Genes associated with “polymicrogyria

669 genes foundHPO: PolymicrogyriaOpen Targets: polymicrogyria1483 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:
HPOClinVarPhen2GeneOpen TargetsPanelApp
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Strong Candidates

15 genes
1
ADGRG1

adhesion G protein-coupled receptor G1

49
score
ClinGen: DefinitiveGTR ↑

polymicrogyria, bilateral perisylvian, autosomal recessive

Frequency
100%
n=5
P/LP Variants
34
OT Score
0.86
2
TUBB2B

tubulin beta 2B class IIb

39
score
ClinGen: DefinitiveP2G #42GTR ↑

complex cortical dysplasia with other brain malformations 7

Frequency
100%
n=5
P/LP Variants
3
OT Score
0.82
3
RTTN

rotatin

33
score
ClinGen: DefinitiveGTR ↑

microcephalic primordial dwarfism due to RTTN deficiency

Frequency
44%
n=9
P/LP Variants
31
OT Score
0.71
4
FIG4

FIG4 phosphoinositide 5-phosphatase

31
score
ClinGen: DefinitiveGTR ↑

bilateral parasagittal parieto-occipital polymicrogyria

Frequency
100%
n=3
P/LP Variants
4
OT Score
0.73
5
GRIN1

glutamate ionotropic receptor NMDA type subunit 1

31
score
ClinGen: DefinitiveGTR ↑

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

Frequency
-
P/LP Variants
101
OT Score
0.39
30
score
ClinGen: DefinitiveP2G #4GTR ↑

megalencephaly-capillary malformation-polymicrogyria syndrome

Frequency
-
P/LP Variants
39
OT Score
-
7
PI4KA

phosphatidylinositol 4-kinase alpha

30
score
P2G #43GTR ↑

polymicrogyria, perisylvian, with cerebellar hypoplasia and arthrogryposis

Frequency
100%
n=3
P/LP Variants
34
OT Score
0.80
27
score
ClinGen: DefinitiveGTR ↑

megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 3

Frequency
100%
n=12
P/LP Variants
6
OT Score
-
9
TUBA1A

tubulin alpha 1a

27
score
ClinGen: DefinitiveP2G #14GTR ↑

lissencephaly due to TUBA1A mutation

Frequency
-
P/LP Variants
1
OT Score
0.46
27AKT3
Def#18

megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 2

26ATP1A3
Def

developmental and epileptic encephalopathy 99

25ATP1A2
Def

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

23PIK3R2
Def

megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 1

23SUFU
Def

Joubert syndrome 32

21COL3A1
DefSF

polymicrogyria with or without vascular-type Ehlers-Danlos syndrome

Consider

60 genes
20MN1
Def

CEBALID syndrome

19PTEN
DefSF#13
18SNAP29
#9

CEDNIK syndrome

17EHMT1
Def

euchromatic histone lysine methyltransferase 1

17SMARCE1
Def
17LAMC3
Dis

occipital pachygyria and polymicrogyria

17NSDHL
Mod#3

CK syndrome

16TUBB3
Def

complex cortical dysplasia with other brain malformations 1

16SCN3A
Def

developmental and epileptic encephalopathy, 62

15RECQL4
Def#2

Baller-Gerold syndrome

15GPSM2
Def#7

Chudley-McCullough syndrome

proteasome 26S subunit, ATPase 3

14NUS1
Def

NUS1 dehydrodolichyl diphosphate synthase subunit

14OFD1
Def

OFD1 centriole and centriolar satellite protein

13POMGNT1
Def

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

FETAL AKINESIA, RESPIRATORY INSUFFICIENCY, MICROCEPHALY, POLYMICROGYRIA, AND DYSMORPHIC FACIES; FARIMPD

13FGFR3
Def

INTELLECTUAL DEVELOPMENTAL DISORDER WITH POLYMICROGYRIA AND SEIZURES; IDDPMGS

13NF2
DefSF

POLYMICROGYRIA WITH OR WITHOUT VASCULAR-TYPE EHLERS-DANLOS SYNDROME; PMGEDSV

13WDR62
Def

microcephaly 2, primary, autosomal recessive, with or without cortical malformations

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 11

12WFS1
Def

wolframin ER transmembrane glycoprotein

11ARX
Def

aristaless related homeobox

11CPT2
Def

carnitine palmitoyl transferase II deficiency, neonatal form

11DDX23
Mod

DEAD-box helicase 23

11TRRAP
Def

developmental delay with or without dysmorphic facies and autism

11SHMT2
Lim

neurodevelopmental disorder with cardiomyopathy, spasticity, and brain abnormalities

neurodevelopmental disorder with or without variable brain abnormalities; NEDBA

10LAMA2
Def

laminin subunit alpha 2

10DOCK6
Def#6

Adams-Oliver syndrome 2

10PEX5
Def
10DHCR24
Def

porencephaly 2

10HSD17B4
Def

d-bifunctional protein deficiency

intellectual disability, autosomal dominant 13

9RELN
Def
9PAH
Def

phenylalanine hydroxylase

intellectual disability, X-linked 103

9EML1
Def

band heterotopia of brain

9BMPR1A
DefSF
9DMD
Def
9NFIA
Def
9SCN2A
Def
9SLC2A1
Def
9SETD5
Def

SET domain containing 5

9DCC
Def

DCC netrin 1 receptor

9PIK3R1
Def
9KIFBP
Def

Goldberg-Shprintzen syndrome

9ATP6V0A2
Def#11

autosomal recessive cutis laxa type 2A

9PEX1
Def

peroxisome biogenesis disorder 1A (Zellweger)

9KIF26A
Str

cortical dysplasia, complex, with other brain malformations 11

8AHI1
Def

Joubert syndrome 3

8DAG1
Def

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A9

8MAP1B
Str

periventricular nodular heterotopia 9

8KIF5C
Mod

complex cortical dysplasia with other brain malformations 2

Possible

564 genes — click to expand

Warburg micro syndrome 2

8SF3B4
Def

Nager acrofacial dysostosis

8WT1
DefSF

aniridia 1

8CUL4B
Def

X-linked intellectual disability, Cabezas type

8MECP2
Def

severe neonatal-onset encephalopathy with microcephaly

8IBA57
Def

multiple mitochondrial dysfunctions syndrome 3

Knobloch syndrome 1

Warburg micro syndrome 1

7PEX26
Def

peroxisome biogenesis disorder 7A (Zellweger)

7RAB18
Mod

Warburg micro syndrome 3

7PEX14
Def

peroxisome biogenesis disorder 13A (Zellweger)

7TCTN2
Def

Joubert syndrome 24

7MBOAT7
Def

intellectual disability, autosomal recessive 57

7SMO
Def

Curry-Jones syndrome

Adams-Oliver syndrome 1

7ACTN2
Def
7AGO1
Def
7AP4M1
Def
7BSND
Def
7CDC45
Def
7COA6
Def
7COA7
Def
7COL5A2
Def
7CTPS1
Def
7EGLN1
Def
7EIF2B3
Def
7EIF2S3
Def
7GJB3
Def
7GLS
Def
7GNPAT
Def
7GP1BB
Def
7HECW2
Def
7HIBCH
Def
7KCNQ4
Def
7LYST
Def
7LZTR1
DefSF
7MARS2
Def
7MMACHC
Def
7MPL
Def
7MTR
Def
7MUTYH
DefSF
7MYSM1
Def
7PCSK9
DefSF
7PDK3
Def
7PPT1
Def
7RYR2
DefSF
7SCP2
Def
7SLC6A9
Def
7STAT1
Def
7STIL
Def
7SZT2
Def
7TPRN
Def
7TRIT1
Def
7TTN
DefSF
7TUBB4B
Def
7UROD
Def
7USP7
Def
7RAC1
Def

intellectual disability, autosomal dominant 48

7PCDH19
Def

protocadherin 19

7GNB1
Def#17

intellectual disability, autosomal dominant 42

7GPHN
Mod

sulfite oxidase deficiency due to molybdenum cofactor deficiency type C

6FKTN
Def#15

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A, 4

diaphanospondylodysostosis

neurodevelopmental disorder with brain anomalies and with or without vertebral or cardiac anomalies

6POMT1
Def#20

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

6POMT2
Def#19

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

developmental and epileptic encephalopathy, 8

6MTOR
Def

macrocephaly-intellectual disability-neurodevelopmental disorder-small thorax syndrome

6SRD5A3
Def

SRD5A3-congenital disorder of glycosylation

orofaciodigital syndrome type 6

6DEPDC5
Def

developmental and epileptic encephalopathy 111

6FOXE3
Mod
6GALNT2
Str
6GFM1
Mod
6HPDL
Mod
6LAMB1
Mod
6MACF1
Mod
6PARS2
Mod
6POLA1
Mod
6TBCE
Mod
6ZFX
Str

Long-Olsen-Distelmaier syndrome

6SCN1A
Def

sodium voltage-gated channel alpha subunit 1

6PAX6
Def

aniridia 1

6KCNQ2
Def

potassium voltage-gated channel subfamily Q member 2

6FKRP
Def

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

6PEX2
Def

peroxisome biogenesis disorder 5A (Zellweger)

6PEX13
Def

peroxisome biogenesis disorder 11A (Zellweger)

6C2CD3
Def

orofaciodigital syndrome type 14

6ARMC9
Def

Joubert syndrome 30

6CRPPA
Def

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A, 7

6LARGE1
Def

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

6SIN3A
Def

chromosome 15q24 deletion syndrome

PEHO-like syndrome

Warburg micro syndrome 4

5LAMA5
Def

laminin subunit alpha 5

microcephalic osteodysplastic primordial dwarfism type I

CORTICAL DYSPLASIA, COMPLEX, WITH OTHER BRAIN MALFORMATIONS 1; CDCBM1

5CFAP57
Lim
5COG2
Lim
5COL9A2
Lim
5DNAH7
Lim
5GREM2
Lim
5KIF4A
Lim

KNOBLOCH SYNDROME 1; KNO1

5MAST2
Lim

MUSCULAR DYSTROPHY-DYSTROGLYCANOPATHY (CONGENITAL WITH BRAIN AND EYE ANOMALIES), TYPE A, 4; MDDGA4

MEGALENCEPHALY-POLYMICROGYRIA-POLYDACTYLY-HYDROCEPHALUS SYNDROME 1; MPPH1

MEGALENCEPHALY-POLYMICROGYRIA-POLYDACTYLY-HYDROCEPHALUS SYNDROME 2; MPPH2

MEGALENCEPHALY-POLYMICROGYRIA-POLYDACTYLY-HYDROCEPHALUS SYNDROME 3; MPPH3

5PTCH2
Lim
5SAT1
Lim
5TUFM
Mod

combined oxidative phosphorylation defect type 4

5UQCRH
Lim

congenital hypotonia, epilepsy, developmental delay, and digital anomalies

5ENTPD1
Def

ectonucleoside triphosphate diphosphohydrolase 1

5FBXL4
Def

mitochondrial DNA depletion syndrome 13

5MICU1
Def

proximal myopathy with extrapyramidal signs

cerebrooculofacioskeletal syndrome 4

Galloway-Mowat syndrome 4

short-rib thoracic dysplasia 14 with polydactyly

4MAN2C1
Mod

congenital disorder of deglycosylation 2

4KCNC2
Def

potassium voltage-gated channel subfamily C member 2

4GABRG2
Def

gamma-aminobutyric acid type A receptor subunit gamma2

4CRKL
No
4EXO1
Ref
4KMO
No
4PMS1
Ref
4GABRA1
Def

gamma-aminobutyric acid type A receptor subunit alpha1

4MYH10
Def

myosin heavy chain 10

BLOC-1 related complex subunit 5

4KCNT1
Def

potassium sodium-activated channel subfamily T member 1

spinal muscular atrophy, lower extremity-predominant, 2b, prenatal onset, autosomal dominant

4NANS
Def

spondyloepimetaphyseal dysplasia, Genevieve type

4NEDD4L
Def

periventricular nodular heterotopia 7

Galloway-Mowat syndrome 2, X-linked

lissencephaly 8

combined oxidative phosphorylation defect type 30

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