Genes associated with “dystonia

217 genes foundHPO: DystoniaOpen Targets: Dystonia23005 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

59 genes
1
GCH1

GTP cyclohydrolase 1

56
score
ClinGen: DefinitivePanel: Green (3)P2G #41GTR ↑

dystonia 5

Frequency
75%
n=4
P/LP Variants
6
OT Score
0.64
47
score
ClinGen: DefinitivePanel: Green (3)P2G #42GTR ↑

deafness dystonia syndrome

Frequency
100%
n=1
P/LP Variants
1
OT Score
-
3
DDC

dopa decarboxylase

44
score
ClinGen: DefinitivePanel: Green (3)P2G #27GTR ↑

aromatic L-amino acid decarboxylase deficiency

Frequency
100%
n=11
P/LP Variants
10
OT Score
0.37
4
WDR45

WD repeat domain 45

44
score
ClinGen: DefinitivePanel: Green (3)GTR ↑
Frequency
-
P/LP Variants
9
OT Score
0.61
5
ATP7B

ATPase copper transporting beta

43
score
ClinGen: DefinitiveACMG SFPanel: Green (3)P2G #4GTR ↑

Wilson disease

Frequency
-
P/LP Variants
43
OT Score
0.50
6
ATP1A3

ATPase Na+/K+ transporting subunit alpha 3

43
score
ClinGen: DefinitivePanel: Green (3)P2G #29GTR ↑

cerebellar ataxia-areflexia-pes cavus-optic atrophy-sensorineural hearing loss syndrome

Frequency
10%
n=10
P/LP Variants
2
OT Score
0.42
7
SLC2A1

solute carrier family 2 member 1

42
score
ClinGen: DefinitivePanel: Green (3)P2G #11GTR ↑

dystonia 9

Frequency
-
P/LP Variants
1
OT Score
0.55
8
FA2H

fatty acid 2-hydroxylase

40
score
ClinGen: DefinitivePanel: Green (3)P2G #6GTR ↑
Frequency
-
P/LP Variants
4
OT Score
0.46
9
PRKRA

protein activator of interferon induced protein kinase EIF2AK2

40
score

dystonia 16

Frequency
100%
n=7
P/LP Variants
9
OT Score
0.51
39ADCY5
DefP:G×3

dyskinesia with orofacial involvement, autosomal recessive

37SPR
DefP:G×3

sepiapterin reductase

36SLC19A3
DefP:G×3

solute carrier family 19 member 3

35VPS16
P:G×2

dystonia 30

35PINK1
DefP:G×3

PTEN induced kinase 1

34SERAC1
DefP:G×3

3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome

34ATM
DefP:G×3#12
33FTL
DefP:G×3

neuroferritinopathy

33SLC6A3
DefP:G×3

classic dopamine transporter deficiency syndrome

33PRKN
DefP:G×3

autosomal recessive juvenile Parkinson disease 2

32HPRT1
DefP:G×3#2

hypoxanthine phosphoribosyltransferase 1

32PANK2
P:G×3

pantothenate kinase 2

31PRRT2
DefP:G×3#8

episodic kinesigenic dyskinesia 1

31PTS
DefP:G×3

6-pyruvoyltetrahydropterin synthase

31TUBB4A
DefP:G×3

hypomyelinating leukodystrophy 6

28GNB1
DefP:G

intellectual disability, autosomal dominant 42

28GLB1
DefP:G×2

GM1 gangliosidosis type 3

27NPC1
DefP:G
27THAP1
P:G×3

torsion dystonia 6

27KMT2B
DefP:G×3

dystonia 28, childhood-onset

27DLAT
ModP:G×3

pyruvate dehydrogenase E2 deficiency

27PLA2G6
DefP:G×3

neurodegeneration with brain iron accumulation 2B

26SUOX
DefP:G×3

isolated sulfite oxidase deficiency

26NPC2
DefP:G×3

NPC intracellular cholesterol transporter 2

26SUCLA2
DefP:G×3
26GCDH
DefP:G×3

glutaryl-CoA dehydrogenase

26ATP13A2
DefP:G×3

ATPase cation transporting 13A2

26HPCA
DefP:G×3

torsion dystonia 2

25PDHA1
DefP:G×2#3

pyruvate dehydrogenase E1-alpha deficiency

25CHMP2B
DefP:G×3

frontotemporal dementia and/or amyotrophic lateral sclerosis 7

24DCAF17
DefP:G×3

DDB1 and CUL4 associated factor 17

24FBXO7
P:G×3

parkinsonian-pyramidal syndrome

24TBC1D24
DefP:G

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

24GNAO1
DefP:G×3

neurodevelopmental disorder with involuntary movements

24CYP27A1
DefP:G×3
23ARX
DefP:G×3#14

developmental and epileptic encephalopathy, 1

23COL6A3
DefP:A

dystonia 27

23MED27
StrP:G

mediator complex subunit 27

23VPS13A
P:G×3#10
23QDPR
DefP:G×3

dihydropteridine reductase deficiency

22ANO3
P:G×3

anoctamin 3

22AFG3L2
DefP:G×3

spastic ataxia 5

21ATP1A2
DefP:G×3

ATPase Na+/K+ transporting subunit alpha 2

21AUH
DefP:A×3

AU RNA binding methylglutaconyl-CoA hydratase

21HEXA
DefP:G×2
21SCN8A
DefP:G×2
21SLC16A2
DefP:G
21SLC20A2
P:G×3

solute carrier family 20 member 2

20NDUFS7
DefP:G×2

mitochondrial complex I deficiency, nuclear type 3

20PDHX
DefP:G×3

pyruvate dehydrogenase E3-binding protein deficiency

Consider

77 genes
20TOR1A
P:G×3

early-onset generalized limb-onset dystonia

19NDUFS1
DefP:G#16

mitochondrial complex I deficiency, nuclear type 5

19CHD8
Def

intellectual developmental disorder with autism and macrocephaly

19HIBCH
DefP:G×2
19DLD
DefP:G

pyruvate dehydrogenase E3 deficiency

19SYNJ1
DefP:G×3

early-onset Parkinson disease 20

18VPS13D
LimP:G

autosomal recessive cerebellar ataxia-saccadic intrusion syndrome

18ACTB
DefP:G×3

developmental malformations-deafness-dystonia syndrome

18BCAP31
DefP:G×3
17C19ORF12
DefP:G×3

neurodegeneration with brain iron accumulation 4

17TPK1
ModP:G×3

childhood encephalopathy due to thiamine pyrophosphokinase deficiency

17CASK
DefP:R
17IRF2BPL
DefP:G
16MECR
ModP:G×3

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

16NR4A2
DefP:R

late-onset Parkinson disease

16VPS41
P:G×2

VPS41 subunit of HOPS complex

16NDUFAF5
DefP:G

mitochondrial complex I deficiency, nuclear type 16

16SLC39A14
P:G×3

hypermanganesemia with dystonia 2

15GRN
DefP:G
15UQCRQ
LimP:R

mitochondrial complex III deficiency nuclear type 4

15COASY
P:G×3

neurodegeneration with brain iron accumulation 6

15VAMP2
P:G×2

vesicle associated membrane protein 2

15CDKL5
Def
15GRIN1
DefP:G

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

14CTNNB1
Def
14STXBP1
Def
14PDGFRB
P:G×3

basal ganglia calcification, idiopathic, 1

14SUCLG1
ModP:R×2
14MECP2
Def

Rett syndrome

14CACNA1E
Def

calcium voltage-gated channel subunit alpha1 E

13SHQ1
P:G

dystonia 35, childhood-onset

13CLPB
LimP:G
13NDUFB11
DefP:R
13EARS2
DefP:R×3

leukoencephalopathy-thalamus and brainstem anomalies-high lactate syndrome

13YY1
DefP:G×3

Gabriele de Vries syndrome

13GRIN2A
Def
13PURA
Def
13RS1
Def
13CAMK4
P:G

calcium/calmodulin dependent protein kinase IV

13GTPBP2
DefP:G×2

Jaberi-Elahi syndrome

13GM2A
DefP:G

Tay-Sachs disease AB variant

13LIPT1
ModP:R

lipoyl transferase 1 deficiency

12GPHN
ModP:R
12DNAJC12
DefP:G

hyperphenylalaninemia due to DNAJC12 deficiency

12PDGFB
P:G×2

basal ganglia calcification, idiopathic, 1

12PPP2R2B
P:R×2

spinocerebellar ataxia type 12

12PSEN1
DisP:R×3

presenilin 1

12SDHD
DefSFP:R

mitochondrial complex 2 deficiency, nuclear type 3

11EPG5
DefP:R
11MOCS1
DefP:R
11FITM2
DefP:G

Siddiqi syndrome

10COL6A1
Def

Ullrich congenital muscular dystrophy 1A

10EIF2AK2
P:G

dystonia 33

10SPG7
P:A
10GRIN2B
Def

developmental and epileptic encephalopathy, 27

10TNR
DefP:A

neurodevelopmental disorder, nonprogressive, with spasticity and transient opisthotonus

10KCTD17
P:G

myoclonic dystonia 26

10COX20
DefP:G

mitochondrial complex IV deficiency, nuclear type 11

neurodevelopmental disorder with impaired speech and hyperkinetic movements

9GRIA2
Def
9SNAP25
Def

synaptosome associated protein 25

9ATRX
Def
9CHD7
Def
9DHDDS
DefP:G

developmental delay and seizures with or without movement abnormalities

9DLG4
Def
9FGD1
Def
9MMADHC
DefP:R×3

methylmalonic aciduria and homocystinuria type cblD

9MOCS2
DefP:R
9PNPO
DefP:R

dystonia 22, juvenile-onset

9POLR3B
Def

hypomyelinating leukodystrophy 8 with or without oligodontia and-or hypogonadotropic hypogonadism

9CWF19L1
DefP:A

autosomal recessive spinocerebellar ataxia 17

9VPS11
DefP:A

dystonia 32

8CTC1
Def

cerebroretinal microangiopathy with calcifications and cysts 1

8VPS37A
LimP:R×3

hereditary spastic paraplegia 53

Possible

46 genes — click to expand
8NUP54
P:G

dystonia 37, early-onset, with striatal lesions

7MICU1
Def

proximal myopathy with extrapyramidal signs

7EPHB4
Def
7GRIN2D
Def
7KCTD7
Def
7SLC9A6
DefP:R

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

zinc finger protein 526

7TRPV4
Def

scapuloperoneal spinal muscular atrophy, autosomal dominant

episodic kinesigenic dyskinesia 3

7COQ9
DefP:R

encephalopathy-hypertrophic cardiomyopathy-renal tubular disease syndrome

7PIGA
DefP:R

multiple congenital anomalies-hypotonia-seizures syndrome 2

7TTC19
DefP:R

mitochondrial complex III deficiency nuclear type 2

6AARS2
DefP:R

leukoencephalopathy, progressive, with ovarian failure

ARF GTPase 3

6EXOSC3
P:R
6AARS1
Def

developmental and epileptic encephalopathy, 29

6STUB1
P:A

spinocerebellar ataxia 48

5CTCF
Def

intellectual disability-feeding difficulties-developmental delay-microcephaly syndrome

5TSFM
DefP:R

fatal mitochondrial disease due to combined oxidative phosphorylation defect type 3

spinocerebellar ataxia type 21

5ATN1
P:R×2

dentatorubral-pallidoluysian atrophy

5GCSH
Def

multiple mitochondrial dysfunctions syndrome 7

developmental and epileptic encephalopathy, 64

5TSEN2
P:R

pontocerebellar hypoplasia type 2B

5GAD1
Def

developmental and epileptic encephalopathy 89

4CARS2
Mod

combined oxidative phosphorylation defect type 27

4GRIK2
Def

intellectual disability, autosomal recessive 6

4ALS2
Def

amyotrophic lateral sclerosis type 2, juvenile

4AMPD2
P:R

pontocerebellar hypoplasia type 9

dystonia 31

4AP3D1
Mod

Hermansky-Pudlak syndrome 10

4ATP5PO
Str

mitochondrial complex V (ATP synthase) deficiency, nuclear type 7

4SPTAN1
Def

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

4NAA10
Def

Ogden syndrome

4TRPM3
Def

neurodevelopmental disorder with hypotonia, dysmorphic facies, and skeletal anomalies, with or without seizures

leukodystrophy, hypomyelinating, 15

fibrosis, neurodegeneration, and cerebral angiomatosis

3AP4B1
Def

hereditary spastic paraplegia 47

3COL4A1
Def

brain small vessel disease 1 with or without ocular anomalies

autosomal recessive limb-girdle muscular dystrophy type R18

3TRIT1
Def

combined oxidative phosphorylation deficiency 35

3WARS2
Def

neurodevelopmental disorder, mitochondrial, with abnormal movements and lactic acidosis, with or without seizures

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