Genes associated with “neonatal seizures

181 genes foundHPO: Neonatal deathOpen Targets: neonatal-onset encephalopathy with rigidity and seizures948 ClinVar P/LP variants3 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

10 genes
37
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
27
OT Score
-
36
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
2
OT Score
-
3
BRAT1

BRCA1 associated ATM activator 1

30
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
-
OT Score
0.78
27
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
3
OT Score
-
25
score
ClinGen: DefinitivePanel: GreenGTR ↑

holoprosencephaly 12 with or without pancreatic agenesis

Frequency
33%
n=3
P/LP Variants
1
OT Score
-
22
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
352
OT Score
-
21
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
2
OT Score
-
20
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
1
OT Score
-
20
score
ClinGen: DefinitiveGTR ↑

fetal akinesia, respiratory insufficiency, microcephaly, polymicrogyria, and dysmorphic facies

Frequency
100%
n=2
P/LP Variants
-
OT Score
-
20GAD1
Def

developmental and epileptic encephalopathy 89

Consider

26 genes
18GRIN2B
Def
16EEF1A2
Def
13ATP6AP1
Def
13ECHS1
Def
13KCNQ3
Def
11PEX10
DefP:G

peroxisome biogenesis disorder 6A (Zellweger)

11PEX14
DefP:G

peroxisome biogenesis disorder 13A (Zellweger)

9TARS2
DefP:A

combined oxidative phosphorylation defect type 21

9DIAPH1
Def
9GALNT3
Def
9RTEL1
Def
9TTC21B
Def
9CREBBP
Def
9FLNA
Def
9IKBKG
Def
9L1CAM
Def
9MECP2
Def
9MEF2C
Def
9NBEA
Def
9PKHD1
DefP:G

polycystic kidney disease 4

8DSP
DefSF

lethal acantholytic epidermolysis bullosa

8GLUL
Mod

congenital brain dysgenesis due to glutamine synthetase deficiency

8BCS1L
DefP:A

mitochondrial complex III deficiency nuclear type 1

8NPHP3
DefP:A

renal-hepatic-pancreatic dysplasia 1

Possible

117 genes — click to expand

restrictive dermopathy 1

7ABCA3
Def

interstitial lung disease due to ABCA3 deficiency

7FAT4
Def
7G6PD
Def
7GLDC
Def
7HCFC1
Def
7KCNQ1
DefSF
7KIF5A
Def
7KMT5B
Def
7MOCS1
Def
7NAA10
Def
7OPN1LW
Def
7OPN1MW
Def
7PROS1
Def
7RPL10
Def
7SUOX
Def
7ZNF292
Def
7FANCB
Def

VACTERL association, X-linked, with or without hydrocephalus

7WT1
DefSF

Denys-Drash syndrome

6RARB
Def

microphthalmia, syndromic 12

6GDI1
Mod
6KDM4B
Str
6RAD51C
Def

Fanconi anemia complementation group O

6RBM10
Def

TARP syndrome

6TMEM70
Def

mitochondrial complex V (ATP synthase) deficiency, nuclear type 2

6LMOD3
Def

nemaline myopathy 10

6NFU1
Def

multiple mitochondrial dysfunctions syndrome 1

5ACTA1
Def

congenital myopathy 2b, severe infantile, autosomal recessive

5EFEMP2
Def

cutis laxa, autosomal recessive, type 1B

5INTU
Def

short-rib thoracic dysplasia 20 with polydactyly

5LMOD2
Def

cardiomyopathy, dilated, 2G

5NDUFS6
Def

mitochondrial complex I deficiency, nuclear type 9

5PLEC
Def

epidermolysis bullosa simplex 5C, with pyloric atresia

5SFTPB
Def

surfactant metabolism dysfunction, pulmonary, 1

pontocerebellar hypoplasia, hypotonia, and respiratory insufficiency syndrome, neonatal lethal

5GPHN
Mod

sulfite oxidase deficiency due to molybdenum cofactor deficiency type C

5SUCLG1
Mod

mitochondrial DNA depletion syndrome 9

alveolar capillary dysplasia with misalignment of pulmonary veins

5GBA1
Def

Gaucher disease perinatal lethal

5HSPG2
Def

Silverman-Handmaker type dyssegmental dysplasia

5MOCS2
Def

sulfite oxidase deficiency due to molybdenum cofactor deficiency type B

mitochondrial complex I deficiency, nuclear type 15

mitochondrial complex I deficiency, nuclear type 30

5PET100
Def

mitochondrial complex IV deficiency, nuclear type 12

5SDHD
DefSF

mitochondrial complex 2 deficiency, nuclear type 3

Fontaine progeroid syndrome

5NEB
Def

arthrogryposis multiplex congenita 6

mitochondrial DNA depletion syndrome 12A (cardiomyopathic type), autosomal dominant

4AARS2
Def

combined oxidative phosphorylation defect type 8

4FGA
Def

congenital afibrinogenemia

4FGB
Def

congenital afibrinogenemia

4FGG
Def

congenital afibrinogenemia

4ALG8
Def

ALG8-congenital disorder of glycosylation

4PHGDH
Def

Neu-Laxova syndrome 1

4MBTPS2
Def

IFAP syndrome 1, with or without BRESHECK syndrome

4CTSD
Def

neuronal ceroid lipofuscinosis 10

4FGFR3
Def

severe achondroplasia-developmental delay-acanthosis nigricans syndrome

4HADHB
Def

mitochondrial trifunctional protein deficiency 2

4HTRA2
Def

3-methylglutaconic aciduria type 8

4RMND1
Def

combined oxidative phosphorylation defect type 11

4SCN4A
Def

congenital myopathy 22A, classic

4EDNRB
Mod

ABCD syndrome

mitochondrial complex 1 deficiency, nuclear type 35

VACTERL association, X-linked, with or without hydrocephalus

4AP1S1
Def

MEDNIK syndrome

4NDUFS4
Def

NADH:ubiquinone oxidoreductase subunit S4

3PPT1
Def

palmitoyl-protein thioesterase 1

3GET3
Lim

cardiomyopathy, dilated, 2H

3ETFA
Def

multiple acyl-CoA dehydrogenase deficiency

3ETFB
Def

multiple acyl-CoA dehydrogenase deficiency

3ETFDH
Def

multiple acyl-CoA dehydrogenase deficiency

3FLNB
Def

atelosteogenesis type I

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