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PNLIPRP1

gene
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Also known as PLRP1

Summary

PNLIPRP1 (pancreatic lipase related protein 1, HGNC:9156) is a protein-coding gene on chromosome 10q25.3, encoding Inactive pancreatic lipase-related protein 1 (P54315). May function as inhibitor of dietary triglyceride digestion.

Predicted to enable calcium ion binding activity; lipoprotein lipase activity; and phospholipase A1 activity. Predicted to be involved in several processes, including cholesterol homeostasis; high-density lipoprotein particle remodeling; and triglyceride catabolic process. Predicted to be located in extracellular region. Biomarker of prostate cancer.

Source: NCBI Gene 5407 — RefSeq curated summary.

At a glance

  • GWAS associations: 6
  • Clinical variants (ClinVar): 68 total
  • MANE Select transcript: NM_006229

Identifiers

Gene identifiers

FieldValue
HGNC IDHGNC:9156
Approved symbolPNLIPRP1
Namepancreatic lipase related protein 1
Location10q25.3
Locus typegene with protein product
StatusApproved
AliasesPLRP1
Ensembl geneENSG00000187021
Ensembl biotypeprotein_coding
OMIM604422
Entrez5407

Gene structure

Transcript identifiers

Ensembl transcripts: 21 — 9 retained_intron, 7 protein_coding, 3 protein_coding_CDS_not_defined, 2 nonsense_mediated_decay

ENST00000358834, ENST00000470678, ENST00000471549, ENST00000480870, ENST00000482159, ENST00000482833, ENST00000484402, ENST00000497792, ENST00000510125, ENST00000525157, ENST00000525820, ENST00000526223, ENST00000527980, ENST00000528052, ENST00000529584, ENST00000530319, ENST00000530626, ENST00000531825, ENST00000531984, ENST00000534513, ENST00000534537

RefSeq mRNA: 2 — MANE Select: NM_006229 NM_001303135, NM_006229

CCDS: CCDS7595

Canonical transcript exons

ENST00000358834 — 13 exons

ExonStartEnd
ENSE00001097300116594730116594864
ENSE00001832518116590959116590995
ENSE00003474330116591771116591925
ENSE00003486482116601072116601201
ENSE00003497945116597828116597947
ENSE00003514180116605386116605553
ENSE00003519418116609053116609175
ENSE00003603124116600047116600165
ENSE00003611781116592416116592541
ENSE00003643156116598047116598166
ENSE00003662767116604030116604138
ENSE00003758567116591130116591178
ENSE00003787069116596214116596322

Expression profiles

Bgee: expression breadth ubiquitous, 153 present calls, max score 99.92.

FANTOM5 (CAGE): breadth tissue_specific, TPM avg 1.8555 / max 2032.7210, expressed in 6 samples.

FANTOM5 promoters (3 alternative TSS)

Promoter IDTPM avgSamples expressed
1072381.37985
1072390.31905
1072370.15674

Top tissues by expression

283 total, by Bgee expression score (0-100, higher = more expressed):

TissueAnatomy IDExpression scoreQuality
body of pancreasUBERON:000115099.92gold quality
pancreasUBERON:000126498.07gold quality
islet of LangerhansUBERON:000000696.57gold quality
epithelial cell of pancreasCL:000008380.11gold quality
male germ line stem cell (sensu Vertebrata) in testisCL:0000089 ∩ UBERON:000047379.60silver quality
buccal mucosa cellCL:000233673.77gold quality
primordial germ cell in gonadCL:0000670 ∩ UBERON:000099172.58gold quality
frontal poleUBERON:000279571.07gold quality
paraflocculusUBERON:000535170.98gold quality
middle frontal gyrusUBERON:000270270.56gold quality
endometrium epitheliumUBERON:000481169.31gold quality
right coronary arteryUBERON:000162568.99gold quality
Brodmann (1909) area 10UBERON:001354164.69gold quality
skeletal muscle tissue of rectus abdominisUBERON:000451164.31gold quality
type B pancreatic cellCL:000016964.28gold quality
CA1 field of hippocampusUBERON:000388163.74gold quality
ectocervixUBERON:001224963.42gold quality
rectumUBERON:000105263.35gold quality
triceps brachiiUBERON:000150963.23gold quality
oocyteCL:000002362.71gold quality
right lobe of liverUBERON:000111462.09gold quality
right adrenal glandUBERON:000123361.95gold quality
lower esophagus mucosaUBERON:003583461.85gold quality
endocervixUBERON:000045861.84gold quality
orbitofrontal cortexUBERON:000416761.68gold quality
left uterine tubeUBERON:000130361.36gold quality
transverse colonUBERON:000115760.98gold quality
fundus of stomachUBERON:000116060.63gold quality
right adrenal gland cortexUBERON:003582760.34gold quality
diaphragmUBERON:000110360.26gold quality

Single-cell (SCXA)

Detected in 4 experiment(s), a significant marker in 3.

ExperimentMarker?Max mean expression
E-MTAB-5061yes4369.04
E-GEOD-81547yes31.32
E-HCAD-31no3.16
E-ANND-3no0.00

Regulation

Is transcription factor: no

Literature-anchored findings (GeneRIF, showing 3)

  • Low PNLIPRP1 expression is associated with pancreatic cancer. (PMID:23918603)
  • Pancreatic lipase and its related proteins: where are we now? (PMID:38081381)
  • PNLIPRP1 Hypermethylation in Exocrine Pancreas Links Type 2 Diabetes and Cholesterol Metabolism. (PMID:39137110)

Cross-species orthologs

28 orthologs

OrganismSymbolGene ID
mus_musculusPnliprp1ENSMUSG00000042179
rattus_norvegicusPnliprp1ENSRNOG00000017908
drosophila_melanogasterYp1FBGN0004045
drosophila_melanogasterYp3FBGN0004047
drosophila_melanogasterYp2FBGN0005391
drosophila_melanogasterCG5162FBGN0030828
drosophila_melanogasterCG6847FBGN0030884
drosophila_melanogasterCG7367FBGN0031976
drosophila_melanogasterCG13282FBGN0032612
drosophila_melanogasterCG6675FBGN0032973
drosophila_melanogasterCG6472FBGN0034166
drosophila_melanogasterCG10163FBGN0035697
drosophila_melanogasterCG10116FBGN0036367
drosophila_melanogasterCG5665FBGN0036977
drosophila_melanogastersxe2FBGN0038398
drosophila_melanogasterCG4582FBGN0039344
drosophila_melanogasterCG6296FBGN0039470
drosophila_melanogasterCG6295FBGN0039471
drosophila_melanogasterCG17192FBGN0039472
drosophila_melanogasterCG17191FBGN0039473
drosophila_melanogasterCG6283FBGN0039474
drosophila_melanogasterCG6277FBGN0039475
drosophila_melanogasterCG6271FBGN0039476
drosophila_melanogasterCG34447FBGN0085476
drosophila_melanogasterCG34448FBGN0085477
drosophila_melanogasterCG14034FBGN0250847
drosophila_melanogasterCG4267FBGN0264979
drosophila_melanogasterCG18258FBGN0265267

Paralogs (9): LIPG (ENSG00000101670), PLA1A (ENSG00000144837), LIPH (ENSG00000163898), LIPC (ENSG00000166035), LPL (ENSG00000175445), PNLIP (ENSG00000175535), LIPI (ENSG00000188992), PNLIPRP3 (ENSG00000203837), PNLIPRP2 (ENSG00000266200)

Protein

Protein identifiers

Inactive pancreatic lipase-related protein 1 — P54315 (reviewed: P54315)

All UniProt accessions (8): E7EX42, E9PJC9, E9PMA6, E9PMX6, E9PR20, E9PRT6, P54315, H0YDY1

UniProt curated annotations — full annotation on UniProt →

Function. May function as inhibitor of dietary triglyceride digestion. Lacks detectable lipase activity towards triglycerides, diglycerides, phosphatidylcholine, galactolipids or cholesterol esters (in vitro).

Subcellular location. Secreted.

Tissue specificity. Pancreas.

Similarity. Belongs to the AB hydrolase superfamily. Lipase family.

Isoforms (3)

UniProt IDNamesCanonical?
P54315-11yes
P54315-22
P54315-33

RefSeq proteins (2): NP_001290064, NP_006220* (*=MANE)

Domains & families (InterPro)

IDNameType
IPR000734TAG_lipaseFamily
IPR001024PLAT/LH2_domDomain
IPR002331Lipase_pancFamily
IPR013818LipaseDomain
IPR016272Lipase_LIPHFamily
IPR029058AB_hydrolase_foldHomologous_superfamily
IPR033906Lipase_NDomain
IPR036392PLAT/LH2_dom_sfHomologous_superfamily

Pfam: PF00151, PF01477

Enzyme classification (BRENDA):

  • EC 3.1.1.26 — galactolipase (BRENDA: 63 organisms, 125 substrates, 44 inhibitors, 15 Km, 2 kcat entries)

Substrate kinetics (BRENDA)

6 substrates with measured Km, best-characterized 6. Km ranges are aggregated across organisms/conditions.

SubstrateKm (mM)Measurements
MONOGALACTOSYLDIACYLGLYCEROL0.119–0.656
PHOSPHATIDYLCHOLINE0.53–1.73
1,2-DIACYL-3-O-ALPHA-D-GALACTOSYL-(1->6)-BETA-D-0.54–1.052
DIGALACTOSYLDIACYLGLYCEROL0.311
DIGALACTOSYLDILINOLENIN7.81
MONOGALACTOSYLDILINOLENIN1.51

UniProt features (66 total): strand 23, helix 13, disulfide bond 6, sequence variant 5, turn 5, splice variant 4, binding site 4, active site 3, signal peptide 1, chain 1, domain 1

Structure

Experimental structures (PDB)

1 structures.

PDBMethodResolution (Å)
2PPLX-RAY DIFFRACTION2.2

Predicted structure (AlphaFold)

ModelpLDDTFraction very-high
AF-P54315-F194.540.88

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Catalytic / active sites (3): 171 (nucleophile); 194 (charge relay system); 281 (charge relay system)

Ligand- & substrate-binding residues (4): 205; 208; 210; 213

Disulfide bonds (6): 21–27, 109–120, 255–279, 303–314, 317–322, 451–467

Function

Pathways and Gene Ontology

Reactome pathways

3 pathways

IDPathway
R-HSA-192456Digestion of dietary lipid
R-HSA-8935690Digestion
R-HSA-8963743Digestion and absorption

MSigDB gene sets: 120 (showing top): YAGI_AML_WITH_INV_16_TRANSLOCATION, GOBP_STEROL_HOMEOSTASIS, GOBP_MONOCARBOXYLIC_ACID_METABOLIC_PROCESS, LEE_LIVER_CANCER_CIPROFIBRATE_DN, GOBP_ORGANIC_ACID_BIOSYNTHETIC_PROCESS, GOBP_LIPID_HOMEOSTASIS, GOBP_GLYCEROLIPID_METABOLIC_PROCESS, GOBP_SMALL_MOLECULE_BIOSYNTHETIC_PROCESS, GATA6_01, GOBP_NEUTRAL_LIPID_CATABOLIC_PROCESS, GOBP_PROTEIN_LIPID_COMPLEX_ORGANIZATION, SCHAEFFER_PROSTATE_DEVELOPMENT_48HR_DN, GOBP_FATTY_ACID_BIOSYNTHETIC_PROCESS, GATA1_04, GOBP_MONOCARBOXYLIC_ACID_BIOSYNTHETIC_PROCESS

GO Biological Process (6): fatty acid biosynthetic process (GO:0006633), triglyceride catabolic process (GO:0019433), high-density lipoprotein particle remodeling (GO:0034375), cholesterol homeostasis (GO:0042632), lipid metabolic process (GO:0006629), lipid catabolic process (GO:0016042)

GO Molecular Function (8): lipoprotein lipase activity (GO:0004465), triacylglycerol lipase activity (GO:0004806), calcium ion binding (GO:0005509), glycerophospholipid phospholipase A1 activity (GO:0008970), protein binding (GO:0005515), lipase activity (GO:0016298), metal ion binding (GO:0046872), carboxylic ester hydrolase activity (GO:0052689)

GO Cellular Component (1): extracellular region (GO:0005576)

Reactome top-level categories

Rollup of top-2 pathways:

CategoryPathways
Digestion1
Digestion and absorption1

GO top-level categories

Rollup of top GO terms by namespace:

CategoryTerms
hydrolase activity, acting on ester bonds2
fatty acid metabolic process1
lipid biosynthetic process1
monocarboxylic acid biosynthetic process1
triglyceride metabolic process1
acylglycerol catabolic process1
plasma lipoprotein particle remodeling1
sterol homeostasis1
primary metabolic process1
lipid metabolic process1
catabolic process1
triacylglycerol lipase activity1
lipase activity1
carboxylic ester hydrolase activity1
metal ion binding1
A1-type glycerophospholipase activity1
binding1
cation binding1
cellular anatomical structure1

Protein interactions and networks

STRING

622 interactions, top by confidence (×1000):

Protein AProtein BPartner UniProtScore
PNLIPRP1CLPSP04118959
PNLIPRP1LIPFP07098920
PNLIPRP1CELP19835696
PNLIPRP1PLA2G1BP04054606
PNLIPRP1SYCNQ0VAF6582
PNLIPRP1CPA1P15085544
PNLIPRP1CELA2AP08217541
PNLIPRP1CELA3BP08861507
PNLIPRP1CPA2P48052470
PNLIPRP1MBOAT1Q6ZNC8460
PNLIPRP1CPB1P15086430
PNLIPRP1CELA3AP09093420
PNLIPRP1HSPA12AO43301418
PNLIPRP1SHTN1A0MZ66411
PNLIPRP1PNLIPP16233382

IntAct

127 interactions, top by confidence:

ABTypeScore
PNLIPRP1TMX2psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1CERS4psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1HSD17B13psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1GJA8psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1TSPAN8psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1SLC18A1psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1MEOX2psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1STX1Apsi-mi:“MI:0915”(physical association)0.560
PNLIPRP1TMEM237psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1GDAP1psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1psi-mi:“MI:0915”(physical association)0.560
FFAR2PNLIPRP1psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1SLC10A6psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1TMEM14Bpsi-mi:“MI:0915”(physical association)0.560
NEMP1PNLIPRP1psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1NDUFAF1psi-mi:“MI:0915”(physical association)0.560
AQP2PNLIPRP1psi-mi:“MI:0915”(physical association)0.560
PNLIPRP1FAM209Apsi-mi:“MI:0915”(physical association)0.560
SLC7A1PNLIPRP1psi-mi:“MI:0915”(physical association)0.560
PEX12PNLIPRP1psi-mi:“MI:0915”(physical association)0.560

BioGRID (60): PNLIPRP1 (Two-hybrid), ITGA8 (Affinity Capture-MS), TMEM2 (Affinity Capture-MS), NPTX1 (Affinity Capture-MS), TMEM132A (Affinity Capture-MS), GPR98 (Affinity Capture-MS), CACNA2D2 (Affinity Capture-MS), UBR1 (Affinity Capture-MS), UBR2 (Affinity Capture-MS), PNLIPRP1 (Two-hybrid), PNLIPRP1 (Two-hybrid), PNLIPRP1 (Two-hybrid), PNLIPRP1 (Two-hybrid), PNLIPRP1 (Two-hybrid), PNLIPRP1 (Two-hybrid)

ESM2 similar proteins: A0A0M3KKW3, A2VBC4, A5PK46, A8PUY1, B2D0J5, C0HLL3, D7EZN2, O88354, P00591, P06857, P0CH47, P0CH86, P0CH87, P0DMB4, P0DMB5, P0DPT0, P0DSI2, P16233, P17892, P27657, P29183, P35501, P35502, P49369, P50903, P51528, P53357, P54315, P54316, P54317, P54318, P81139, P86100, P9WEM6, Q02157, Q04456, Q06478, Q17RR3, Q3ZU95, Q5BKQ4

Diamond homologs: A0A0M3KKW3, A2VBC4, C0HLL3, O46559, P06857, P07867, P0CH47, P0CH86, P0CH87, P0DMB4, P0DMB5, P0DMB7, P0DMB8, P0DPT0, P0DSI2, P11150, P11602, P27656, P29183, P49369, P51528, P53357, P54315, P54316, P81139, Q02157, Q06478, Q3SZ79, Q3ZU95, Q5BKQ4, Q5XGE9, Q68KK0, Q6NYZ4, Q6Q249, Q6Q250, Q6Q251, Q6Q252, Q6XZB0, Q7M3V3, Q7M3V4

SIGNOR signaling

0 interactions.

Disease & clinical

Clinical variants and AI predictions

ClinVar

68 variants total. Per-class counts are floors (≥ shown; pagination cap):

ClassificationCount (floor)
Pathogenic0
Likely pathogenic0
Uncertain significance64
Likely benign3
Benign0

Top pathogenic / likely-pathogenic (0)

SpliceAI

1693 predictions. Top by Δscore:

VariantEffectΔscore
10:116591926:G:GGdonor_gain1.0000
10:116594725:A:AGacceptor_gain1.0000
10:116594729:GAA:Gacceptor_gain1.0000
10:116594865:G:GGdonor_gain1.0000
10:116600162:GTCT:Gdonor_gain1.0000
10:116601182:A:Tdonor_gain1.0000
10:116601197:CGCTC:Cdonor_gain1.0000
10:116601198:GCTC:Gdonor_gain1.0000
10:116601198:GCTCG:Gdonor_gain1.0000
10:116601199:CTC:Cdonor_gain1.0000
10:116601200:TC:Tdonor_gain1.0000
10:116601200:TCGTA:Tdonor_loss1.0000
10:116601202:G:GAdonor_loss1.0000
10:116601202:G:GGdonor_gain1.0000
10:116601203:TAA:Tdonor_loss1.0000
10:116601204:AAGTT:Adonor_loss1.0000
10:116604134:TTCAG:Tdonor_loss1.0000
10:116604137:AG:Adonor_loss1.0000
10:116604138:GGT:Gdonor_loss1.0000
10:116604139:G:Cdonor_loss1.0000
10:116604140:T:Adonor_loss1.0000
10:116605552:GT:Gdonor_gain1.0000
10:116605554:G:GGdonor_gain1.0000
10:116591175:AAAGG:Adonor_loss0.9900
10:116591176:AAGGT:Adonor_loss0.9900
10:116591179:G:Tdonor_loss0.9900
10:116591180:T:Adonor_loss0.9900
10:116591925:AG:Adonor_loss0.9900
10:116591926:G:GAdonor_loss0.9900
10:116591927:TGAGA:Tdonor_loss0.9900

AlphaMissense

3095 scored. Top likely-pathogenic:

VariantProtein changeam_pathogenicity
10:116600082:A:CS284R0.994
10:116600084:C:AS284R0.994
10:116600084:C:GS284R0.994
10:116600103:A:CS291R0.992
10:116600105:C:AS291R0.992
10:116600105:C:GS291R0.992
10:116594772:T:AW125R0.991
10:116594772:T:CW125R0.991
10:116597851:T:CF200L0.991
10:116597853:C:AF200L0.991
10:116597853:C:GF200L0.991
10:116596259:A:CS171R0.990
10:116596261:C:AS171R0.990
10:116596261:C:GS171R0.990
10:116594774:G:CW125C0.989
10:116594774:G:TW125C0.989
10:116597903:T:AV217D0.987
10:116597914:C:GH221D0.987
10:116596322:G:TG192W0.986
10:116597876:G:CR208P0.986
10:116597879:T:AL209H0.986
10:116598115:T:AC255S0.986
10:116598116:G:CC255S0.986
10:116594816:C:AN139K0.985
10:116594816:C:GN139K0.985
10:116596260:G:TS171I0.985
10:116600073:C:GH281D0.985
10:116605471:T:AW420R0.985
10:116605471:T:CW420R0.985
10:116600075:C:AH281Q0.984

dbSNP variants (sampled 300 via entrez): RS1000301278 (10:116596230 C>A,T), RS1000745637 (10:116603878 G>A), RS1001500715 (10:116592135 C>G), RS1001598230 (10:116591357 G>A,T), RS1001649350 (10:116597396 A>G), RS1001982878 (10:116599004 C>G), RS1001985833 (10:116592175 T>C), RS1002035441 (10:116598817 C>A), RS1003192907 (10:116606087 G>A), RS1003267175 (10:116604565 T>C), RS1003601213 (10:116593911 C>G,T), RS1003656013 (10:116605787 C>T), RS1004058493 (10:116593717 A>G), RS1004262396 (10:116599070 C>T), RS1004314721 (10:116605240 G>A)

Disease associations

OMIM: gene MIM:604422 | disease phenotypes:

GenCC curated gene-disease

Mondo (0):

Orphanet (0):

HPO phenotypes

0 total (0 of 0 shown, HPO-id order):

GWAS associations

6 associations (top):

StudyTraitp-value
GCST002324_9Anger4.000000e-06
GCST003771_4Loneliness1.000000e-06
GCST003772_15Loneliness (linear analysis)8.000000e-07
GCST003773_9Loneliness (multivariate analysis)4.000000e-06
GCST005951_66Body mass index4.000000e-08
GCST006585_2227Blood protein levels5.000000e-09

EFO canonical traits (3, from GWAS)

EFO IDTrait name
EFO:0003015aggressive behavior
EFO:0007865loneliness measurement
EFO:0004340body mass index

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

PharmGKB: 1 entry (VIP=true, CPIC=false)

CTD chemical–gene interactions

9 total (human), top 9 by PubMed support.

ChemicalActions (top 5)PubMed papers
Benzo(a)pyrenedecreases methylation, increases expression2
Valproic Acidaffects methylation, increases methylation2
tebuconazoledecreases expression1
Resveratroldecreases expression, affects cotreatment1
Plant Extractsaffects cotreatment, decreases expression1
Silicon Dioxidedecreases expression1
Testosteronedecreases expression1
Tretinoinaffects expression1
Vitamin Edecreases expression1

Clinical trials (associated diseases)

0 trials via MONDO — disease-level, not drug-specific.

No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.

About this page

Generated deterministically by Sugi Atlas from primary biomedical databases via BioBTree. Every record on this page traces to a primary source.

Generated
Atlas version
v1.1.4
BioBTree
v2.0.2

Sources 75

This page pulls from 75 datasets indexed by BioBTree:

alphafoldalphamissenseantibodybgeebgee_evidencebindingdbbiogrid_interactionbrendabrenda_kineticsccdscellosauruschembl_activitychembl_targetciviccivic_evidenceclingen_dosageclingen_gene_validityclinical_trialsclinvarcollectrictd_gene_interactiondbsnpdepmapdiamond_similarityefoensemblentrezesm2_similarityexonfantom5_genefantom5_promotergenccgenerifgogoparentgtopdbgwasgwas_studyhgnchpointactinterprointogenjasparmeshmimmirdbmondomsigdborphanetorthologparalogpdbpfampharmgkb_clinicalpharmgkb_genepharmgkb_guidelinepharmgkb_variantpubchempubchem_activitypubchem_assaypubmedreactomereactomeparentrefseqrhearnacentralscxa_expressionscxa_gene_experimentsignorspliceaistring_interactiontranscriptufeatureuniprot