Genes associated with “hypotonia

354 genes foundHPO: HypotoniaOpen Targets: Hypotonia40305 ClinVar P/LP variants
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

19 genes
1
RPS6KA3

ribosomal protein S6 kinase A3

38
score
ClinGen: DefinitiveP2G #6GTR ↑

Coffin-Lowry syndrome

Frequency
-
P/LP Variants
20
OT Score
0.34
2
POLR2A

RNA polymerase II subunit A

34
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
1
OT Score
0.37
3
EBF3

EBF transcription factor 3

30
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
-
OT Score
0.48
4
AP2M1

adaptor related protein complex 2 subunit mu 1

29
score
ClinGen: DefinitiveGTR ↑

intellectual developmental disorder 60 with seizures

Frequency
100%
n=4
P/LP Variants
5
OT Score
0.46
5
GRM7

glutamate metabotropic receptor 7

26
score

neurodevelopmental disorder with seizures, hypotonia, and brain imaging abnormalities

Frequency
100%
n=8
P/LP Variants
17
OT Score
0.35
6
PURA

purine rich element binding protein A

26
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
14
OT Score
0.52
7
AHDC1

AT-hook DNA binding motif containing 1

26
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
3
OT Score
0.51
8
DDC

dopa decarboxylase

25
score
ClinGen: DefinitiveGTR ↑
Frequency
-
P/LP Variants
7
OT Score
0.37
24
score
ClinGen: DefinitiveP2G #13GTR ↑
Frequency
-
P/LP Variants
5
OT Score
-

procollagen-lysine,2-oxoglutarate 5-dioxygenase 1

23PEX16
Def
23GRIN1
Def

developmental and epileptic encephalopathy 101

23PEX12
Def#15

peroxisome biogenesis disorder 3A (Zellweger)

22GRIN2B
Def
21PEX5
Def#20
21RNU4-2
Str
21PEX13
Def
20PEX19
Def#1
20SPTBN4
Def

Consider

114 genes
20ARID1B
Def

transportin 2

20GAA
DefSF
19PEX26
Def
19ATRX
Def
19CASK
Def
19INPP5E
Def#11

Joubert syndrome 1

19SLC16A2
Def
19KAT6B
Def

genitopatellar syndrome

18GALE
Def
18CEP290
Def

centrosomal protein 290

18TMEM67
Def

transmembrane protein 67

18OCRL
Def
17ARSA
Def
17PMM2
Def

PMM2-congenital disorder of glycosylation

17SLC7A7
Def
17COL1A2
Def
17COL6A1
Def
17GNB1
Def

G protein subunit beta 1

17GAMT
Def

guanidinoacetate methyltransferase deficiency

16SLC19A3
Def
16SYNE1
Def

arthrogryposis multiplex congenita 3, myogenic type

16FGFR3
Def#2

thanatophoric dysplasia type 1

16PLA2G6
Def
16PEX10
Def#12

peroxisome biogenesis disorder 6A (Zellweger)

16PPP2CA
Def

protein phosphatase 2 catalytic subunit alpha

15SHANK3
Def
15COL5A1
Def
15KANSL1
Def
15DLD
Def
14MAP2K2
Def

cardiofaciocutaneous syndrome 4

14NFIX
Def
14PDHA1
Def

pyruvate dehydrogenase E1-alpha deficiency

14SATB2
Def

chromosome 2q32-q33 deletion syndrome

14MECP2
Def

X-linked intellectual disability-psychosis-macroorchidism syndrome

14RAB18
Mod

Warburg micro syndrome 3

13ALDH5A1
Def#17

succinic semialdehyde dehydrogenase deficiency

13PEX2
Def

peroxisome biogenesis disorder 5B

Ehlers-Danlos syndrome, kyphoscoliotic type, 2

13ACADVL
Def
13COL6A3
Def
13DDX3X
Def
13DNMT3A
Def
13GLDC
Def
13LAMA2
Def
13NPC1
Def
13PCCA
Def
13PIGN
Def
13SLC6A8
Def
13SLC9A6
Def
13VPS13B
Def
13MKS1
Def

Joubert syndrome 28

12COL5A2
Def
12FBN2
Def
12KCNT1
Def
12POMT2
Def

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A2

Warburg micro syndrome 2

12COQ2
Def

coenzyme Q10 deficiency, primary, 1

12ATP1A3
Def

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

12BCS1L
Def

GRACILE syndrome

12TCF20
Def

transcription factor 20

11STXBP1
Def

syntaxin binding protein 1

11CHD8
Def
11DLG4
Def
11KMT2A
Def
11MEF2C
Def
11NFIA
Def
11PLP1
Def
11POMGNT1
Def

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

11COL6A2
Def
11HNRNPU
Def
11KPTN
Def
11PCCB
Def
11POLG
Lim
11PTS
Def
11SUCLA2
Def
11PLCB1
Def

developmental and epileptic encephalopathy, 12

11FKRP
Def

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A1

mitochondrial DNA depletion syndrome 8a

11SCN8A
Def

developmental and epileptic encephalopathy, 13

11MSL3
Def

MSL complex subunit 3

10SOX5
Def

SRY-box transcription factor 5

10SERAC1
Def

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

10SMARCA5
Mod

SNF2 related chromatin remodeling ATPase 5

10HLCS
Def

holocarboxylase synthetase deficiency

10MED27
Str

mediator complex subunit 27

10NSD2
Def

Rauch-Steindl syndrome

9EFEMP1
Def

EGF-like fibulin extracellular matrix protein 1

9DHX30
Def

DExH-box helicase 30

9GBE1
Def
9MOCS1
Def
9MTR
Def
9PMPCA
Def

peptidase, mitochondrial processing subunit alpha

9SIL1
Def

SIL1 nucleotide exchange factor

free sialic acid storage disease, infantile form

9ADNP
Def
9BCL11A
Def
9MTM1
Def
9WDR26
Def
9POGZ
Def

intellectual disability-microcephaly-strabismus-behavioral abnormalities syndrome

9MAN1B1
Def

Rafiq syndrome

B4GALT1-congenital disorder of glycosylation

9DNM1L
Def

encephalopathy, lethal, due to defective mitochondrial peroxisomal fission 1

calcium voltage-gated channel subunit alpha1 D

9POU3F3
Def

POU class 3 homeobox 3

8MEGF10
Def

MEGF10-related myopathy

8GPHN
Mod

intellectual developmental disorder with impaired language and dysmorphic facies

8USP7
Def

ubiquitin specific peptidase 7

8DST
Def

dystonin

velocardiofacial syndrome

Possible

173 genes — click to expand
8MIPEP
Mod

mitochondrial intermediate peptidase

vesicle associated membrane protein 2

8HACD1
Def

congenital myopathy 11

8TRAF7
Def

cardiac, facial, and digital anomalies with developmental delay

activating signal cointegrator 1 complex subunit 1

8KAT6A
Def

autosomal dominant intellectual disability-craniofacial anomalies-cardiac defects syndrome

8NEMF
Str

nuclear export mediator factor

8NFIB
Def

macrocephaly, acquired, with impaired intellectual development

epilepsy with myoclonic atonic seizures

7GPAA1
Str

glycosylphosphatidylinositol biosynthesis defect 15

7CLPB
Lim
7ACO2
Def

infantile cerebellar-retinal degeneration

7ZFX
Str

intellectual developmental disorder, X-linked, syndromic 37

7YIF1B
Def

Kaya-Barakat-Masson syndrome

7KMT2C
Def

Kleefstra syndrome 2

7AP4M1
Def
7CSF1R
Def
7EPG5
Def
7GATM
Def
7IVD
Def
7KCTD7
Def
7LMBRD1
Def
7MAGEL2
Def
7MLC1
Def
7PNPO
Def
7PRPF8
Def
7RERE
Def
7TET3
Def

prolyl 4-hydroxylase, transmembrane

SWI/SNF related BAF chromatin remodeling complex subunit D1

transmembrane protein 222

7CHST3
Def

Larsen-like syndrome, B3GAT3 type

ARF GTPase 3

7TBCK
Def

hypotonia, infantile, with psychomotor retardation and characteristic facies 3

7GCH1
Def

GTP cyclohydrolase I deficiency with hyperphenylalaninemia

7JAG1
Def

Alagille syndrome due to a JAG1 point mutation

kinesin family member 5B

6USP9X
Def

intellectual disability, X-linked 99, syndromic, female-restricted

dedicator of cytokinesis 3

rubicon autophagy regulator

sorting nexin 27

TAM41 mitochondrial translocator assembly and maintenance homolog

6PIGB
Mod

developmental and epileptic encephalopathy, 80

6NARS1
Mod
6SUCLG1
Mod

Sp9 transcription factor

6AP4B1
Def

hereditary spastic paraplegia 47

autosomal recessive spinocerebellar ataxia 17

developmental and epileptic encephalopathy, 48

6SOX9
Def

campomelic dysplasia

6CYFIP2
Def

developmental and epileptic encephalopathy, 65

6SUFU
Def

Joubert syndrome 32

nudix hydrolase 2

siah E3 ubiquitin protein ligase 1

VISS syndrome

ATPase plasma membrane Ca2+ transporting 3

5ECHS1
Def

mitochondrial short-chain Enoyl-Coa hydratase 1 deficiency

5GMPPA
Def

alacrima, achalasia, and intellectual disability syndrome

5NT5C2
Def

hereditary spastic paraplegia 45

5SFXN4
Def

growth and developmental delay-hypotonia-vision impairment-lactic acidosis syndrome

developmental and epileptic encephalopathy, 16

neurodevelopmental disorder with motor and language delay, ocular defects, and brain abnormalities

SSR4-congenital disorder of glycosylation

5FLNA
Def

FG syndrome 2

5KDM5C
Def

syndromic X-linked intellectual disability Claes-Jensen type

5RPA1
Lim
5SMS
Def

syndromic X-linked intellectual disability Snyder type

5TIMM22
Lim
5FNIP1
Def

immunodeficiency 93 and hypertrophic cardiomyopathy

5MTRFR
Def

combined oxidative phosphorylation defect type 7

5SPEG
Def

myopathy, centronuclear, 5

5PGM3
Def

immunodeficiency 23

5SPTBN1
Str

developmental delay, impaired speech, and behavioral abnormalities

5ABCC8
Def

diabetes mellitus, permanent neonatal 3

5CPT2
Def

carnitine palmitoyl transferase II deficiency, neonatal form

5FUCA1
Def

fucosidosis

5GCSH
Def

multiple mitochondrial dysfunctions syndrome 7

5HECW2
Def

neurodevelopmental disorder with hypotonia, seizures, and absent language

5MMAA
Def

methylmalonic aciduria, cblA type

5SCN1B
Def

developmental and epileptic encephalopathy, 52

5SCN3A
Def

epilepsy, familial focal, with variable foci 4

5RFX7
Def

intellectual developmental disorder, autosomal dominant 71, with behavioral abnormalities

5KARS1
Lim

leukoencephalopathy, progressive, infantile-onset, with or without deafness

4MED23
Mod

intellectual disability, autosomal recessive 18

4MED12L
Def

Nizon-Isidor syndrome

4B9D1
Def

Joubert syndrome 27

4TNNT1
Def

nemaline myopathy 5C, autosomal dominant

combined oxidative phosphorylation deficiency 52

neurodevelopmental disorder with gait disturbance, dysmorphic facies, and behavioral abnormalities, X-linked

acrofacial dysostosis Cincinnati type

Ferguson-Bonni neurodevelopmental syndrome

4TUBB4A
Def

hypomyelinating leukodystrophy 6

4SATB1
Def

Kohlschutter-Tonz syndrome-like

4MYO1C
Dis
4MTO1
Def

mitochondrial hypertrophic cardiomyopathy with lactic acidosis due to MTO1 deficiency

4SLC1A2
Def

developmental and epileptic encephalopathy, 41

Hogue-Janssens syndrome 1

Rajab interstitial lung disease with brain calcifications 1

4SET
Def

intellectual disability, autosomal dominant 58

4AP3D1
Mod

Hermansky-Pudlak syndrome 10

4TBCE
Mod

hypoparathyroidism-retardation-dysmorphism syndrome

neurodevelopmental disorder with hypotonia, neonatal respiratory insufficiency, and thermodysregulation

neurodevelopmental disorder with eye movement abnormalities and ataxia

developmental and epileptic encephalopathy, 39

intellectual developmental disorder, autosomal recessive 83

multiple mitochondrial dysfunctions syndrome 6

intellectual disability, autosomal recessive 46

3AGK
Def

Sengers syndrome

3AP4S1
Def

hereditary spastic paraplegia 52

3BIN1
Def

myopathy, centronuclear, 2

cortical dysplasia-focal epilepsy syndrome

3DDX11
Def

Warsaw breakage syndrome

3DNM2
Def

fetal akinesia-cerebral and retinal hemorrhage syndrome

3DSE
Def

Ehlers-Danlos syndrome, musculocontractural type 2

3GFPT1
Def

congenital myasthenic syndrome 4C

3HADH
Def

3-hydroxyacyl-CoA dehydrogenase deficiency

HSD10 mitochondrial disease

3IFIH1
Def

Aicardi-Goutieres syndrome 7

3MANBA
Def

beta-mannosidosis

3MOGS
Def

MOGS-congenital disorder of glycosylation

3OSTM1
Def

autosomal recessive osteopetrosis 5

3SCN4A
Def

paramyotonia congenita of Von Eulenburg

Fontaine progeroid syndrome

3TRNT1
Def

congenital sideroblastic anemia-B-cell immunodeficiency-periodic fever-developmental delay syndrome

3VLDLR
Def

cerebellar ataxia, intellectual disability, and dysequilibrium syndrome 1

3VPS11
Def

hypomyelinating leukodystrophy 12

3WNK1
Def

neuropathy, hereditary sensory and autonomic, type 2A

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