Genes associated with “myoclonus

314 genes foundHPO: MyoclonusOpen Targets: Myoclonus3735 ClinVar P/LP variants
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:
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Strong Candidates

15 genes
39
score
ClinGen: DefinitiveGTR ↑

progressive myoclonic epilepsy type 7

Frequency
100%
n=16
P/LP Variants
87
OT Score
-
34
score
ClinGen: DefinitiveP2G #29GTR ↑

action myoclonus-renal failure syndrome

Frequency
100%
n=2
P/LP Variants
24
OT Score
-
3
SCN8A

sodium voltage-gated channel alpha subunit 8

33
score
ClinGen: DefinitiveGTR ↑

myoclonus, familial, 2

Frequency
100%
n=5
P/LP Variants
4
OT Score
0.27
32
score
ClinGen: DefinitiveGTR ↑

progressive myoclonic epilepsy type 6

Frequency
100%
n=6
P/LP Variants
4
OT Score
-
32
score
ClinGen: DefinitiveP2G #2GTR ↑

myoclonic epilepsy of Lafora 2

Frequency
100%
n=17
P/LP Variants
8
OT Score
-
24
score
ClinGen: DefinitiveGTR ↑

myoclonus, intractable, neonatal

Frequency
100%
n=3
P/LP Variants
8
OT Score
-
23
score
ClinGen: DefinitiveP2G #5GTR ↑
Frequency
-
P/LP Variants
22
OT Score
-
23
score
ClinGen: DefinitiveGTR ↑

Unverricht-Lundborg syndrome

Frequency
100%
n=42
P/LP Variants
3
OT Score
-
22
score

epilepsy, familial adult myoclonic, 2

Frequency
86%
n=227
P/LP Variants
2
OT Score
-
22STAMBP
#6

microcephaly-capillary malformation syndrome

22CHD2
Def
22KCNQ2
Def
20KMT2B
Def

dystonia 28, childhood-onset

20SEMA6B
Def

epilepsy, progressive myoclonic, 11

familial encephalopathy with neuroserpin inclusion bodies

Consider

51 genes
19PGM3
Def

immunodeficiency 23

17ASAH1
Def

spinal muscular atrophy-progressive myoclonic epilepsy syndrome

epilepsy, progressive myoclonic, 1B

17NEU1
Def

neuraminidase 1

17TBC1D24
Def

rolandic epilepsy-paroxysmal exercise-induced dystonia-writer's cramp syndrome

16GRN
Def
16SLC2A1
Def

solute carrier family 2 member 1

16GFAP
Def

Alexander disease

14ADCY5
Def

neurodevelopmental disorder with hyperkinetic movements and dyskinesia

14DDC
Def#15

aromatic L-amino acid decarboxylase deficiency

14ATM
Def#7

ataxia telangiectasia

14INPP5E
Def#3

Joubert syndrome 1

MYOCLONUS, FAMILIAL, 1; MYOCL1

MYOCLONUS, FAMILIAL, 2; MYOCL2

MYOCLONUS, INTRACTABLE, NEONATAL; NEIMY

13PLCB1
Def
13CERS1
Mod

progressive myoclonic epilepsy type 8

13ATP13A2
Def#20

Kufor-Rakeb syndrome

12DNM1
Def

developmental and epileptic encephalopathy, 31B

12SUCLG1
Mod
12GCSH
Def

multiple mitochondrial dysfunctions syndrome 7

11SCN1A
Def

developmental and epileptic encephalopathy 6B

11CLN8
Def

neuronal ceroid lipofuscinosis 8

11CLN6
Def

ceroid lipofuscinosis, neuronal, 6B (Kufs type)

10TCF20
Def

transcription factor 20

10GLDC
Def#18

glycine encephalopathy 1

10ADSL
Def

adenylosuccinate lyase deficiency

10CLN5
Def

neuronal ceroid lipofuscinosis 5

10ATXN2
Def

spinocerebellar ataxia type 2

10NIPBL
Def

NIPBL cohesin loading factor

9GRIN2B
Def

developmental and epileptic encephalopathy, 27

9GAMT
Def

guanidinoacetate methyltransferase deficiency

neurodevelopmental disorder with seizures and nonepileptic hyperkinetic movements

9PCDH19
Def
9SETD2
Def
9ZNF462
Def
9TBR1
Def

T-box brain transcription factor 1

9PIGA
Def

multiple congenital anomalies-hypotonia-seizures syndrome 2

GM3 synthase deficiency

neurodevelopmental disorder with epilepsy and brain atrophy

8PSAP
Def

Gaucher disease due to saposin C deficiency

8SDHD
DefSF

mitochondrial complex 2 deficiency, nuclear type 3

8FARS2
Def

combined oxidative phosphorylation defect type 14

8GRIN1
Def

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

8PPT1
Def

neuronal ceroid lipofuscinosis 1

Possible

183 genes — click to expand
8AFG3L2
Def

spastic ataxia 5

8SPTAN1
Def

spastic paraplegia 91, autosomal dominant, with or without cerebellar ataxia

8GRIA3
Def

syndromic X-linked intellectual disability 94

8TPP1
Def

neuronal ceroid lipofuscinosis 2

7CTSF
Def

neuronal ceroid lipofuscinosis 13

7HMGCL
Def

3-hydroxy-3-methylglutaric aciduria

pontocerebellar hypoplasia, type 1E

7CLN3
Def

neuronal ceroid lipofuscinosis 3

7PLA2G6
Def

autosomal recessive Parkinson disease 14

7POLG
Lim

sensory ataxic neuropathy, dysarthria, and ophthalmoparesis

7ALMS1
Def

ALMS1 centrosome and basal body associated protein

7PSAT1
Def

PSAT deficiency

7TNR
Def

tenascin R

7ABCA3
Def
7ACVRL1
Def
7ADGRV1
Def
7ATP1A2
Def
7CBS
Def
7DEPDC5
Def
7DHDDS
Def
7EPG5
Def
7FOLR1
Def
7KMT5B
Def
7RELN
Def
7THOC6
Def
7TRIO
Def
7WARS2
Def

calcium/calmodulin dependent protein kinase IV

7GRIK2
Def

intellectual disability, autosomal recessive 6

7CNTN2
Def

epilepsy, familial adult myoclonic, 5

7AHDC1
Def

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

7DNAJC5
Mod

ceroid lipofuscinosis, neuronal, 4 (Kufs type)

7KCNC3
Def

spinocerebellar ataxia type 13

mitochondrial complex I deficiency, nuclear type 18

6SLC6A5
Def

hyperekplexia 3

6ARX
Def

developmental and epileptic encephalopathy, 1

6JAG1
Def

Charcot-Marie-Tooth disease, axonal, Type 2HH

6PDHA1
Def

pyruvate dehydrogenase E1-alpha deficiency

6MOCS1
Def

sulfite oxidase deficiency due to molybdenum cofactor deficiency type A

6MOCS2
Def

sulfite oxidase deficiency due to molybdenum cofactor deficiency type B

developmental and epileptic encephalopathy, 69

6NDUFV1
Def

mitochondrial complex I deficiency, nuclear type 4

6MECR
Mod

dystonia, childhood-onset, with optic atrophy and basal ganglia abnormalities

6MT-TR
Mod
6BRAT1
Def

neonatal-onset encephalopathy with rigidity and seizures

6TNIK
Lim

TRAF2 and NCK interacting kinase

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A3

6QDPR
Def

dihydropteridine reductase deficiency

6CHMP2B
Def

frontotemporal dementia and/or amyotrophic lateral sclerosis 7

6PNPO
Def

pyridoxal phosphate-responsive seizures

6CDKL5
Def

developmental and epileptic encephalopathy, 2

6CIC
Def

intellectual disability, autosomal dominant 45

6SNCA
Def

autosomal dominant Parkinson disease 1

6GABRB2
Def

developmental and epileptic encephalopathy 92

myoclonus, familial, 1

6KCNA2
Def

developmental and epileptic encephalopathy, 32

5GPHN
Mod

hyperekplexia 1

cadherin 8

5CCNF
Lim

DYSTONIA 11, MYOCLONIC; DYT11

Dystonia 23

DYSTONIA 26, MYOCLONIC; DYT26

EPILEPSY, PROGRESSIVE MYOCLONIC, 4, WITH OR WITHOUT RENAL FAILURE; EPM4

EPILEPSY, PROGRESSIVE MYOCLONIC, 6; EPM6

GAUCHER DISEASE, TYPE III; GD3

MUSCLE HYPERTROPHY; MSLHP

5SIK1
Lim
5SLC9A6
Def

neurodegenerative disorder, X-linked, female-restricted, with parkinsonism and cognitive impairment

5PSEN2
Lim

Alzheimer disease 4

5CARS2
Mod

combined oxidative phosphorylation defect type 27

5RFT1
Mod

RFT1-congenital disorder of glycosylation

5LIAS
Mod

lipoic acid synthetase deficiency

serine/arginine repetitive matrix 4

5FUS
Def

FUS RNA binding protein

5COQ2
Def

coenzyme Q10 deficiency, primary, 1

5GTPBP2
Def

Jaberi-Elahi syndrome

5HNRNPU
Def

developmental and epileptic encephalopathy, 54

5ITPR1
Def

aniridia-cerebellar ataxia-intellectual disability syndrome

5KCNC2
Def

developmental and epileptic encephalopathy 103

5NFU1
Def

multiple mitochondrial dysfunctions syndrome 1

5SCO2
Def

cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 1

tubulin tyrosine ligase like 12

polypeptide N-acetylgalactosaminyltransferase 17

COG8-congenital disorder of glycosylation

EGF like and discoidin domains 3

heparan sulfate-glucosamine 3-sulfotransferase 4

myoclonic dystonia 26

divergent protein kinase domain 1A

epilepsy, progressive myoclonic, 12

4PET100
Def

mitochondrial complex IV deficiency, nuclear type 12

CD38 molecule

catenin alpha 2

p21 (RAC1) activated kinase 1

adhesion G protein-coupled receptor L3

4DOCK7
Def

developmental and epileptic encephalopathy, 23

polypeptide N-acetylgalactosaminyltransferase like 6

LDL receptor related protein 1B

mitochondrial complex I deficiency, nuclear type 31

growth arrest specific 7

4AP2M1
Def

intellectual developmental disorder 60 with seizures

4MYLK
Str

myosin light chain kinase

4ABCB7
Mod

X-linked sideroblastic anemia with ataxia

4NAXD
Def

NAD(P)HX dehydratase deficiency

4SATB1
Def

Kohlschutter-Tonz syndrome-like

serine and arginine rich splicing factor 6

4FRRS1L
Def

developmental and epileptic encephalopathy, 37

4POLR1C
Def

hypomyelinating leukodystrophy 11

gap junction protein gamma 1

interleukin 4

leucine rich repeat containing 70

3GNB1
Def

intellectual disability, autosomal dominant 42

3VPS13D
Lim

autosomal recessive cerebellar ataxia-saccadic intrusion syndrome

3ATP1A3
Def

developmental and epileptic encephalopathy 99

inositol-tetrakisphosphate 1-kinase

3CACNB4
Ref

episodic ataxia type 5

sterile alpha motif domain containing 5

contactin 5

3BOLA3
Def

multiple mitochondrial dysfunctions syndrome 2

3BSCL2
Def

severe neurodegenerative syndrome with lipodystrophy

3CRELD1
Lim

Jeffries-Lakhani neurodevelopmental syndrome

mitochondrial complex I deficiency, nuclear type 19

3GBA1
Def

Gaucher disease type III

3GLB1
Def

GM1 gangliosidosis type 3

3NAGA
Def

alpha-N-acetylgalactosaminidase deficiency type 1

3PURA
Def

PURA-related severe neonatal hypotonia-seizures-encephalopathy syndrome due to a point mutation

3RMND1
Def

combined oxidative phosphorylation defect type 11

3TWNK
Def

sensory ataxic neuropathy, dysarthria, and ophthalmoparesis

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