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Protein

Lipase member H

Gene

LIPH

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

Functioni

Hydrolyzes specifically phosphatidic acid (PA) to produce 2-acyl lysophosphatidic acid (LPA; a potent bioactive lipid mediator) and fatty acid. Does not hydrolyze other phospholipids, like phosphatidylserine (PS), phosphatidylcholine (PC) and phosphatidylethanolamine (PE) or triacylglycerol (TG).2 Publications

Enzyme regulationi

Inhibited by sodium vanadate.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei154Nucleophile1
Active sitei178Charge relay systemBy similarity1
Active sitei248Charge relay systemBy similarity1

GO - Molecular functioni

  • carboxylic ester hydrolase activity Source: InterPro
  • heparin binding Source: UniProtKB
  • phospholipase activity Source: UniProtKB

GO - Biological processi

  • lipid catabolic process Source: UniProtKB
  • lipid digestion Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Keywords - Biological processi

Lipid degradation, Lipid metabolism

Keywords - Ligandi

Heparin-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000163898-MONOMER.
BRENDAi3.1.1.32. 2681.
ReactomeiR-HSA-192456. Digestion of dietary lipid.
SIGNORiQ8WWY8.

Protein family/group databases

ESTHERihuman-LIPH. Phospholipase.

Chemistry databases

SwissLipidsiSLP:000000625.

Names & Taxonomyi

Protein namesi
Recommended name:
Lipase member H (EC:3.1.1.-)
Short name:
LIPH
Alternative name(s):
LPD lipase-related protein
Membrane-associated phosphatidic acid-selective phospholipase A1-alpha
Short name:
mPA-PLA1 alpha
Phospholipase A1 member B
Gene namesi
Name:LIPH
Synonyms:LPDLR, MPAPLA1, PLA1B
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

HGNCiHGNC:18483. LIPH.

Subcellular locationi

GO - Cellular componenti

  • extracellular space Source: UniProtKB
  • plasma membrane Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Secreted

Pathology & Biotechi

Involvement in diseasei

Hypotrichosis 7 (HYPT7)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA condition characterized by the presence of less than the normal amount of hair. Affected individuals have sparse or absent scalp, axillary and body hair and sparse eyebrows and eyelashes.
See also OMIM:604379
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_059050108W → R in HYPT7 and ARWH2. 2 PublicationsCorresponds to variant rs267607219dbSNPEnsembl.1
Natural variantiVAR_030125172 – 205Missing in HYPT7. 1 PublicationAdd BLAST34
Woolly hair autosomal recessive 2 (ARWH2)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA hair shaft disorder characterized by fine and tightly curled hair. Compared to normal curly hair that is observed in some populations, woolly hair grows slowly and stops growing after a few inches. Under light microscopy, woolly hair shows some structural anomalies, including trichorrhexis nodosa and tapered ends. Some individuals may present with hypotrichosis.
See also OMIM:604379
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_059050108W → R in HYPT7 and ARWH2. 2 PublicationsCorresponds to variant rs267607219dbSNPEnsembl.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi154S → A: Loss of lipase activity. 1 Publication1

Keywords - Diseasei

Disease mutation, Hypotrichosis

Organism-specific databases

DisGeNETi200879.
MalaCardsiLIPH.
MIMi604379. phenotype.
OpenTargetsiENSG00000163898.
Orphaneti55654. Hypotrichosis simplex.
170. Woolly hair.
PharmGKBiPA134934352.

Polymorphism and mutation databases

BioMutaiLIPH.
DMDMi74762634.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 18Sequence analysisAdd BLAST18
ChainiPRO_000027332119 – 451Lipase member HAdd BLAST433

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi50N-linked (GlcNAc...)Sequence analysis1
Glycosylationi66N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi233 ↔ 246By similarity
Disulfide bondi270 ↔ 281By similarity
Disulfide bondi284 ↔ 292By similarity
Glycosylationi357N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi427 ↔ 446By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiQ8WWY8.
PeptideAtlasiQ8WWY8.
PRIDEiQ8WWY8.

PTM databases

iPTMnetiQ8WWY8.
PhosphoSitePlusiQ8WWY8.

Expressioni

Tissue specificityi

Present in intestine (at protein level). Expressed in colon, prostate, kidney, pancreas, ovary, testis, intestine, lung and pancreas. Expressed at lower level in brain, spleen and heart. In hair, it is prominently expressed in hair follicles, including the stem cell-rich bulge region.4 Publications

Gene expression databases

BgeeiENSG00000163898.
CleanExiHS_LIPH.
ExpressionAtlasiQ8WWY8. baseline and differential.
GenevisibleiQ8WWY8. HS.

Organism-specific databases

HPAiHPA049079.

Interactioni

Protein-protein interaction databases

BioGridi128352. 52 interactors.
STRINGi9606.ENSP00000296252.

Structurei

3D structure databases

ProteinModelPortaliQ8WWY8.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the AB hydrolase superfamily. Lipase family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiENOG410IKYJ. Eukaryota.
ENOG4111AHY. LUCA.
GeneTreeiENSGT00760000119069.
HOGENOMiHOG000234386.
HOVERGENiHBG080640.
InParanoidiQ8WWY8.
KOiK19404.
OMAiITAYPCD.
OrthoDBiEOG091G0680.
PhylomeDBiQ8WWY8.
TreeFamiTF324997.

Family and domain databases

CDDicd00707. Pancreat_lipase_like. 1 hit.
Gene3Di3.40.50.1820. 1 hit.
InterProiIPR029058. AB_hydrolase.
IPR013818. Lipase/vitellogenin.
IPR016272. Lipase_LIPH.
IPR033906. Lipase_N.
IPR000734. TAG_lipase.
[Graphical view]
PANTHERiPTHR11610. PTHR11610. 1 hit.
PfamiPF00151. Lipase. 1 hit.
[Graphical view]
PIRSFiPIRSF000865. Lipoprotein_lipase_LIPH. 1 hit.
PRINTSiPR00821. TAGLIPASE.
SUPFAMiSSF53474. SSF53474. 1 hit.

Sequencei

Sequence statusi: Complete.

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

Q8WWY8-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLRFYLFISL LCLSRSDAEE TCPSFTRLSF HSAVVGTGLN VRLMLYTRKN
60 70 80 90 100
LTCAQTINSS AFGNLNVTKK TTFIVHGFRP TGSPPVWMDD LVKGLLSVED
110 120 130 140 150
MNVVVVDWNR GATTLIYTHA SSKTRKVAMV LKEFIDQMLA EGASLDDIYM
160 170 180 190 200
IGVSLGAHIS GFVGEMYDGW LGRITGLDPA GPLFNGKPHQ DRLDPSDAQF
210 220 230 240 250
VDVIHSDTDA LGYKEPLGNI DFYPNGGLDQ PGCPKTILGG FQYFKCDHQR
260 270 280 290 300
SVYLYLSSLR ESCTITAYPC DSYQDYRNGK CVSCGTSQKE SCPLLGYYAD
310 320 330 340 350
NWKDHLRGKD PPMTKAFFDT AEESPFCMYH YFVDIITWNK NVRRGDITIK
360 370 380 390 400
LRDKAGNTTE SKINHEPTTF QKYHQVSLLA RFNQDLDKVA AISLMFSTGS
410 420 430 440 450
LIGPRYKLRI LRMKLRSLAH PERPQLCRYD LVLMENVETV FQPILCPELQ

L
Length:451
Mass (Da):50,859
Last modified:March 1, 2002 - v1
Checksum:i949CE32B0C15868B
GO

Sequence cautioni

The sequence BAB85023 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_059050108W → R in HYPT7 and ARWH2. 2 PublicationsCorresponds to variant rs267607219dbSNPEnsembl.1
Natural variantiVAR_030125172 – 205Missing in HYPT7. 1 PublicationAdd BLAST34

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY036912 mRNA. Translation: AAK63178.1.
AY093498 mRNA. Translation: AAM18803.1.
EF186229 mRNA. Translation: ABM67095.1.
AK074229 mRNA. Translation: BAB85023.1. Different initiation.
AK122651 mRNA. Translation: BAG53642.1.
CH471052 Genomic DNA. Translation: EAW78218.1.
BC064941 mRNA. Translation: AAH64941.1.
CCDSiCCDS3272.1.
RefSeqiNP_640341.1. NM_139248.2.
UniGeneiHs.68864.

Genome annotation databases

EnsembliENST00000296252; ENSP00000296252; ENSG00000163898.
GeneIDi200879.
KEGGihsa:200879.
UCSCiuc003fpm.4. human.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY036912 mRNA. Translation: AAK63178.1.
AY093498 mRNA. Translation: AAM18803.1.
EF186229 mRNA. Translation: ABM67095.1.
AK074229 mRNA. Translation: BAB85023.1. Different initiation.
AK122651 mRNA. Translation: BAG53642.1.
CH471052 Genomic DNA. Translation: EAW78218.1.
BC064941 mRNA. Translation: AAH64941.1.
CCDSiCCDS3272.1.
RefSeqiNP_640341.1. NM_139248.2.
UniGeneiHs.68864.

3D structure databases

ProteinModelPortaliQ8WWY8.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi128352. 52 interactors.
STRINGi9606.ENSP00000296252.

Chemistry databases

SwissLipidsiSLP:000000625.

Protein family/group databases

ESTHERihuman-LIPH. Phospholipase.

PTM databases

iPTMnetiQ8WWY8.
PhosphoSitePlusiQ8WWY8.

Polymorphism and mutation databases

BioMutaiLIPH.
DMDMi74762634.

Proteomic databases

PaxDbiQ8WWY8.
PeptideAtlasiQ8WWY8.
PRIDEiQ8WWY8.

Protocols and materials databases

DNASUi200879.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000296252; ENSP00000296252; ENSG00000163898.
GeneIDi200879.
KEGGihsa:200879.
UCSCiuc003fpm.4. human.

Organism-specific databases

CTDi200879.
DisGeNETi200879.
GeneCardsiLIPH.
HGNCiHGNC:18483. LIPH.
HPAiHPA049079.
MalaCardsiLIPH.
MIMi604379. phenotype.
607365. gene.
neXtProtiNX_Q8WWY8.
OpenTargetsiENSG00000163898.
Orphaneti55654. Hypotrichosis simplex.
170. Woolly hair.
PharmGKBiPA134934352.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IKYJ. Eukaryota.
ENOG4111AHY. LUCA.
GeneTreeiENSGT00760000119069.
HOGENOMiHOG000234386.
HOVERGENiHBG080640.
InParanoidiQ8WWY8.
KOiK19404.
OMAiITAYPCD.
OrthoDBiEOG091G0680.
PhylomeDBiQ8WWY8.
TreeFamiTF324997.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000163898-MONOMER.
BRENDAi3.1.1.32. 2681.
ReactomeiR-HSA-192456. Digestion of dietary lipid.
SIGNORiQ8WWY8.

Miscellaneous databases

ChiTaRSiLIPH. human.
GeneWikiiLIPH.
GenomeRNAii200879.
PROiQ8WWY8.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000163898.
CleanExiHS_LIPH.
ExpressionAtlasiQ8WWY8. baseline and differential.
GenevisibleiQ8WWY8. HS.

Family and domain databases

CDDicd00707. Pancreat_lipase_like. 1 hit.
Gene3Di3.40.50.1820. 1 hit.
InterProiIPR029058. AB_hydrolase.
IPR013818. Lipase/vitellogenin.
IPR016272. Lipase_LIPH.
IPR033906. Lipase_N.
IPR000734. TAG_lipase.
[Graphical view]
PANTHERiPTHR11610. PTHR11610. 1 hit.
PfamiPF00151. Lipase. 1 hit.
[Graphical view]
PIRSFiPIRSF000865. Lipoprotein_lipase_LIPH. 1 hit.
PRINTSiPR00821. TAGLIPASE.
SUPFAMiSSF53474. SSF53474. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiLIPH_HUMAN
AccessioniPrimary (citable) accession number: Q8WWY8
Secondary accession number(s): A2IBA7, Q8TEC7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 23, 2007
Last sequence update: March 1, 2002
Last modified: November 30, 2016
This is version 119 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. 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

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.