
Protein-protein interaction databases
BioGRIDi | 106569, 71 interactors |
ELMi | P11117 |
IntActi | P11117, 39 interactors |
STRINGi | 9606.ENSP00000256997 |
Miscellaneous databases
RNActi | P11117, protein |
Family & Domainsi
Sequence similaritiesi
Belongs to the histidine acid phosphatase family.Curated
Keywords - Domaini
Signal, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG3720, Eukaryota |
GeneTreei | ENSGT00940000158446 |
InParanoidi | P11117 |
OMAi | QESDWPQ |
OrthoDBi | 1221585at2759 |
PhylomeDBi | P11117 |
TreeFami | TF312893 |
Family and domain databases
CDDi | cd07061, HP_HAP_like, 1 hit |
Gene3Di | 3.40.50.1240, 1 hit |
InterProi | View protein in InterPro IPR033379, Acid_Pase_AS IPR000560, His_Pase_clade-2 IPR029033, His_PPase_superfam |
Pfami | View protein in Pfam PF00328, His_Phos_2, 1 hit |
SUPFAMi | SSF53254, SSF53254, 1 hit |
PROSITEi | View protein in PROSITE PS00616, HIS_ACID_PHOSPHAT_1, 1 hit PS00778, HIS_ACID_PHOSPHAT_2, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 8 potential isoforms that are computationally mapped.Show allAlign All
Isoform 1 (identifier: P11117-1) [UniParc]FASTAAdd to basket
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
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
Computationally mapped potential isoform sequencesi
There are 8 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketA0A5F9ZHR7 | A0A5F9ZHR7_HUMAN | Lysosomal acid phosphatase | ACP2 | 434 | Annotation score: | ||
E9PHY0 | E9PHY0_HUMAN | Lysosomal acid phosphatase | ACP2 | 360 | Annotation score: | ||
B7Z7D2 | B7Z7D2_HUMAN | Lysosomal acid phosphatase | ACP2 | 391 | Annotation score: | ||
E9PQY3 | E9PQY3_HUMAN | Lysosomal acid phosphatase | ACP2 | 395 | Annotation score: | ||
B7Z6U3 | B7Z6U3_HUMAN | Lysosomal acid phosphatase | ACP2 | 162 | Annotation score: | ||
A0A5F9ZH40 | A0A5F9ZH40_HUMAN | Lysosomal acid phosphatase | ACP2 | 160 | Annotation score: | ||
A0A5F9ZH82 | A0A5F9ZH82_HUMAN | Lysosomal acid phosphatase | ACP2 | 305 | Annotation score: | ||
A0A5F9ZI20 | A0A5F9ZI20_HUMAN | Lysosomal acid phosphatase | ACP2 | 151 | Annotation score: |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_027801 | 29 | R → Q2 PublicationsCorresponds to variant dbSNP:rs2167079EnsemblClinVar. | 1 | |
Natural variantiVAR_034394 | 320 | S → F. Corresponds to variant dbSNP:rs34425282Ensembl. | 1 | |
Natural variantiVAR_050519 | 402 | V → I. Corresponds to variant dbSNP:rs4647764Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_045629 | 151 – 160 | LLKFPLGPCP → VRVASPSLGW in isoform 2. 1 Publication | 10 | |
Alternative sequenceiVSP_045630 | 161 – 423 | Missing in isoform 2. 1 PublicationAdd BLAST | 263 |
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 |
CCDSi | CCDS7928.1 [P11117-1] |
PIRi | S06167 |
RefSeqi | NP_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] |
Genome annotation databases
Ensembli | ENST00000256997; ENSP00000256997; ENSG00000134575 ENST00000672073; ENSP00000500291; ENSG00000134575 |
GeneIDi | 53 |
KEGGi | hsa:53 |
MANE-Selecti | ENST00000672073.1; ENSP00000500291.1; NM_001610.4; NP_001601.1 |
UCSCi | uc001nei.3, human [P11117-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
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 |
CCDSi | CCDS7928.1 [P11117-1] |
PIRi | S06167 |
RefSeqi | NP_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] |
3D structure databases
BMRBi | P11117 |
SMRi | P11117 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 106569, 71 interactors |
ELMi | P11117 |
IntActi | P11117, 39 interactors |
STRINGi | 9606.ENSP00000256997 |
PTM databases
DEPODi | ACP2 |
GlyConnecti | 1476, 13 N-Linked glycans (5 sites), 1 O-Linked glycan (1 site) |
GlyGeni | P11117, 9 sites, 13 N-linked glycans (5 sites), 1 O-linked glycan (1 site) |
iPTMneti | P11117 |
PhosphoSitePlusi | P11117 |
Genetic variation databases
BioMutai | ACP2 |
DMDMi | 115502439 |
Proteomic databases
CPTACi | CPTAC-1309 |
EPDi | P11117 |
jPOSTi | P11117 |
MassIVEi | P11117 |
MaxQBi | P11117 |
PaxDbi | P11117 |
PeptideAtlasi | P11117 |
PRIDEi | P11117 |
ProteomicsDBi | 19451 52693 [P11117-1] |
TopDownProteomicsi | P11117-1 [P11117-1] |
Protocols and materials databases
Antibodypediai | 26620, 109 antibodies from 24 providers |
DNASUi | 53 |
Genome annotation databases
Ensembli | ENST00000256997; ENSP00000256997; ENSG00000134575 ENST00000672073; ENSP00000500291; ENSG00000134575 |
GeneIDi | 53 |
KEGGi | hsa:53 |
MANE-Selecti | ENST00000672073.1; ENSP00000500291.1; NM_001610.4; NP_001601.1 |
UCSCi | uc001nei.3, human [P11117-1] |
Organism-specific databases
CTDi | 53 |
DisGeNETi | 53 |
GeneCardsi | ACP2 |
HGNCi | HGNC:123, ACP2 |
HPAi | ENSG00000134575, Low tissue specificity |
MalaCardsi | ACP2 |
MIMi | 171650, gene |
neXtProti | NX_P11117 |
NIAGADSi | ENSG00000134575 |
OpenTargetsi | ENSG00000134575 |
PharmGKBi | PA24447 |
VEuPathDBi | HostDB:ENSG00000134575 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG3720, Eukaryota |
GeneTreei | ENSGT00940000158446 |
InParanoidi | P11117 |
OMAi | QESDWPQ |
OrthoDBi | 1221585at2759 |
PhylomeDBi | P11117 |
TreeFami | TF312893 |
Enzyme and pathway databases
PathwayCommonsi | P11117 |
SignaLinki | P11117 |
Miscellaneous databases
BioGRID-ORCSi | 53, 7 hits in 1042 CRISPR screens |
ChiTaRSi | ACP2, human |
GeneWikii | ACP2 |
GenomeRNAii | 53 |
Pharosi | P11117, Tbio |
PROi | PR:P11117 |
RNActi | P11117, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000134575, Expressed in right lobe of liver and 233 other tissues |
ExpressionAtlasi | P11117, baseline and differential |
Genevisiblei | P11117, HS |
Family and domain databases
CDDi | cd07061, HP_HAP_like, 1 hit |
Gene3Di | 3.40.50.1240, 1 hit |
InterProi | View protein in InterPro IPR033379, Acid_Pase_AS IPR000560, His_Pase_clade-2 IPR029033, His_PPase_superfam |
Pfami | View protein in Pfam PF00328, His_Phos_2, 1 hit |
SUPFAMi | SSF53254, SSF53254, 1 hit |
PROSITEi | View protein in PROSITE PS00616, HIS_ACID_PHOSPHAT_1, 1 hit PS00778, HIS_ACID_PHOSPHAT_2, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | PPAL_HUMAN | |
Accessioni | P11117Primary (citable) accession number: P11117 Secondary accession number(s): E9PCI1, Q561W5, Q9BTU7 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | July 1, 1989 |
Last sequence update: | October 3, 2006 | |
Last modified: | February 23, 2022 | |
This is version 192 of the entry and version 3 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Direct protein sequencing, Reference proteomeDocuments
- Human chromosome 11
Human chromosome 11: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families