Genes associated with “cortical visual impairment

187 genes foundHPO: Cerebral visual impairmentOpen Targets: Cerebral visual impairment1142 ClinVar P/LP variants2 PanelApp panels
Some sources returned errors (2)

omim: Error: OMIM search: 429

omimClinSyn: Error: OMIM CS: 429

How are genes scored? (0–100 composite)
-5–15
ClinGen
0–15
HPO Freq
0–15
Open Targets
0–12
Phen2Gene
0–10
ClinVar
0–8
Constraint
0–8
Dosage
0–8
OMIM CS
0–8
PanelApp
0–5
OMIM
0–5
G2P
0–4
Breadth
Tiers:Strong Candidates (≥20)Consider (≥8)Possible (≥3)
Evidence dots:
HPOClinVarPhen2GeneOpen TargetsPanelApp
hover for details

Strong Candidates

12 genes
1
GRIN2B

glutamate ionotropic receptor NMDA type subunit 2B

29
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
141
OT Score
0.00
29
score
ClinGen: DefinitivePanel: GreenP2G #11GTR ↑

developmental and epileptic encephalopathy, 62

Frequency
17%
n=6
P/LP Variants
30
OT Score
-
28
score
ClinGen: ModeratePanel: GreenP2G #42GTR ↑

Warburg micro syndrome 3

Frequency
67%
n=12
P/LP Variants
3
OT Score
-
4
GRIN1

glutamate ionotropic receptor NMDA type subunit 1

28
score
ClinGen: DefinitivePanel: GreenP2G #19GTR ↑

neurodevelopmental disorder with or without hyperkinetic movements and seizures, autosomal dominant

Frequency
-
P/LP Variants
2
OT Score
0.00
5
GNB1

G protein subunit beta 1

27
score
ClinGen: DefinitiveP2G #15GTR ↑

intellectual disability, autosomal dominant 42

Frequency
23%
n=13
P/LP Variants
2
OT Score
0.42
26
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
39
OT Score
-
23
score
ClinGen: DefinitiveP2G #22GTR ↑

developmental and epileptic encephalopathy, 46

Frequency
50%
n=2
P/LP Variants
22
OT Score
-
8
GRIK2

glutamate ionotropic receptor kainate type subunit 2

22
score
ClinGen: DefinitiveGTR ↑

neurodevelopmental disorder with impaired language and ataxia and with or without seizures

Frequency
18%
n=11
P/LP Variants
1
OT Score
0.33
22
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
18
OT Score
-
22ADNP
Def
22SPTBN4
Def#18

neurodevelopmental disorder with hypotonia, neuropathy, and deafness

22NR2F1
Def

Bosch-Boonstra-Schaaf optic atrophy syndrome

Consider

34 genes
19FOXG1
Def
19POGZ
Def

intellectual disability-microcephaly-strabismus-behavioral abnormalities syndrome

18CACNA1E
Def

developmental and epileptic encephalopathy, 69

18SCN1A
Def#1

developmental and epileptic encephalopathy, 6A

17MAPK8IP3
DefP:G

neurodevelopmental disorder with or without variable brain abnormalities; NEDBA

17TIMM8A
Def#2

deafness dystonia syndrome

congenital hypotonia, epilepsy, developmental delay, and digital anomalies

neurodevelopmental disorder with speech impairment and with or without seizures

16ACTB
DefP:G

actin beta

15DIAPH1
Def

progressive microcephaly-seizures-cortical blindness-developmental delay syndrome

14KCNQ3
Def

potassium voltage-gated channel subfamily Q member 3

14NEDD4L
DefP:G

periventricular nodular heterotopia 7

13MACF1
ModP:G

lissencephaly 9 with complex brainstem malformation

13POLG
Lim#6

mitochondrial DNA depletion syndrome 4a

developmental and epileptic encephalopathy 110

11ST3GAL5
Def#5

GM3 synthase deficiency

neurodevelopmental disorder with hypotonia and autistic features with or without hyperkinetic movements

10DOCK7
Def

developmental and epileptic encephalopathy, 23

10CEP85L
DefP:G

lissencephaly 10

10SHANK3
Def#10

Phelan-McDermid syndrome

10PIGA
Def

multiple congenital anomalies-hypotonia-seizures syndrome 2

9MED23
Mod
9CDKL5
Def

developmental and epileptic encephalopathy, 2

9PIGS
Def

glycosylphosphatidylinositol biosynthesis defect 18

9TBCK
Def

hypotonia, infantile, with psychomotor retardation and characteristic facies 3

9MEF2C
Def
9TELO2
Def

TELO2-related intellectual disability-neurodevelopmental disorder

9ASNS
Def#12

congenital microcephaly - severe encephalopathy - progressive cerebral atrophy syndrome

8PDSS2
Mod

coenzyme Q10 deficiency, primary, 3

8KAT6A
Def

autosomal dominant intellectual disability-craniofacial anomalies-cardiac defects syndrome

8HECW2
Def

neurodevelopmental disorder with hypotonia, seizures, and absent language

8SON
Def

ZTTK syndrome

8CACNA1D
Def#14

aldosterone-producing adenoma with seizures and neurological abnormalities

Possible

102 genes — click to expand

squalene synthase deficiency

8KIF5A
Def#20

myoclonus, intractable, neonatal

8PSMD12
Def#16

Stankiewicz-Isidor syndrome

mitochondrial complex I deficiency, nuclear type 19

8FGF12
Def

developmental and epileptic encephalopathy, 47

neurodevelopmental disorder with seizures and nonepileptic hyperkinetic movements

7KIF1A
Def

intellectual disability, autosomal dominant 9

7AFG2A
Def

microcephaly-intellectual disability-sensorineural hearing loss-epilepsy-abnormal muscle tone syndrome

7VPS11
Def

hypomyelinating leukodystrophy 12

7CHD3
Def

Snijders Blok-Campeau syndrome

intellectual disability, autosomal recessive 42

7GNAI1
Def
7IARS2
Def
7PGAP3
Def
7PIGY
Lim

hyperphosphatasia with intellectual disability syndrome 6

7TGFB2
Def
7LMNB1
Def

microcephaly 26, primary, autosomal dominant

7TRAF7
Def

cardiac, facial, and digital anomalies with developmental delay

7PPP3CA
Str

developmental and epileptic encephalopathy 91

7NADK2
Mod

progressive encephalopathy with leukodystrophy due to DECR deficiency

pachygyria, microcephaly, developmental delay, and dysmorphic facies, with or without seizures

7NUS1
Def

congenital disorder of glycosylation, type IAA

7AHDC1
Def

AHDC1-related intellectual disability - obstructive sleep apnea - mild dysmorphism syndrome

6DPM1
Def

congenital disorder of glycosylation type 1E

pontocerebellar hypoplasia type 9

6PIGT
Def

multiple congenital anomalies-hypotonia-seizures syndrome 3

D,L-2-hydroxyglutaric aciduria

6GABRB2
Def

developmental and epileptic encephalopathy 92

6NARS2
Def

combined oxidative phosphorylation defect type 24

6CLCN4
Def

intellectual disability, X-linked 49

developmental and epileptic encephalopathy, 73

6AFG2B
Lim

neurodevelopmental disorder with hearing loss and spasticity

6NAGA
Def

alpha-N-acetylgalactosaminidase deficiency type 1

trichohepatoneurodevelopmental syndrome

6GLUL
Mod
6GABRB1
Lim

developmental and epileptic encephalopathy, 45

microcephaly 19, primary, autosomal recessive

encephalopathy, progressive, early-onset, with episodic rhabdomyolysis

6PIGP
Mod

developmental and epileptic encephalopathy, 55

6COQ4
Def

neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome

5FDXR
Def

multiple mitochondrial dysfunctions syndrome 9b

5GTPBP2
Def

Jaberi-Elahi syndrome

5CHMP1A
#13

pontocerebellar hypoplasia type 8

5FCSK
Lim

congenital disorder of glycosylation with defective fucosylation 2

5PARS2
Mod

developmental and epileptic encephalopathy, 75

early-onset progressive encephalopathy-hearing loss-pons hypoplasia-brain atrophy syndrome

nephrotic syndrome, type 8

5OTC
DefSF

ornithine transcarbamylase

5SCN8A
Def

sodium voltage-gated channel alpha subunit 8

5EMC1
Mod

cerebellar atrophy, visual impairment, and psychomotor retardation;

5DLG4
Def

discs large MAGUK scaffold protein 4

5PRKD1
Lim
5WARS1
Lim
5YIF1B
Def

Kaya-Barakat-Masson syndrome

5COX6B1
Def

mitochondrial complex IV deficiency, nuclear type 7

5ITPR1
Def

spinocerebellar ataxia type 29

5PIGQ
Def

developmental and epileptic encephalopathy, 77

5RERE
Def

neurodevelopmental disorder with or without anomalies of the brain, eye, or heart

5SUOX
Def

isolated sulfite oxidase deficiency

neurodevelopmental disorder with microcephaly, epilepsy, and hypomyelination

Houge-Janssens syndrome 2

developmental and epileptic encephalopathy, 72

4BRAT1
Def

neonatal-onset encephalopathy with rigidity and seizures

4D2HGDH
Def

D-2-hydroxyglutaric aciduria 1

cortical dysplasia, complex, with other brain malformations 12

intellectual disability, autosomal dominant 34

4BRAF
Def

cardiofaciocutaneous syndrome 1

Joubert syndrome 14

4CC2D2A
Def

Joubert syndrome 9

mitochondrial complex I deficiency, nuclear type 11

4KMT2B
Def

intellectual developmental disorder, autosomal dominant 68

4PET100
Def

mitochondrial complex IV deficiency, nuclear type 12

progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant 4

3PIGU
Lim

glycosylphosphatidylinositol biosynthesis defect 21

hypotonia, hypoventilation, impaired intellectual development, dysautonomia, epilepsy, and eye abnormalities

3MT-ND3
Def

mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 3

3SNAP25
Def

synaptosome associated protein 25

3CLN5
Def

CLN5 lysosomal BMP synthase

3P2RY12
Def

purinergic receptor P2Y12

3CYB5R3
Def

cytochrome b5 reductase 3

3GFAP
Def

glial fibrillary acidic protein

3NEFL
Def

neurofilament light chain

3PCSK9
DefSF

proprotein convertase subtilisin/kexin type 9

3PPARG
Def

peroxisome proliferator activated receptor gamma

3SUMF1
Def

sulfatase modifying factor 1

3TREX1
Def

three prime repair exonuclease 1

Data aggregated from HPO/Monarch, ClinVar, OMIM (phenotypes + clinical synopsis), Open Targets, PanelApp, Phen2Gene, and Gene2Phenotype. Click a gene to view full details.