perianal Crohn disease

disease
On this page

Also known as perianal Crohn's disease

Summary

perianal Crohn disease (MONDO:0005537) is a disease with 2 cohort genes (2 GWAS associations across 1 studies) and 8 clinical trials.

At a glance

  • Cohort genes: 2
  • GWAS associations: 2
  • Clinical trials: 8

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

FieldValue
Canonical nameperianal Crohn disease
Mondo IDMONDO:0005537
EFOEFO:0005627
ICD-11464644598
SNOMED CT235796008
UMLSC0341395
MedGen574362
Anatomy (UBERON)UBERON:0001353
Is cancer (heuristic)no

Also known as: perianal Crohn’s disease

Data availability: 2 GWAS associations (1 study).

Disease family

Classification path: disease › human disease › disease by etiologic mechanism › disease of genetic or genomic mechanism › hereditary diseaseinflammatory bowel diseaseCrohn diseaseperianal Crohn disease

Related subtypes (4): Crohn’s colitis, oral Crohn disease, small bowel Crohn disease, Crohn disease of the esophagus

Genetics & variants

GWAS landscape

2 GWAS associations across 1 studies. Top hits map to 2 distinct genes (as reported by GWAS).

Top associations by p-value

rsIDp-valueGeneRisk alleleOdds ratio
rs121223371e-06CFAP45 - TAGLN2G2.2
rs98688982e-06PLCXD2, PHLDB2G1.49

Top studies (by case count)

StudyLead authorYearCasesControlsTitle
GCST002729Alonso A20141,0900Identification of risk loci for Crohn’s disease phenotypes using a genome-wide association study.

Variant details and genetic-evidence tiers

Tier distribution (top 50 variants)

TierVariants
Tier 1: coding0
Tier 2: splice/UTR0
Tier 3: regulatory1
Tier 4: intronic/intergenic1

MAF distribution

BucketVariants
common (>=0.05)2
low_freq (0.01-0.05)0
rare (<0.01)0
unknown0

Functional consequences

ConsequenceCount
regulatory_region_variant1
intron_variant1

Top variants

rsIDChrPosAllelesMAFConsequenceGenep-valueTier
rs121223371159916632A>C,G0.06regulatory_region_variantCFAP45 - TAGLN21e-06Tier 3: regulatory
rs98688983111762099A>G0.16intron_variantPLCXD2, PHLDB22e-06Tier 4: intronic/intergenic

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 0 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Cohort genes → proteins

2 cohort genes, 2 distinct canonical proteins.

Evidence partition

SubsetGenes
gwas_only2

Cohort genes (full)

SymbolHGNCEnsemblUniProtNameEvidence
TAGLN2HGNC:11554ENSG00000158710P37802Transgelin-2gwas
PHLDB2HGNC:29573ENSG00000144824Q86SQ0Pleckstrin homology-like domain family B member 2gwas

Cohort function summary

Lead sentence per gene, UniProt-curated.

SymbolProtein nameFunction (lead sentence)
PHLDB2Pleckstrin homology-like domain family B member 2Seems to be involved in the assembly of the postsynaptic apparatus.

Protein-family classification

Druggable: 0 · Difficult: 1 · Unknown: 1 · Druggable fraction: 0.0

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

FamilyGenesFoldFDR
Scaffold/PPI18.6×0.225
Other/Unknown10.9×0.805

Per-gene assignment

SymbolFamilyDruggable?ECInterPro (top 3)
TAGLN2Other/UnknownnoCalponin_repeat, CH_dom, SM22_calponin
PHLDB2Scaffold/PPInoPH_domain, PH-like_dom_sf, PHLDB1/2/3_PH

Expression context

Cohort genes with no expression data: 0.

2 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

BucketGenes
narrow (1-5 tissues)0
moderate (6-20)0
broad (>20)2
unknown0

Top tissues across cohort

TissueCohort genes
monocyte1
right lung1
upper lobe of left lung1
cauda epididymis1
kidney epithelium1
left ventricle myocardium1

Per-gene tissue summary (top 30)

SymbolBgee breadthFANTOM5 breadthSCXATop tissues
TAGLN2260ubiquitousmarkerupper lobe of left lung, monocyte, right lung
PHLDB2249ubiquitousmarkerleft ventricle myocardium, kidney epithelium, cauda epididymis

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

SymbolInteractor count
TAGLN24,271
PHLDB2995

Structural data

PDB: 2 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

SymbolUniProtPDB entries
PHLDB2Q86SQ03
TAGLN2P378021

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 4. Enrichment computed across 2 evidence-associated genes (1 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 1 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

PathwayCohort genesFoldFDRSample cohort genes
Response to elevated platelet cytosolic Ca2+1163.1×0.015TAGLN2
Platelet activation, signaling and aggregation1105.7×0.015TAGLN2
Platelet degranulation187.8×0.015TAGLN2
Hemostasis136.0×0.028TAGLN2

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO termCohort genesFoldFDRSample cohort genes
negative regulation of wound healing, spreading of epidermal cells11203.7×0.008PHLDB2
negative regulation of focal adhesion assembly1383.0×0.009PHLDB2
negative regulation of stress fiber assembly1290.6×0.009PHLDB2
basement membrane organization1255.3×0.009PHLDB2
establishment of protein localization1216.1×0.009PHLDB2
establishment of cell polarity1191.5×0.009PHLDB2
epithelial cell differentiation187.8×0.016TAGLN2
microtubule cytoskeleton organization160.6×0.019PHLDB2
actin filament organization159.3×0.019TAGLN2
cell migration130.8×0.032PHLDB2

Therapeutics

Drug target analysis

Approved (phase 4): 0 · Phase ≥3: 0 · Phased (≥1): 0 · Undrugged: 2

Druggability breadth: 1 of 2 evidence-associated genes (50%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Top cohort targets by molecule count

SymbolMoleculesMax phase
TAGLN200
PHLDB200

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 0.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

SymbolAssaysType breakdown
TAGLN21Binding:1

Pharmacogenomics

Cohort genes with a PharmGKB record: 2; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Chemical tractability of cohort targets

0 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

TierDefinitionGenesSymbols
AApproved (phase 4 drug)0
BPhased (≥1) drug, not yet approved0
CDruggable family + PDB, no drug0
DDruggable family + AlphaFold only, no drug0
EDifficult family or no structure, no drug2TAGLN2, PHLDB2

Undrugged target profiles

2 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

SymbolChEMBL assaysDrugged partners (top 3)
TAGLN21
PHLDB20

Clinical trials & evidence

Clinical trials

Clinical trials: 8.

Phase distribution (across all retrieved trials)

PhaseTrials
Not specified5
PHASE21
PHASE1/PHASE21
PHASE11

Top trials by phase / activity

NCTPhaseStatusTitle
NCT01442363PHASE2TERMINATEDA Safety and Efficacy Study of BLI-1300 Ointment in Symptomatic Perianal Crohn’s Disease
NCT04519671PHASE1/PHASE2TERMINATEDMesenchymal Stem Cells for the Treatment of Perianal Fistulizing Crohn’s Disease
NCT01915927PHASE1COMPLETEDStem Cell Fistula Plug in Perianal Crohn’s Disease
NCT07519850Not specifiedRECRUITINGThe Impact of Perianal Disease on Patient’s Sex Life
NCT03654482Not specifiedCOMPLETEDSuperSeton Pilot Studie
NCT03994224Not specifiedTERMINATEDA Prospective Longitudinal Study of Fecal Microbiome and Calprotectin to Predict Response to Biological Therapy in Patients With CD
NCT04129723Not specifiedWITHDRAWNStopping Biological Therapy in PCD Study
NCT05709717Not specifiedUNKNOWNRegenerative Therapy With Autologous Stromal Vascular Fraction Derived Mesenchymal Stem Cells and Platelet-rich Plasma to Treat Complex Perianal Diseases