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Protein

Lipase member H

Gene

LIPH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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
  • phosphatidic acid biosynthetic process Source: Reactome

Keywordsi

Molecular functionHeparin-binding, Hydrolase
Biological processLipid degradation, Lipid metabolism

Enzyme and pathway databases

BRENDAi3.1.1.32 2681
ReactomeiR-HSA-1483166 Synthesis of PA
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

Organism-specific databases

EuPathDBiHostDB:ENSG00000163898.9
HGNCiHGNC:18483 LIPH
MIMi607365 gene
neXtProtiNX_Q8WWY8

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

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_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

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...) asparagineSequence analysis1
Glycosylationi66N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi233 ↔ 246By similarity
Disulfide bondi270 ↔ 281By similarity
Disulfide bondi284 ↔ 292By similarity
Glycosylationi357N-linked (GlcNAc...) asparagineSequence 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
SMRiQ8WWY8
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
OMAiSFHSAVV
OrthoDBiEOG091G0680
PhylomeDBiQ8WWY8
TreeFamiTF324997

Family and domain databases

CDDicd00707 Pancreat_lipase_like, 1 hit
Gene3Di3.40.50.1820, 1 hit
InterProiView protein in InterPro
IPR029058 AB_hydrolase
IPR013818 Lipase/vitellogenin
IPR016272 Lipase_LIPH
IPR033906 Lipase_N
IPR000734 TAG_lipase
PANTHERiPTHR11610 PTHR11610, 1 hit
PfamiView protein in Pfam
PF00151 Lipase, 1 hit
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 dbSNP:rs267607219EnsemblClinVar.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

Similar proteinsi

Entry informationi

Entry nameiLIPH_HUMAN
AccessioniPrimary (citable) accession number: Q8WWY8
Secondary accession number(s): A2IBA7, Q8TEC7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 23, 2007
Last sequence update: March 1, 2002
Last modified: February 28, 2018
This is version 126 of the entry and version 1 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, 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

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