Vitreoretinal degeneration

disease
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Also known as degenerative vitreoretinopathy

Summary

Vitreoretinal degeneration (MONDO:0020248) is a disease (an umbrella term covering 9 Mondo subtypes) with 9 cohort genes.

At a glance

  • Umbrella term: 9 Mondo subtypes
  • Cohort genes: 9
  • ClinVar variants: 226

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

FieldValue
Canonical namevitreoretinal degeneration
Mondo IDMONDO:0020248
Orphanet98670
SNOMED CT247182006
UMLSC0344290
MedGen87480
GARD0005506
Is cancer (heuristic)no

Also known as: degenerative vitreoretinopathy

Data availability: 226 ClinVar variants · 1 HPO phenotype.

Disease family

An umbrella term covering 9 Mondo subtypes.

Classification path: disease › human disease › disease by body system or component › disorder of orbital regioneye disordervitreous body disordervitreous disordervitreous syneresisvitreoretinal degeneration

Subtypes (9): Wagner disease, snowflake vitreoretinal degeneration, X-linked retinoschisis, Stickler syndrome, exudative vitreoretinopathy, enhanced S-cone syndrome, CAPN5-related vitreoretinopathy, BEST1-related vitreoretinochoroidopathy, Knobloch syndrome

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

226 retrieved; paginated sample, class counts are floors:

70 benign, 61 uncertain significance, 49 conflicting classifications of pathogenicity, 34 benign/likely benign, 8 likely benign, 2 pathogenic, 1 pathogenic/likely pathogenic, 1 likely pathogenic

ClinVarVariant (HGVS)GeneClassificationReview
99428NM_000350.3(ABCA4):c.6089G>A (p.Arg2030Gln)ABCA4Pathogenicreviewed by expert panel
545640NM_001128922.2(LRRC32):c.1630C>T (p.Arg544Ter)LRRC32Pathogenic/Likely pathogenicno assertion criteria provided
523501NM_012338.4(TSPAN12):c.100del (p.Trp34fs)TSPAN12Pathogeniccriteria provided, single submitter
560373NM_002335.4(LRP5):c.2090A>G (p.Lys697Arg)LRP5Likely pathogenicno assertion criteria provided
7892NM_000350.3(ABCA4):c.5908C>T (p.Leu1970Phe)ABCA4Conflicting classifications of pathogenicitycriteria provided, conflicting classifications
167819NM_004385.5(VCAN):c.927T>C (p.Thr309=)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
167825NM_004385.5(VCAN):c.5859G>T (p.Thr1953=)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
198510NM_004385.5(VCAN):c.3713G>C (p.Arg1238Thr)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
198796NM_004385.5(VCAN):c.6767T>C (p.Leu2256Pro)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
198801NM_004385.5(VCAN):c.6902T>G (p.Phe2301Cys)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
259362NM_004385.5(VCAN):c.2234A>C (p.Lys745Thr)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354388NM_004385.5(VCAN):c.109T>G (p.Ser37Ala)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354393NM_004385.5(VCAN):c.854C>T (p.Ala285Val)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354401NM_004385.5(VCAN):c.1931C>T (p.Pro644Leu)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354405NM_004385.5(VCAN):c.2315C>G (p.Ala772Gly)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354407NM_004385.5(VCAN):c.2542G>A (p.Glu848Lys)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354412NM_004385.5(VCAN):c.3094C>G (p.Gln1032Glu)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354419NM_004385.5(VCAN):c.3683C>T (p.Ala1228Val)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354432NM_004385.5(VCAN):c.6127G>A (p.Glu2043Lys)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354441NM_004385.5(VCAN):c.7480G>C (p.Glu2494Gln)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354443NM_004385.5(VCAN):c.7493G>T (p.Ser2498Ile)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354449NM_004385.5(VCAN):c.7884C>A (p.Asn2628Lys)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354450NM_004385.5(VCAN):c.7895C>T (p.Thr2632Ile)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354451NM_004385.5(VCAN):c.7966A>G (p.Thr2656Ala)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354453NM_004385.5(VCAN):c.8063C>T (p.Pro2688Leu)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354458NM_004385.5(VCAN):c.8362A>G (p.Met2788Val)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354460NM_004385.5(VCAN):c.8683G>C (p.Asp2895His)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354461NM_004385.5(VCAN):c.9025G>A (p.Glu3009Lys)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
354466NM_004385.5(VCAN):c.9481C>G (p.Leu3161Val)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications
843872NM_004385.5(VCAN):c.157C>T (p.Pro53Ser)VCANConflicting classifications of pathogenicitycriteria provided, conflicting classifications

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 22 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Orphanet rare-disease linkage (cohort genes)

GeneOrphanet IDRare disease
CDH23Orphanet:231169Usher syndrome type 1
CDH23Orphanet:2965Prolactinoma
CDH23Orphanet:314777Familial isolated pituitary adenoma
CDH23Orphanet:90636Rare autosomal recessive non-syndromic sensorineural deafness type DFNB
CDH23Orphanet:91347TSH-secreting pituitary adenoma
CDH23Orphanet:96253Cushing disease
TSPAN12Orphanet:891Familial exudative vitreoretinopathy
COL9A3Orphanet:166002Multiple epiphyseal dysplasia due to collagen 9 anomaly
COL9A3Orphanet:250984Autosomal recessive Stickler syndrome
VCANOrphanet:898Wagner disease
ABCA4Orphanet:1872Cone rod dystrophy
ABCA4Orphanet:791Retinitis pigmentosa
ABCA4Orphanet:827Stargardt disease
LRP5Orphanet:178377Osteosclerosis-developmental delay-craniosynostosis syndrome
LRP5Orphanet:2783Autosomal dominant osteopetrosis type 1
LRP5Orphanet:2788Osteoporosis-pseudoglioma syndrome
LRP5Orphanet:2790Endosteal hyperostosis, Worth type
LRP5Orphanet:2924Isolated polycystic liver disease
LRP5Orphanet:3416Hyperostosis corticalis generalisata
LRP5Orphanet:498481LRP5-related primary osteoporosis
LRP5Orphanet:891Familial exudative vitreoretinopathy
LRP5Orphanet:90050Retinopathy of prematurity

Cohort genes → proteins

9 cohort genes, 8 distinct canonical proteins.

Evidence partition

SubsetGenes
multi_evidence9

Cohort genes (full)

SymbolHGNCEnsemblUniProtNameEvidence
CDH23HGNC:13733ENSG00000107736Q9H251Cadherin-23clinvar
C10orf105HGNC:20304ENSG00000214688Q8TEF2Uncharacterized protein C10orf105clinvar
TSPAN12HGNC:21641ENSG00000106025O95859Tetraspanin-12clinvar
COL9A3HGNC:2219ENSG00000092758Q14050Collagen alpha-3(IX) chainclinvar
VCANHGNC:2464ENSG00000038427P13611Versican core proteinclinvar
ABCA4HGNC:34ENSG00000198691P78363Retinal-specific phospholipid-transporting ATPase ABCA4clinvar
VCAN-AS1HGNC:40163ENSG00000249835VCAN antisense RNA 1clinvar
LRRC32HGNC:4161ENSG00000137507Q14392Transforming growth factor beta activator LRRC32clinvar
LRP5HGNC:6697ENSG00000162337O75197Low-density lipoprotein receptor-related protein 5clinvar

Cohort function summary

Lead sentence per gene, UniProt-curated.

SymbolProtein nameFunction (lead sentence)
CDH23Cadherin-23Cadherins are calcium-dependent cell adhesion proteins.
TSPAN12Tetraspanin-12Regulator of cell surface receptor signal transduction.
COL9A3Collagen alpha-3(IX) chainStructural component of hyaline cartilage and vitreous of the eye.
VCANVersican core proteinMay play a role in intercellular signaling and in connecting cells with the extracellular matrix.
ABCA4Retinal-specific phospholipid-transporting ATPase ABCA4Flippase that catalyzes in an ATP-dependent manner the transport of retinal-phosphatidylethanolamine conjugates like 11-cis and all-trans isomers of N-retinylidene-phosphatidylethanolamine (N-Ret-PE) from the lumen to the cytoplasmic leafl…
LRRC32Transforming growth factor beta activator LRRC32Key regulator of transforming growth factor beta (TGFB1, TGFB2 and TGFB3) that controls TGF-beta activation by maintaining it in a latent state during storage in extracellular space.
LRP5Low-density lipoprotein receptor-related protein 5Acts as a coreceptor with members of the frizzled family of seven-transmembrane spanning receptors to transduce signal by Wnt proteins.

Protein-family classification

Druggable: 2 · Difficult: 0 · Unknown: 7 · Druggable fraction: 0.22

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
Complement129.8×0.099
Transporter18.6×0.161
Other/Unknown71.4×0.161

Per-gene assignment

SymbolFamilyDruggable?ECInterPro (top 3)
CDH23Other/UnknownnoCadherin-like_dom, Cadherin-like_sf, Cadherin_CS
C10orf105Other/UnknownnoDUF5527
TSPAN12Other/UnknownnoTetraspanin_animals, Tetraspanin_EC2_sf, Tetraspanin/Peripherin
COL9A3Other/UnknownnoCollagen, Collagen_superfamily
VCANComplementyesEGF-type_Asp/Asn_hydroxyl_site, Sushi_SCR_CCP_dom, Link_dom
ABCA4TransporteryesABC_transporter-like_ATP-bd, AAA+_ATPase, ABCA4/ABCR
VCAN-AS1Other/Unknownno
LRRC32Other/UnknownnoLRRNT, Leu-rich_rpt, Leu-rich_rpt_typical-subtyp
LRP5Other/UnknownnoLDLR_classB_rpt, EGF, LDrepeatLR_classA_rpt

Expression context

Cohort genes with no expression data: 0.

7 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)9
unknown0

Top tissues across cohort

TissueCohort genes
ventricular zone2
left ovary1
right ovary1
blood1
cerebellar vermis1
quadriceps femoris1
nephron tubule1
oocyte1
secondary oocyte1
cartilage tissue1
inferior vagus X ganglion1
tibia1
leukocyte1
monocyte1
mononuclear cell1
male germ line stem cell (sensu Vertebrata) in testis1
pigmented layer of retina1
primordial germ cell in gonad1
calcaneal tendon1
granulocyte1

Per-gene tissue summary (top 30)

SymbolBgee breadthFANTOM5 breadthSCXATop tissues
CDH23161broadmarkerventricular zone, left ovary, right ovary
C10orf105107tissue_specificyesquadriceps femoris, blood, cerebellar vermis
TSPAN12267ubiquitousmarkeroocyte, nephron tubule, secondary oocyte
COL9A3218broadmarkertibia, cartilage tissue, inferior vagus X ganglion
VCAN293ubiquitousmarkermonocyte, mononuclear cell, leukocyte
ABCA4164tissue_specificmarkerpigmented layer of retina, primordial germ cell in gonad, male germ line stem cell (sensu Vertebrata) in testis
VCAN-AS1126yesventricular zone, granulocyte, calcaneal tendon
LRRC32220ubiquitousmarkerright coronary artery, left coronary artery, right lung
LRP5224ubiquitousmarkerright lobe of liver, mucosa of transverse colon, ascending aorta

Protein interactions among cohort

Intra-cohort edges: 2.

Hub genes (top 10 by interactor count)

SymbolInteractor count
VCAN2,806
LRP52,619
CDH231,575
ABCA41,532
COL9A31,482
LRRC321,441
TSPAN12686
C10orf10553
VCAN-AS10

Intra-cohort edges

ABSources
C10orf105CDH23string_interaction
LRP5TSPAN12string_interaction

Structural data

PDB: 5 · AlphaFold-only: 3 · No structure: 1

Cohort genes with PDB structures (top 30)

SymbolUniProtPDB entries
ABCA4P783638
CDH23Q9H2516
LRRC32Q143926
COL9A3Q140504
TSPAN12O958591

AlphaFold-only cohort genes (top 30 by pLDDT)

SymbolUniProtpLDDT
LRP5O7519778.65
C10orf105Q8TEF263.46
VCANP13611

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 43. Enrichment computed across 9 evidence-associated genes (5 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 5 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

PathwayCohort genesFoldFDRSample cohort genes
Defective visual phototransduction due to ABCA4 loss of function12284.0×0.009ABCA4
ECM proteoglycans260.1×0.009COL9A3, VCAN
Retinoid cycle disease events1571.0×0.013ABCA4
Diseases associated with visual transduction1571.0×0.013ABCA4
Diseases of the neuronal system1571.0×0.013ABCA4
Sensory Perception238.1×0.013CDH23, ABCA4
Signaling by LRP5 mutants1326.3×0.015LRP5
Defective CHST3 causes SEDCJD1285.5×0.015VCAN
Defective CHST14 causes EDS, musculocontractural type1285.5×0.015VCAN
Defective CHSY1 causes TPBS1285.5×0.015VCAN
Signaling by RNF43 mutants1253.8×0.015LRP5
DS-GAG biosynthesis1190.3×0.019VCAN
Negative regulation of TCF-dependent signaling by WNT ligand antagonists1142.8×0.020LRP5
Signaling by WNT in cancer1120.2×0.020LRP5
Defective B4GALT7 causes EDS, progeroid type1114.2×0.020VCAN
Defective B3GAT3 causes JDSSDHD1114.2×0.020VCAN
Defective B3GALT6 causes EDSP2 and SEMDJL11114.2×0.020VCAN
CS-GAG biosynthesis1108.8×0.020VCAN
CS/DS degradation1108.8×0.020VCAN
The canonical retinoid cycle in rods (twilight vision)1103.8×0.020ABCA4
Regulation of FZD by ubiquitination1103.8×0.020LRP5
Glycosaminoglycan-protein linkage region biosynthesis178.8×0.025VCAN
Disassembly of the destruction complex and recruitment of AXIN to the membrane171.4×0.026LRP5
Sensory processing of sound161.7×0.029CDH23
Visual phototransduction151.9×0.031ABCA4
Collagen chain trimerization151.9×0.031COL9A3
Signaling by PDGF150.8×0.031COL9A3
NCAM1 interactions149.6×0.031COL9A3
Sensory processing of sound by outer hair cells of the cochlea140.8×0.035CDH23
Assembly of collagen fibrils and other multimeric structures140.1×0.035COL9A3

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 6 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO termCohort genesFoldFDRSample cohort genes
Norrin signaling pathway21123.5×9e-05TSPAN12, LRP5
photoreceptor cell maintenance2119.5×0.005CDH23, ABCA4
establishment of protein localization to extracellular region11404.3×0.015LRRC32
phospholipid transfer to membrane1936.2×0.015ABCA4
cell-cell signaling involved in mammary gland development1936.2×0.015LRP5
mesodermal cell migration1561.7×0.015LRP5
extracellular matrix-cell signaling1561.7×0.015LRP5
anatomical structure regression1561.7×0.015LRP5
apoptotic process involved in blood vessel morphogenesis1468.1×0.015LRP5
establishment of blood-retinal barrier1468.1×0.015LRP5
glucose catabolic process1401.2×0.015LRP5
equilibrioception1401.2×0.015CDH23
retinal blood vessel morphogenesis1401.2×0.015LRP5
visual perception226.5×0.015CDH23, ABCA4
cell recognition1312.1×0.016VCAN
sensory perception of light stimulus1312.1×0.016CDH23
retina morphogenesis in camera-type eye1312.1×0.016LRP5
cell migration involved in gastrulation1255.3×0.016LRP5
bone marrow development1255.3×0.016LRP5
glial cell migration1234.1×0.016VCAN
osteoblast proliferation1234.1×0.016LRP5
branching involved in mammary gland duct morphogenesis1234.1×0.016LRP5
establishment of blood-brain barrier1234.1×0.016LRP5
phototransduction, visible light1216.1×0.016ABCA4
positive regulation of osteoblast proliferation1200.6×0.016LRP5
secondary palate development1200.6×0.016LRRC32
obsolete cell-cell adhesion via plasma-membrane adhesion molecules1187.2×0.016CDH23
negative regulation of activated T cell proliferation1175.5×0.016LRRC32
osteoblast development1165.2×0.016LRP5
gastrulation with mouth forming second1156.0×0.016LRP5

Therapeutics

Drug target analysis

Approved (phase 4): 0 · Phase ≥3: 0 · Phased (≥1): 0 · Undrugged: 9

Druggability breadth: 1 of 9 evidence-associated genes (11%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Top cohort targets by molecule count

SymbolMoleculesMax phase
CDH2300
C10orf10500
TSPAN1200
COL9A300
VCAN00
ABCA400
VCAN-AS100
LRRC3200
LRP500

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 0.

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

SymbolAssaysType breakdown
LRP51Binding:1

Pharmacogenomics

Cohort genes with a PharmGKB record: 8; 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 drug1ABCA4
DDruggable family + AlphaFold only, no drug1VCAN
EDifficult family or no structure, no drug7CDH23, C10orf105, TSPAN12, COL9A3, VCAN-AS1, LRRC32, LRP5

Undrugged target profiles

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

SymbolChEMBL assaysDrugged partners (top 3)
CDH230
C10orf1050
TSPAN120
COL9A30
VCAN0
ABCA40
VCAN-AS10
LRRC320
LRP51

Clinical trials & evidence

Clinical trials

Clinical trials: 0.