LADD syndrome 1

disease
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Also known as lacrimoauriculodentodigital syndrome 1LADD1

Summary

LADD syndrome 1 (MONDO:0100302) is a disease caused by FGFR2 (GenCC Definitive), with 2 cohort genes.

At a glance

  • Causal gene: FGFR2 (GenCC Definitive)
  • Cohort genes: 2
  • ClinVar variants: 73

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

FieldValue
Canonical nameLADD syndrome 1
Mondo IDMONDO:0100302
OMIM149730
UMLSC5774323
MedGen1824096
GARD0026132
Is cancer (heuristic)no

Also known as: lacrimoauriculodentodigital syndrome 1 · LADD1

Data availability: 73 ClinVar variants · 4 GenCC gene-disease records.

Disease family

Classification path: disease › human disease › disease by etiologic mechanism › disease of genetic or genomic mechanism › hereditary disease › autosomal genetic disease › autosomal dominant disease › LADD syndromeLADD syndrome 1

Related subtypes (2): lacrimoauriculodentodigital syndrome 2, lacrimoauriculodentodigital syndrome 3

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

ClinVar germline variants

73 retrieved; paginated sample, class counts are floors:

53 uncertain significance, 14 conflicting classifications of pathogenicity, 3 pathogenic, 2 likely benign, 1 pathogenic/likely pathogenic

ClinVarVariant (HGVS)GeneClassificationReview
13263NM_000141.5(FGFR2):c.1025G>A (p.Cys342Tyr)FGFR2Pathogenic/Likely pathogeniccriteria provided, multiple submitters, no conflicts
13296NM_000141.5(FGFR2):c.1942G>A (p.Ala648Thr)FGFR2Pathogeniccriteria provided, multiple submitters, no conflicts
13297NM_000141.5(FGFR2):c.1947_1949del (p.Arg649_Asp650delinsSer)FGFR2Pathogenicno assertion criteria provided
13298NM_000141.5(FGFR2):c.1882G>A (p.Ala628Thr)FGFR2Pathogeniccriteria provided, single submitter
1052478NM_000141.5(FGFR2):c.1348C>T (p.Arg450Cys)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
134387NM_000141.5(FGFR2):c.34G>A (p.Val12Met)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
1503438NM_000141.5(FGFR2):c.287G>T (p.Gly96Val)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
2891853NM_000141.5(FGFR2):c.1029G>A (p.Leu343=)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
2977031NM_000141.5(FGFR2):c.963C>A (p.Asp321Glu)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
298998NM_000141.5(FGFR2):c.1774C>T (p.Arg592Cys)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
3064171NM_000141.5(FGFR2):c.943G>A (p.Ala315Thr)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
3075688NM_000141.5(FGFR2):c.1124A>T (p.Tyr375Phe)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
3591007NM_000141.5(FGFR2):c.556A>G (p.Met186Val)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
3591089NM_000141.5(FGFR2):c.151G>A (p.Val51Met)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
374814NM_000141.5(FGFR2):c.940-2A>GFGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
577711NM_000141.5(FGFR2):c.989G>A (p.Arg330Gln)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
877638NM_000141.5(FGFR2):c.1213A>G (p.Lys405Glu)FGFR2Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
579912NM_000142.5(FGFR3):c.2005C>G (p.Arg669Gly)FGFR3Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
1317487NM_000141.5(FGFR2):c.67C>A (p.Pro23Thr)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
1372714NM_000141.5(FGFR2):c.877C>T (p.His293Tyr)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
1388509NM_000141.5(FGFR2):c.419C>T (p.Ala140Val)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
1412875NM_000141.5(FGFR2):c.1985A>G (p.Asn662Ser)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
1506367NM_000141.5(FGFR2):c.1085C>T (p.Ala362Val)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
1700436NM_000141.5(FGFR2):c.1637A>C (p.Asn546Thr)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
1903593NM_000141.5(FGFR2):c.1342A>G (p.Thr448Ala)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
1966091NM_000141.5(FGFR2):c.257T>G (p.Val86Gly)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
1971541NM_000141.5(FGFR2):c.1486G>A (p.Val496Ile)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
2072601NM_000141.5(FGFR2):c.293C>T (p.Thr98Met)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
2076324NM_000141.5(FGFR2):c.143A>C (p.Glu48Ala)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts
2172927NM_000141.5(FGFR2):c.544G>T (p.Gly182Trp)FGFR2Uncertain significancecriteria provided, multiple submitters, no conflicts

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 90 · Orphanet: 25 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

GenCC gene–disease validity (cohort genes)

the Disease column is the GenCC-asserted condition — a cohort gene’s strongest validity may be for a related predisposition syndrome.

GeneClassificationInheritanceDiseaseRecords
FGFR2DefinitiveAutosomal dominantLADD syndrome 138
FGFR3SupportiveAutosomal dominantLADD syndrome52

Orphanet rare-disease linkage (cohort genes)

GeneOrphanet IDRare disease
FGFR2Orphanet:1540Jackson-Weiss syndrome
FGFR2Orphanet:1555Cutis gyrata-acanthosis nigricans-craniosynostosis syndrome
FGFR2Orphanet:168624Familial scaphocephaly syndrome, McGillivray type
FGFR2Orphanet:207Crouzon syndrome
FGFR2Orphanet:2363Lacrimoauriculodentodigital syndrome
FGFR2Orphanet:313855FGFR2-related bent bone dysplasia
FGFR2Orphanet:596008Antley-Bixler syndrome without genital anomaly or disorder of steroidogenesis
FGFR2Orphanet:794Saethre-Chotzen syndrome
FGFR2Orphanet:87Apert syndrome
FGFR2Orphanet:93258Pfeiffer syndrome type 1
FGFR2Orphanet:93259Pfeiffer syndrome type 2
FGFR2Orphanet:93260Pfeiffer syndrome type 3
FGFR3Orphanet:15Achondroplasia
FGFR3Orphanet:1860Thanatophoric dysplasia type 1
FGFR3Orphanet:2363Lacrimoauriculodentodigital syndrome
FGFR3Orphanet:251576Gliosarcoma
FGFR3Orphanet:251579Giant cell glioblastoma
FGFR3Orphanet:35099Non-syndromic bicoronal craniosynostosis
FGFR3Orphanet:429Hypochondroplasia
FGFR3Orphanet:53271Muenke syndrome
FGFR3Orphanet:794Saethre-Chotzen syndrome
FGFR3Orphanet:85164Camptodactyly-tall stature-scoliosis-hearing loss syndrome
FGFR3Orphanet:85165Severe achondroplasia-developmental delay-acanthosis nigricans syndrome
FGFR3Orphanet:93262Crouzon syndrome-acanthosis nigricans syndrome
FGFR3Orphanet:93274Thanatophoric dysplasia type 2

Cohort genes → proteins

2 cohort genes, 2 distinct canonical proteins.

Evidence partition

SubsetGenes
multi_evidence2

Cohort genes (full)

SymbolHGNCEnsemblUniProtNameEvidence
FGFR2HGNC:3689ENSG00000066468P21802Fibroblast growth factor receptor 2gencc,clinvar
FGFR3HGNC:3690ENSG00000068078P22607Fibroblast growth factor receptor 3gencc,clinvar

Cohort function summary

Lead sentence per gene, UniProt-curated.

SymbolProtein nameFunction (lead sentence)
FGFR2Fibroblast growth factor receptor 2Tyrosine-protein kinase that acts as a cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of cell proliferation, differentiation, migration and apoptosis, and in the regulation of embryonic de…
FGFR3Fibroblast growth factor receptor 3Tyrosine-protein kinase that acts as a cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of cell proliferation, differentiation and apoptosis.

Protein-family classification

Druggable: 2 · Difficult: 0 · Unknown: 0 · Druggable fraction: 1.0

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
Kinase227.7×0.001

Per-gene assignment

SymbolFamilyDruggable?ECInterPro (top 3)
FGFR2Kinaseyes2.7.10.1Prot_kinase_dom, Ser-Thr/Tyr_kinase_cat_dom, Ig_sub2
FGFR3Kinaseyes2.7.10.1Prot_kinase_dom, Ser-Thr/Tyr_kinase_cat_dom, Ig_sub2

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
C1 segment of cervical spinal cord1
corpus callosum1
spinal cord1
skin of hip1
upper arm skin1
upper leg skin1

Per-gene tissue summary (top 30)

SymbolBgee breadthFANTOM5 breadthSCXATop tissues
FGFR2272broadmarkerC1 segment of cervical spinal cord, spinal cord, corpus callosum
FGFR3262broadmarkerupper leg skin, skin of hip, upper arm skin

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

SymbolInteractor count
FGFR34,510
FGFR2449

Structural data

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

Cohort genes with PDB structures (top 30)

SymbolUniProtPDB entries
FGFR2P2180263
FGFR3P2260715

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 29. Enrichment computed across 2 evidence-associated genes (2 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 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

PathwayCohort genesFoldFDRSample cohort genes
PI3K Cascade2271.9×4e-04FGFR2, FGFR3
Signaling by FGFR2 amplification mutants15710.0×7e-04FGFR2
t(4;14) translocations of FGFR315710.0×7e-04FGFR3
Signaling by FGFR2 fusions15710.0×7e-04FGFR2
Signaling by FGFR3 fusions in cancer15710.0×7e-04FGFR3
Constitutive Signaling by Aberrant PI3K in Cancer2126.9×7e-04FGFR2, FGFR3
PI5P, PP2A and IER3 Regulate PI3K/AKT Signaling296.8×7e-04FGFR2, FGFR3
PIP3 activates AKT signaling266.8×8e-04FGFR2, FGFR3
RAF/MAP kinase cascade261.1×9e-04FGFR2, FGFR3
FGFR3b ligand binding and activation1815.7×0.004FGFR3
FGFR2b ligand binding and activation1571.0×0.004FGFR2
Signaling by activated point mutants of FGFR31475.8×0.004FGFR3
FGFR3c ligand binding and activation1439.2×0.004FGFR3
FGFR2c ligand binding and activation1439.2×0.004FGFR2
Phospholipase C-mediated cascade; FGFR31439.2×0.004FGFR3
Activated point mutants of FGFR21335.9×0.005FGFR2
Phospholipase C-mediated cascade; FGFR21317.2×0.005FGFR2
PI-3K cascade:FGFR31317.2×0.005FGFR3
SHC-mediated cascade:FGFR31300.5×0.005FGFR3
Signaling by FGFR2 IIIa TM1300.5×0.005FGFR2
FRS-mediated FGFR3 signaling1271.9×0.005FGFR3
PI-3K cascade:FGFR21248.3×0.005FGFR2
Signaling by FGFR3 in disease1248.3×0.005FGFR3
SHC-mediated cascade:FGFR21237.9×0.005FGFR2
FRS-mediated FGFR2 signaling1219.6×0.005FGFR2
Negative regulation of FGFR3 signaling1219.6×0.005FGFR3
FGFR2 alternative splicing1211.5×0.005FGFR2
Negative regulation of FGFR2 signaling1184.2×0.006FGFR2
Signaling by FGFR2 in disease1132.8×0.008FGFR2

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
positive regulation of phospholipase activity23370.4×7e-06FGFR2, FGFR3
endochondral bone growth21685.2×2e-05FGFR2, FGFR3
bone morphogenesis2601.9×9e-05FGFR2, FGFR3
fibroblast growth factor receptor signaling pathway2285.6×3e-04FGFR2, FGFR3
bone mineralization2271.8×3e-04FGFR2, FGFR3
fibroblast growth factor receptor signaling pathway involved in negative regulation of apoptotic process in bone marrow cell18426.0×0.001FGFR2
fibroblast growth factor receptor signaling pathway involved in hemopoiesis18426.0×0.001FGFR2
fibroblast growth factor receptor signaling pathway involved in positive regulation of cell proliferation in bone marrow18426.0×0.001FGFR2
negative regulation of developmental growth18426.0×0.001FGFR3
lateral sprouting from an epithelium18426.0×0.001FGFR2
orbitofrontal cortex development14213.0×0.001FGFR2
prostate gland morphogenesis14213.0×0.001FGFR2
squamous basal epithelial stem cell differentiation involved in prostate gland acinus development14213.0×0.001FGFR2
mammary gland bud formation14213.0×0.001FGFR2
branch elongation involved in salivary gland morphogenesis14213.0×0.001FGFR2
mesenchymal cell differentiation involved in lung development14213.0×0.001FGFR2
fibroblast growth factor receptor apoptotic signaling pathway14213.0×0.001FGFR3
positive regulation of ERK1 and ERK2 cascade285.1×0.001FGFR2, FGFR3
positive regulation of MAPK cascade280.6×0.001FGFR2, FGFR3
cell-cell signaling269.6×0.001FGFR2, FGFR3
regulation of osteoblast proliferation12808.7×0.002FGFR2
fibroblast growth factor receptor signaling pathway involved in orbitofrontal cortex development12808.7×0.002FGFR2
prostate epithelial cord elongation12808.7×0.002FGFR2
bone maturation12808.7×0.002FGFR3
ventricular zone neuroblast division12106.5×0.002FGFR2
embryonic organ morphogenesis12106.5×0.002FGFR2
reproductive structure development12106.5×0.002FGFR2
regulation of morphogenesis of a branching structure12106.5×0.002FGFR2
regulation of smooth muscle cell differentiation11685.2×0.002FGFR2
branching involved in prostate gland morphogenesis11685.2×0.002FGFR2

Therapeutics

Drug target analysis

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

Druggability breadth: 2 of 2 evidence-associated genes (100%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Genes with an approved drug

The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.

SymbolExample approved molecule
FGFR2PONATINIB
FGFR3PONATINIB

Top cohort targets by molecule count

SymbolMoleculesMax phase
FGFR3644
FGFR2594

Drugs targeting cohort genes (top 30)

MoleculeMax phaseTargets in cohort
PONATINIB4FGFR2, FGFR3
PEMIGATINIB4FGFR2, FGFR3
NINTEDANIB4FGFR2, FGFR3
FEDRATINIB4FGFR2, FGFR3
LENVATINIB4FGFR2, FGFR3
AXITINIB4FGFR2, FGFR3
SORAFENIB4FGFR2, FGFR3
INFIGRATINIB PHOSPHATE4FGFR2, FGFR3
INFIGRATINIB4FGFR2, FGFR3
IBRUTINIB4FGFR2
CERITINIB4FGFR2, FGFR3
VANDETANIB4FGFR2, FGFR3
NINTEDANIB ESYLATE4FGFR2, FGFR3
BRIGATINIB4FGFR2, FGFR3
ERDAFITINIB4FGFR2, FGFR3
FUTIBATINIB4FGFR2, FGFR3
PAZOPANIB4FGFR2, FGFR3
SUNITINIB4FGFR2, FGFR3
DASATINIB4FGFR2, FGFR3
ERLOTINIB4FGFR2
MIDOSTAURIN4FGFR2, FGFR3
ENTRECTINIB4FGFR3
CRIZOTINIB4FGFR3
LINIFANIB3FGFR2, FGFR3
SEMAXANIB3FGFR2, FGFR3
BRIVANIB3FGFR2, FGFR3
CEDIRANIB3FGFR2, FGFR3
DOVITINIB3FGFR2, FGFR3
LESTAURTINIB3FGFR2, FGFR3
ALISERTIB3FGFR3

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 2.

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

SymbolAssaysType breakdown
FGFR3975Binding:948, Functional:18, ADMET:9
FGFR2966Binding:940, Functional:22, ADMET:4

Cohort enzymes (BRENDA EC)

SymbolEC numbersNames
FGFR22.7.10.1receptor protein-tyrosine kinase
FGFR32.7.10.1receptor protein-tyrosine kinase

Cohort genes with high screening signal

≥100 ChEMBL assays — a studied-ness signal; see Therapeutics for approved-drug status.

SymbolChEMBL assays
FGFR2966
FGFR3975

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

30 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.

CompoundMax phaseCohort target (bioactivity)
PONATINIB4FGFR2, FGFR3
PEMIGATINIB4FGFR2, FGFR3
NINTEDANIB4FGFR2, FGFR3
FEDRATINIB4FGFR2, FGFR3
LENVATINIB4FGFR2, FGFR3
AXITINIB4FGFR2, FGFR3
SORAFENIB4FGFR2, FGFR3
INFIGRATINIB PHOSPHATE4FGFR2, FGFR3
INFIGRATINIB4FGFR2, FGFR3
IBRUTINIB4FGFR2
CERITINIB4FGFR2, FGFR3
VANDETANIB4FGFR2, FGFR3
NINTEDANIB ESYLATE4FGFR2, FGFR3
BRIGATINIB4FGFR2, FGFR3
ERDAFITINIB4FGFR2, FGFR3
FUTIBATINIB4FGFR2, FGFR3
PAZOPANIB4FGFR2, FGFR3
SUNITINIB4FGFR2, FGFR3
DASATINIB4FGFR2, FGFR3
ERLOTINIB4FGFR2
MIDOSTAURIN4FGFR2, FGFR3
ENTRECTINIB4FGFR3
CRIZOTINIB4FGFR3
LINIFANIB3FGFR2, FGFR3
SEMAXANIB3FGFR2, FGFR3
BRIVANIB3FGFR2, FGFR3
CEDIRANIB3FGFR2, FGFR3
DOVITINIB3FGFR2, FGFR3
LESTAURTINIB3FGFR2, FGFR3
ALISERTIB3FGFR3

Druggability pyramid

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

TierDefinitionGenesSymbols
AApproved (phase 4 drug)2FGFR2, FGFR3
BPhased (≥1) drug, not yet approved0
CDruggable family + PDB, no drug0
DDruggable family + AlphaFold only, no drug0
EDifficult family or no structure, no drug0

Undrugged target profiles

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

Clinical trials & evidence

Clinical trials

Clinical trials: 0.