CEP290-related ciliopathy
disease diseaseOn this page
Also known as amaurosis congenita of Leber, type 10Bardet-Biedl syndrome 14Bardet-Biedl syndrome type 14BBS14CEP290 ciliopathyCEP290 Joubert syndromeCEP290 Leber congenital amaurosisCEP290 Meckel syndromeCEP290 Senior-Loken syndromeJBTS5Joubert syndrome 5Joubert syndrome caused by mutation in CEP290Joubert syndrome type 5LCA10Leber congenital amaurosis 10Leber congenital amaurosis caused by mutation in CEP290Leber congenital amaurosis type 10Meckel syndrome 4Meckel syndrome caused by mutation in CEP290Meckel syndrome, type 4
Summary
CEP290-related ciliopathy (MONDO:0100451) is a disease (an umbrella term covering 5 Mondo subtypes) with 3 cohort genes and 6 clinical trials. Top therapeutic interventions include sepofarsen and brinretigene vesgedparvovec.
At a glance
- Umbrella term: 5 Mondo subtypes
- Cohort genes: 3
- ClinVar variants: 40
- Clinical trials: 6
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | CEP290-related ciliopathy |
| Mondo ID | MONDO:0100451 |
| GARD | 0026225 |
| Is cancer (heuristic) | no |
Also known as: amaurosis congenita of Leber, type 10 · Bardet-Biedl syndrome 14 · Bardet-Biedl syndrome type 14 · BBS14 · CEP290 ciliopathy · CEP290 Joubert syndrome · CEP290 Leber congenital amaurosis · CEP290 Meckel syndrome · CEP290 Senior-Loken syndrome · JBTS5 · Joubert syndrome 5 · Joubert syndrome caused by mutation in CEP290 · Joubert syndrome type 5 · LCA10 · Leber congenital amaurosis 10 · Leber congenital amaurosis caused by mutation in CEP290 · Leber congenital amaurosis type 10 · Meckel syndrome 4 · Meckel syndrome caused by mutation in CEP290 · Meckel syndrome, type 4 (+8 more)
Data availability: 40 ClinVar variants.
Disease family
An umbrella term covering 5 Mondo subtypes.
Classification path: disease › human disease › disease by etiologic mechanism › disease of genetic or genomic mechanism › hereditary disease › ciliopathy › CEP290-related ciliopathy
Related subtypes (35): Alstrom syndrome, Marden-Walker syndrome, nephronophthisis 1, Bardet-Biedl syndrome, primary ciliary dyskinesia, Senior-Loken syndrome, Jeune syndrome, Joubert syndrome, Meckel syndrome, retinal ciliopathy, oculocerebrodental syndrome, IFT140-related recessive ciliopathy, BBS9-related ciliopathy, BBS10-related ciliopathy, CEP164-related ciliopathy, CFAP418-related ciliopathy, WDPCP-related ciliopathy, SDCCAG8-related ciliopathy, KIF7-related ciliopathy, Alsahan-Harris syndrome, OFD1-related ciliopathy, BBS7-related ciliopathy, BBS1-related ciliopathy, BBS4-related ciliopathy, BBS12-related ciliopathy, LZTFL1-related ciliopathy, BBS5-related ciliopathy, BBS2-related ciliopathy, TTC8-related ciliopathy, MKKS-related ciliopathy, ARL6-related ciliopathy, MKS1-related ciliopathy, TUBB4B-related ciliopathy, INTU-related skeletal ciliopathy, ciliopathy-IFT74
Subtypes (5): Joubert syndrome 5, Senior-Loken syndrome 6, Meckel syndrome, type 4, Leber congenital amaurosis 10, Bardet-Biedl syndrome 14
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
40 retrieved; paginated sample, class counts are floors:
16 pathogenic, 10 uncertain significance, 8 pathogenic/likely pathogenic, 5 conflicting classifications of pathogenicity, 1 likely pathogenic
| ClinVar | Variant (HGVS) | Gene | Classification | Review |
|---|---|---|---|---|
| 1069579 | NM_025114.4(CEP290):c.5235_5238del (p.Ser1745fs) | CEP290 | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 1072859 | NM_025114.4(CEP290):c.3922C>T (p.Gln1308Ter) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 1074486 | NM_025114.4(CEP290):c.4983del (p.Lys1661_Val1662insTer) | CEP290 | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 1339 | NM_025114.4(CEP290):c.4723A>T (p.Lys1575Ter) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 1986597 | NM_025114.4(CEP290):c.1616del (p.Leu538_Leu539insTer) | CEP290 | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 2114918 | NM_025114.4(CEP290):c.6838A>T (p.Lys2280Ter) | CEP290 | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 217624 | NM_025114.4(CEP290):c.164_167del (p.Thr55fs) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 4081609 | NM_025114.4(CEP290):c.4544_4548del (p.Arg1515fs) | CEP290 | Pathogenic | criteria provided, single submitter |
| 497937 | NM_025114.4(CEP290):c.6798G>A (p.Trp2266Ter) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 522742 | NM_025114.4(CEP290):c.1419_1423del (p.Ile474fs) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 56729 | NM_025114.4(CEP290):c.1219_1220del (p.Met407fs) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 56734 | NM_025114.4(CEP290):c.289G>T (p.Glu97Ter) | CEP290 | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 572652 | NM_025114.4(CEP290):c.4962_4963del (p.Glu1656fs) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 635087 | NM_025114.4(CEP290):c.4792_4795del (p.Lys1598fs) | CEP290 | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 654872 | NM_025114.4(CEP290):c.6869del (p.Asn2290fs) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 654881 | NM_025114.4(CEP290):c.322C>T (p.Arg108Ter) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 665410 | NM_025114.4(CEP290):c.3802C>T (p.Gln1268Ter) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 830333 | NM_025114.4(CEP290):c.7341_7344dup (p.Ser2449fs) | CEP290 | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 855145 | NM_025114.4(CEP290):c.6448_6455del (p.Gln2150fs) | CEP290 | Pathogenic/Likely pathogenic | criteria provided, multiple submitters, no conflicts |
| 857949 | NM_025114.4(CEP290):c.1189+1G>A | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 930681 | NM_025114.4(CEP290):c.7027del (p.Val2343fs) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 99859 | NM_025114.4(CEP290):c.5515_5518del (p.Glu1839fs) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 99860 | NM_025114.4(CEP290):c.5649dup (p.Leu1884fs) | CEP290 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 856893 | NM_025114.4(CEP290):c.7328_7332del (p.Glu2443fs) | RLIG1 | Pathogenic | criteria provided, multiple submitters, no conflicts |
| 2574041 | NM_020944.3(GBA2):c.715dup (p.Tyr239fs) | GBA2 | Likely pathogenic | criteria provided, single submitter |
| 310628 | NM_025114.4(CEP290):c.503G>A (p.Arg168His) | CEP290 | Conflicting classifications of pathogenicity | criteria provided, conflicting classifications |
| 3575347 | NM_025114.4(CEP290):c.7256del (p.Ser2419fs) | CEP290 | Conflicting classifications of pathogenicity | criteria provided, conflicting classifications |
| 449448 | NM_025114.4(CEP290):c.4438-3del | CEP290 | Conflicting classifications of pathogenicity | criteria provided, conflicting classifications |
| 695268 | NM_025114.4(CEP290):c.2090C>G (p.Ala697Gly) | CEP290 | Conflicting classifications of pathogenicity | criteria provided, conflicting classifications |
| 697728 | NM_025114.4(CEP290):c.2638G>T (p.Ala880Ser) | CEP290 | Conflicting classifications of pathogenicity | criteria provided, conflicting classifications |
Genes & proteins
Mendelian disease overlap and somatic drivers
GenCC: 0 · Orphanet: 7 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0
Orphanet rare-disease linkage (cohort genes)
| Gene | Orphanet ID | Rare disease |
|---|---|---|
| GBA2 | Orphanet:320391 | Autosomal recessive spastic paraplegia type 46 |
| GBA2 | Orphanet:352641 | Autosomal recessive cerebellar ataxia with late-onset spasticity |
| CEP290 | Orphanet:110 | Bardet-Biedl syndrome |
| CEP290 | Orphanet:2318 | Joubert syndrome with oculorenal defect |
| CEP290 | Orphanet:3156 | Senior-Loken syndrome |
| CEP290 | Orphanet:564 | Meckel syndrome |
| CEP290 | Orphanet:65 | Leber congenital amaurosis |
Cohort genes → proteins
3 cohort genes, 3 distinct canonical proteins.
Evidence partition
| Subset | Genes |
|---|---|
| multi_evidence | 3 |
Cohort genes (full)
| Symbol | HGNC | Ensembl | UniProt | Name | Evidence |
|---|---|---|---|---|---|
| GBA2 | HGNC:18986 | ENSG00000070610 | Q9HCG7 | Non-lysosomal glucosylceramidase | clinvar |
| RLIG1 | HGNC:25322 | ENSG00000133641 | Q8N999 | RNA ligase 1 | clinvar |
| CEP290 | HGNC:29021 | ENSG00000198707 | O15078 | Centrosomal protein of 290 kDa | clinvar |
Cohort function summary
Lead sentence per gene, UniProt-curated.
| Symbol | Protein name | Function (lead sentence) |
|---|---|---|
| GBA2 | Non-lysosomal glucosylceramidase | Non-lysosomal glucosylceramidase that catalyzes the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1’)-N-acylsphing-4-enine) to free glucose and ceramides (such as N-acylsphing-4-enine). |
| RLIG1 | RNA ligase 1 | Functions as an RNA ligase, in vitro. |
| CEP290 | Centrosomal protein of 290 kDa | Involved in early and late steps in cilia formation. |
Protein-family classification
Druggable: 1 · Difficult: 0 · Unknown: 2 · Druggable fraction: 0.33
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.
| Family | Genes | Fold | FDR |
|---|---|---|---|
| Enzyme (other) | 1 | 4.0× | 0.460 |
| Other/Unknown | 2 | 1.2× | 0.587 |
Per-gene assignment
| Symbol | Family | Druggable? | EC | InterPro (top 3) |
|---|---|---|---|---|
| GBA2 | Enzyme (other) | yes | 3.2.1.45 | GH116_catalytic, 6-hairpin_glycosidase_sf, 6hp_glycosidase-like_sf |
| RLIG1 | Other/Unknown | no | RLIG1 | |
| CEP290 | Other/Unknown | no | Cep290, Cep209_CC5 |
Expression context
Cohort genes with no expression data: 0.
3 cohort genes are a single-cell marker in ≥1 SCXA experiment.
Breadth distribution (Bgee present_calls)
| Bucket | Genes |
|---|---|
| narrow (1-5 tissues) | 0 |
| moderate (6-20) | 0 |
| broad (>20) | 3 |
| unknown | 0 |
Top tissues across cohort
| Tissue | Cohort genes |
|---|---|
| metanephros cortex | 1 |
| right hemisphere of cerebellum | 1 |
| small intestine Peyer’s patch | 1 |
| Brodmann (1909) area 23 | 1 |
| endothelial cell | 1 |
| oocyte | 1 |
| male germ line stem cell (sensu Vertebrata) in testis | 1 |
| right uterine tube | 1 |
| ventricular zone | 1 |
Per-gene tissue summary (top 30)
| Symbol | Bgee breadth | FANTOM5 breadth | SCXA | Top tissues |
|---|---|---|---|---|
| GBA2 | 248 | ubiquitous | marker | metanephros cortex, right hemisphere of cerebellum, small intestine Peyer’s patch |
| RLIG1 | 288 | ubiquitous | marker | endothelial cell, Brodmann (1909) area 23, oocyte |
| CEP290 | 278 | ubiquitous | marker | right uterine tube, male germ line stem cell (sensu Vertebrata) in testis, ventricular zone |
Protein interactions among cohort
Intra-cohort edges: 0.
Hub genes (top 10 by interactor count)
| Symbol | Interactor count |
|---|---|
| CEP290 | 2,778 |
| GBA2 | 1,709 |
| RLIG1 | 506 |
Structural data
PDB: 0 · AlphaFold-only: 3 · No structure: 0
AlphaFold-only cohort genes (top 30 by pLDDT)
| Symbol | UniProt | pLDDT |
|---|---|---|
| RLIG1 | Q8N999 | 92.03 |
| GBA2 | Q9HCG7 | 89.77 |
| CEP290 | O15078 | 60.90 |
Function
Pathway analysis
Distinct Reactome pathways touched by cohort: 20. Enrichment computed across 3 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.
| Pathway | Cohort genes | Fold | FDR | Sample cohort genes |
|---|---|---|---|---|
| Glycosphingolipid catabolism | 1 | 146.4× | 0.031 | GBA2 |
| Centrosome maturation | 1 | 126.9× | 0.031 | CEP290 |
| Loss of Nlp from mitotic centrosomes | 1 | 79.3× | 0.031 | CEP290 |
| Loss of proteins required for interphase microtubule organization from the centrosome | 1 | 79.3× | 0.031 | CEP290 |
| AURKA Activation by TPX2 | 1 | 76.1× | 0.031 | CEP290 |
| Recruitment of mitotic centrosome proteins and complexes | 1 | 68.0× | 0.031 | CEP290 |
| Regulation of PLK1 Activity at G2/M Transition | 1 | 63.4× | 0.031 | CEP290 |
| Mitotic G2-G2/M phases | 1 | 63.4× | 0.031 | CEP290 |
| G2/M Transition | 1 | 63.4× | 0.031 | CEP290 |
| Recruitment of NuMA to mitotic centrosomes | 1 | 58.3× | 0.031 | CEP290 |
| Anchoring of the basal body to the plasma membrane | 1 | 56.5× | 0.031 | CEP290 |
| Cilium Assembly | 1 | 54.4× | 0.031 | CEP290 |
| Mitotic Prometaphase | 1 | 34.6× | 0.040 | CEP290 |
| Organelle biogenesis and maintenance | 1 | 33.0× | 0.040 | CEP290 |
| M Phase | 1 | 33.0× | 0.040 | CEP290 |
| Cell Cycle, Mitotic | 1 | 24.1× | 0.051 | CEP290 |
| Cell Cycle | 1 | 18.0× | 0.064 | CEP290 |
| Innate Immune System | 1 | 12.8× | 0.085 | CEP290 |
| Neutrophil degranulation | 1 | 11.5× | 0.089 | CEP290 |
| Immune System | 1 | 6.5× | 0.148 | CEP290 |
GO biological processes by enrichment
Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.
| GO term | Cohort genes | Fold | FDR | Sample cohort genes |
|---|---|---|---|---|
| obsolete ciliary basal body-plasma membrane docking | 1 | 2808.7× | 0.004 | CEP290 |
| glucosylceramide catabolic process | 1 | 1872.4× | 0.004 | GBA2 |
| RNA repair | 1 | 1872.4× | 0.004 | RLIG1 |
| ciliary transition zone assembly | 1 | 1872.4× | 0.004 | CEP290 |
| glycoside catabolic process | 1 | 936.2× | 0.005 | GBA2 |
| regulation of membrane lipid distribution | 1 | 936.2× | 0.005 | GBA2 |
| pronephros development | 1 | 802.5× | 0.005 | CEP290 |
| regulation of establishment of protein localization | 1 | 802.5× | 0.005 | CEP290 |
| otic vesicle formation | 1 | 702.2× | 0.005 | CEP290 |
| glycosphingolipid catabolic process | 1 | 510.7× | 0.006 | GBA2 |
| glycolipid biosynthetic process | 1 | 468.1× | 0.006 | GBA2 |
| hindbrain development | 1 | 374.5× | 0.006 | CEP290 |
| regulation of microtubule polymerization | 1 | 374.5× | 0.006 | GBA2 |
| response to reactive oxygen species | 1 | 351.1× | 0.006 | RLIG1 |
| bile acid metabolic process | 1 | 330.4× | 0.006 | GBA2 |
| eye photoreceptor cell development | 1 | 280.9× | 0.006 | CEP290 |
| central nervous system neuron development | 1 | 267.5× | 0.006 | GBA2 |
| positive regulation of intracellular protein transport | 1 | 224.7× | 0.007 | CEP290 |
| regulation of actin filament polymerization | 1 | 193.7× | 0.008 | GBA2 |
| camera-type eye development | 1 | 119.5× | 0.012 | CEP290 |
| non-motile cilium assembly | 1 | 96.8× | 0.014 | CEP290 |
| hematopoietic progenitor cell differentiation | 1 | 79.1× | 0.017 | RLIG1 |
| cholesterol metabolic process | 1 | 65.3× | 0.019 | GBA2 |
| kidney development | 1 | 46.8× | 0.025 | CEP290 |
| carbohydrate metabolic process | 1 | 45.3× | 0.025 | GBA2 |
| central nervous system development | 1 | 38.5× | 0.029 | GBA2 |
| cilium assembly | 1 | 24.5× | 0.043 | CEP290 |
| protein transport | 1 | 14.6× | 0.069 | CEP290 |
| positive regulation of DNA-templated transcription | 1 | 9.3× | 0.104 | CEP290 |
Therapeutics
Drug target analysis
Approved (phase 4): 1 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 2
Druggability breadth: 1 of 3 evidence-associated genes (33%) 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.
| Symbol | Example approved molecule |
|---|---|
| GBA2 | MIGLUSTAT |
Top cohort targets by molecule count
| Symbol | Molecules | Max phase |
|---|---|---|
| GBA2 | 6 | 4 |
| RLIG1 | 0 | 0 |
| CEP290 | 0 | 0 |
Drugs targeting cohort genes (top 30)
| Molecule | Max phase | Targets in cohort |
|---|---|---|
| MIGLUSTAT | 4 | GBA2 |
| MIGALASTAT | 4 | GBA2 |
| LUCERASTAT | 3 | GBA2 |
| AFEGOSTAT | 2 | GBA2 |
| DUVOGLUSTAT | 2 | GBA2 |
| NIZUBAGLUSTAT | 2 | GBA2 |
Bioactivity and enzyme data
Enzyme cohort genes (≥1 EC): 1.
Cohort genes with ChEMBL bioactivity (full, sorted by assay count)
| Symbol | Assays | Type breakdown |
|---|---|---|
| GBA2 | 38 | Binding:38 |
Cohort enzymes (BRENDA EC)
| Symbol | EC numbers | Names |
|---|---|---|
| GBA2 | 3.2.1.45 | glucosylceramidase |
Pharmacogenomics
Cohort genes with a PharmGKB record: 3; with CPIC/DPWG dosing guidelines: 0.
No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).
Chemical tractability of cohort targets
6 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.
| Compound | Max phase | Cohort target (bioactivity) |
|---|---|---|
| MIGLUSTAT | 4 | GBA2 |
| MIGALASTAT | 4 | GBA2 |
| LUCERASTAT | 3 | GBA2 |
| AFEGOSTAT | 2 | GBA2 |
| DUVOGLUSTAT | 2 | GBA2 |
| NIZUBAGLUSTAT | 2 | GBA2 |
Druggability pyramid
Cohort genes binned by druggability tier (high → low):
| Tier | Definition | Genes | Symbols |
|---|---|---|---|
| A | Approved (phase 4 drug) | 1 | GBA2 |
| B | Phased (≥1) drug, not yet approved | 0 | |
| C | Druggable family + PDB, no drug | 0 | |
| D | Druggable family + AlphaFold only, no drug | 0 | |
| E | Difficult family or no structure, no drug | 2 | RLIG1, CEP290 |
Undrugged target profiles
2 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).
| Symbol | ChEMBL assays | Drugged partners (top 3) |
|---|---|---|
| RLIG1 | 0 | — |
| CEP290 | 0 | — |
Clinical trials & evidence
Clinical trials
Clinical trials: 6.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2/PHASE3 | 2 |
| PHASE1/PHASE2 | 2 |
| PHASE3 | 1 |
| Not specified | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT03913143 | PHASE2/PHASE3 | ACTIVE_NOT_RECRUITING | A Study to Evaluate Efficacy, Safety, Tolerability and Exposure After a Repeat-dose of Sepofarsen (QR-110) in LCA10 (ILLUMINATE) |
| NCT06891443 | PHASE3 | RECRUITING | Study to Evaluate Sepofarsen in Subjects With Leber Congenital Amaurosis (LCA) Type 10 (HYPERION) |
| NCT04855045 | PHASE2/PHASE3 | UNKNOWN | An Open-label, Dose Escalation and Double-masked, Randomized, Controlled Trial Evaluating Safety and Tolerability of Sepofarsen in Children (<8 Years of Age) With LCA10 Caused by Mutations in the CEP290 Gene. |
| NCT03872479 | PHASE1/PHASE2 | UNKNOWN | Single Ascending Dose Study in Participants With LCA10 |
| NCT03913130 | PHASE1/PHASE2 | TERMINATED | Extension Study to Study PQ-110-001 (NCT03140969) |
| NCT03396042 | Not specified | COMPLETED | Natural History Study of CEP290-Related Retinal Degeneration |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| SEPOFARSEN | 2 | 4 |
| BRINRETIGENE VESGEDPARVOVEC | 2 | 1 |