Genes associated with “intellectual disability

383 genes foundHPO: Intellectual disabilityOpen Targets: Intellectual disability64056 ClinVar P/LP variants1 PanelApp panel
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

89 genes
1
POGZ

pogo transposable element derived with ZNF domain

54
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
147
OT Score
0.68
2
MED13L

mediator complex subunit 13L

54
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
203
OT Score
0.67
3
AHDC1

AT-hook DNA binding motif containing 1

53
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
3
OT Score
0.71
4
DDX3X

DEAD-box helicase 3 X-linked

51
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
3
OT Score
0.63
5
KAT6A

lysine acetyltransferase 6A

49
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
2
OT Score
0.58
46
score
ClinGen: DefinitivePanel: GreenGTR ↑

intellectual disability, autosomal dominant 9

Frequency
100%
n=15
P/LP Variants
8
OT Score
-
45
score
ClinGen: DefinitivePanel: GreenGTR ↑

X-linked intellectual disability-cerebellar hypoplasia syndrome

Frequency
100%
n=9
P/LP Variants
2
OT Score
-
44
score
ClinGen: DefinitivePanel: GreenGTR ↑

FG syndrome 4

Frequency
-
P/LP Variants
25
OT Score
-
43
score
ClinGen: DefinitivePanel: GreenGTR ↑

ADNP-related multiple congenital anomalies - intellectual disability - autism spectrum disorder

Frequency
100%
n=10
P/LP Variants
1
OT Score
-
40TRIO
DefP:G

micrognathia-recurrent infections-behavioral abnormalities-mild intellectual disability syndrome

39CTCF
DefP:G

CCCTC-binding factor

39RPS6KA3
DefP:G#17

intellectual disability, X-linked 19

38NFIX
DefP:G

Marshall-Smith syndrome

38IQSEC2
DefP:G

intellectual disability, X-linked 1

38SLC6A8
DefP:G

solute carrier family 6 member 8

38KMT2C
DefP:G

lysine methyltransferase 2C

35NAA15
DefP:G
35MYT1L
DefP:G

myelin transcription factor 1 like

35KDM6A
DefP:G

Kabuki syndrome 1

35KCNQ5
P:G

intellectual disability, autosomal dominant 46

35TAOK1
DefP:G

developmental delay with or without intellectual impairment or behavioral abnormalities

35PURA
DefP:G

purine rich element binding protein A

34AFF2
DefP:G

FRAXE intellectual disability

34ATRX
DefP:G
33KDM5C
DefP:G
33ZDHHC9
DefP:G
33TBL1XR1
DefP:G

Pierpont syndrome

33EZH2
DefP:G

Weaver syndrome

32GATAD2B
DefP:G

severe intellectual disability-poor language-strabismus-grimacing face-long fingers syndrome

32TCF20
DefP:G

transcription factor 20

32OFD1
DefP:G#7
32SYNGAP1
DefP:G

synaptic Ras GTPase activating protein 1

31AUTS2
DefP:G

autism spectrum disorder due to AUTS2 deficiency

31PHF8
DefP:G

syndromic X-linked intellectual disability Siderius type

30RAB39B
DefP:G

early-onset parkinsonism-intellectual disability syndrome

30CSNK2A1
DefP:G

Okur-Chung neurodevelopmental syndrome

30PIDD1
DefP:G

p53-induced death domain protein 1

30BCL11A
DefP:G

BCL11 transcription factor A

30PPM1D
DefP:G

intellectual developmental disorder with gastrointestinal difficulties and high pain threshold

30CDK13
DefP:G

congenital heart defects, dysmorphic facial features, and intellectual developmental disorder

29HNRNPU
DefP:G

heterogeneous nuclear ribonucleoprotein U

29CHD8
DefP:G

intellectual developmental disorder with autism and macrocephaly

29KANSL1
DefP:G

KAT8 regulatory NSL complex subunit 1

28CIC
DefP:G

capicua transcriptional repressor

28ATP8A2
DefP:G
28GMPPA
DefP:G
27SLC2A1
DefP:G

solute carrier family 2 member 1

27USP9X
DefP:G
27ALDH7A1
DefP:G
27GRIN2B
DefP:G
26SLC16A2
DefP:G
26GUSB
DefP:G

mucopolysaccharidosis type 7

26QRICH1
DefP:G

glutamine rich 1

25PPP2R1A
DefP:G

protein phosphatase 2 scaffold subunit Aalpha

25ANKRD11
DefP:G
25ARID1A
DefP:G
25CNNM2
DefP:G
25JARID2
DefP:G
25NSUN2
DefP:G
25SET
DefP:G
25PTPN11
DefP:G

LEOPARD syndrome 1

24GRIN2A
DefP:G

early-onset epileptic encephalopathy and intellectual disability due to GRIN2A mutation

24BRAF
DefP:G

cardiofaciocutaneous syndrome 1

24GABRB2
DefP:G

gamma-aminobutyric acid type A receptor subunit beta2

23ALDH18A1
DefP:G

autosomal recessive complex spastic paraplegia type 9B

23PAK3
DefP:G

intellectual disability, X-linked 30

23MECP2
DefP:G
23DHCR7
DefP:G
23EP300
DefP:G#14

Rubinstein-Taybi syndrome due to EP300 haploinsufficiency

23ARG1
DefP:G

arginase deficiency

23PAX6
DefP:R

WAGR syndrome

23GLI3
P:A
23PTEN
DefSFP:G#4

macrocephaly-autism syndrome

22TCF4
DefP:G
22CAMK2A
DefP:G

calcium/calmodulin dependent protein kinase II alpha

22MAPK1
P:G

mitogen-activated protein kinase 1

22HPRT1
DefP:G

Lesch-Nyhan syndrome

22UBE3A
DefP:G

Angelman syndrome

22SHANK3
DefP:G
21CNKSR2
DefP:G
21DYRK1A
DefP:G
21KMT2D
DefP:G
21UPF3B
DefP:G
21GAMT
DefP:G
21RPGRIP1L
DefP:G

Joubert syndrome 7

20FGFR3
DefP:R#1

severe achondroplasia-developmental delay-acanthosis nigricans syndrome

20NBEA
DefP:G

neurobeachin

20ASH1L
DefP:G

ASH1 like histone lysine methyltransferase

20SETD2
DefP:G

SET domain containing 2, histone lysine methyltransferase

Consider

205 genes
20STXBP1
DefP:G

syntaxin binding protein 1

20ZMYND11
DefP:G

zinc finger MYND-type containing 11

20KMT2E
DefP:G

lysine methyltransferase 2E (inactive)

19DLD
DefP:G
19ARX
DefP:G
19DNMT3A
DefP:G
19KCNC1
DefP:G
19PHIP
DefP:G
19PQBP1
DefP:G
19SGSH
DefP:G
19SPG11
DefP:G
19WDR45
DefP:G
19FGFR1
DefP:R

hypogonadotropic hypogonadism 2 with or without anosmia

19SMAD4
DefSFP:G

Myhre syndrome

19PPP2R5D
DefP:G

protein phosphatase 2 regulatory subunit B'delta

19CAMK2B
P:G

calcium/calmodulin dependent protein kinase II beta

19ELN
DefP:R#3

Williams syndrome

18FGFR2
DefP:A#5

Crouzon syndrome

18ARID1B
DefP:G

Coffin-Siris syndrome 1

18KDM5B
ModP:G
18RAB3GAP2
DefP:G
18TWIST1
DefP:G

Saethre-Chotzen syndrome

18SMARCE1
DefP:G
18TBC1D24
DefP:G
18NRAS
DefP:G

Noonan syndrome 6

18PIK3CA
DefP:G#16

megalencephaly-capillary malformation-polymicrogyria syndrome

18PACS1
DefP:G

phosphofurin acidic cluster sorting protein 1

18TUBA1A
DefP:G

tubulin alpha 1a

18YY1
DefP:G

YY1 transcription factor

18GRIA2
DefP:G

neurodevelopmental disorder with language impairment and behavioral abnormalities

18POU3F3
DefP:G

POU class 3 homeobox 3

18RALA
DefP:G

RAS like proto-oncogene A

17KCNH1
DefP:G

potassium voltage-gated channel subfamily H member 1

17KCNQ2
DefP:G
17KMT2A
DefP:G
17OTC
DefSFP:G
17NDUFA2
DefP:G

mitochondrial complex I deficiency, nuclear type 13

17ATM
DefP:R
17HGSNAT
DefP:G
17IDUA
DefP:G
17KMT5B
DefP:G
17NAGLU
DefP:G
17POMT1
DefP:G
17POMT2
DefP:G
17SETBP1
DefP:G

Schinzel-Giedion syndrome

17WT1
DefSFP:R
17ZEB2
DefP:G

Mowat-Wilson syndrome

17GDI1
ModP:G

intellectual disability, X-linked 41

16PCGF2
StrP:G

polycomb group ring finger 2

16COG6
DefP:G

hypohidrosis-enamel hypoplasia-palmoplantar keratoderma-intellectual disability syndrome

16MED23
ModP:G
16SLC9A6
DefP:G

Christianson syndrome

16ARFGEF1
P:G

ARF guanine nucleotide exchange factor 1

16MN1
DefP:G

CEBALID syndrome

16PEX7
DefP:G

rhizomelic chondrodysplasia punctata type 1

16KCNN2
P:G

potassium calcium-activated channel subfamily N member 2

16VAMP2
P:G

vesicle associated membrane protein 2

15ARF3
P:G

ARF GTPase 3

15KDM5A
LimP:G

lysine demethylase 5A

15TBCK
DefP:G

hypotonia, infantile, with psychomotor retardation and characteristic facies 3

15DYNC1H1
DefP:G
15GRIN1
DefP:G
15KPTN
DefP:G
15MTHFR
DefP:G
15NTRK1
DefP:G
15PCCA
DefP:G
15PCCB
DefP:G
15PNPLA6
DefP:G
15VPS13B
DefP:G
15SCN8A
DefP:G

cognitive impairment with or without cerebellar ataxia

15CAMTA1
DefP:G
15CHD2
DefP:G
15DLG4
DefP:G
15L1CAM
DefP:G
15LAMP2
DefP:G
15NF1
DefP:G
15SYNE1
DefP:R
15WDR26
DefP:G
15PGAP2
P:G

post-GPI attachment to proteins 2

15DHX30
DefP:G

neurodevelopmental disorder with severe motor impairment and absent language

15CHD5
P:G

chromodomain helicase DNA binding protein 5

15STX1A
P:G

syntaxin 1A

15USP27X
LimP:A

ubiquitin specific peptidase 27 X-linked

14NBN
DefP:A

Nijmegen breakage syndrome

14AP4E1
DefP:G

hereditary spastic paraplegia 51

14RHOBTB2
DefP:G

developmental and epileptic encephalopathy, 64

14GSS
DefP:A

glutathione synthetase deficiency with 5-oxoprolinuria

14TP53
DefSF

bone marrow failure syndrome 5

14FLNA
DefP:R

heterotopia, periventricular, X-linked dominant

14ZIC1
DefP:G

structural brain anomalies with impaired intellectual development and craniosynostosis

14ARL6
DefP:G

Bardet-Biedl syndrome 3

14TRPS1
P:R
14CEP41
P:G

Joubert syndrome 15

14IRF2BPL
DefP:G

neurodevelopmental disorder with regression, abnormal movements, loss of speech, and seizures

14NACC1
DefP:G

neurodevelopmental disorder with epilepsy, cataracts, feeding difficulties, and delayed brain myelination

13TBR1
DefP:G

intellectual developmental disorder with autism and speech delay

13CHAMP1
DefP:G

intellectual disability, autosomal dominant 40

ALOPECIA-INTELLECTUAL DISABILITY SYNDROME 1; APMR1

Alopecia-intellectual disability syndrome 2

Alopecia-intellectual disability syndrome 3

ALOPECIA-INTELLECTUAL DISABILITY SYNDROME 4; APMR4

CLEFT PALATE, CARDIAC DEFECTS, AND IMPAIRED INTELLECTUAL DEVELOPMENT; CPCMR

13DCAF17
DefP:G
13DEPDC5
DefP:G

DEVELOPMENTAL DELAY WITH VARIABLE INTELLECTUAL DISABILITY AND DYSMORPHIC FACIES; DIDDF

13ERLIN2
DefP:G
13FARS2
DefP:G
13GAN
DefP:G
13GMPPB
DefP:G
13HNF1B
DefP:A

INTELLECTUAL DISABILITY AND MYOPATHY SYNDROME; IDMYS

13MAGEL2
DefP:G
13MAN2B1
DefP:G
13MLC1
DefP:G

INTELLECTUAL DEVELOPMENTAL DISORDER, AUTOSOMAL RECESSIVE 65; MRT65

INTELLECTUAL DISABILITY-HYPOTONIC FACIES SYNDROME, X-LINKED, 1; MRXHF1

INTELLECTUAL DEVELOPMENTAL DISORDER, X-LINKED, SYNDROMIC, SNIJDERS BLOK TYPE; MRXSSB

13NGLY1
DefP:G
13NT5C2
DefP:G
13PHGDH
DefP:G
13PMM2
DefP:G
13POMGNT1
DefP:G
13RERE
DefP:G

SHORT STATURE, BRACHYDACTYLY, IMPAIRED INTELLECTUAL DEVELOPMENT, AND SEIZURES; SBIDDS

13SOS2
DefP:G
13SPTBN4
DefP:G
13TAT
DefP:G
13TET3
DefP:G
13TLK2
DefP:G

INTELLECTUAL DEVELOPMENTAL DISORDER, X-LINKED 1; XLID1

13RUSC2
ModP:A

intellectual disability, autosomal recessive 61

13CUX2
ModP:G

developmental and epileptic encephalopathy, 67

13AIFM1
DefP:G

spondyloepimetaphyseal dysplasia, Bieganski type

13CEP104
DefP:G

intellectual developmental disorder, autosomal recessive 77

12GNAO1
DefP:G

neurodevelopmental disorder with involuntary movements

12TUBB2B
DefP:G

complex cortical dysplasia with other brain malformations 7

12CAPN3
Def
12CUL3
DefP:G

neurodevelopmental disorder with or without autism or seizures

12ATP1A2
DefP:G

alternating hemiplegia of childhood 1

12ACTB
DefP:G

Baraitser-Winter syndrome 1

12PIGV
ModP:G

hyperphosphatasia with intellectual disability syndrome 1

12PPP1R12A
StrP:G
12SLC6A1
P:G
12MSL2
P:G

Karayol-Borroto-Haghshenas neurodevelopmental syndrome

12GABRA1
DefP:G

developmental and epileptic encephalopathy, 19

12STIL
DefP:G

microcephaly 7, primary, autosomal recessive

12GRIA4
ModP:G

neurodevelopmental disorder with or without seizures and gait abnormalities

12CDH11
DefP:G

Teebi hypertelorism syndrome 2

11LAMB1
ModP:G

cobblestone lissencephaly without muscular or ocular involvement

11SCAF4
DefP:G

Fliedner-Zweier syndrome

11ACTL6B
P:G

intellectual developmental disorder with severe speech and ambulation defects

11PHF6
DefP:G

Borjeson-Forssman-Lehmann syndrome

11PIGW
LimP:G

hyperphosphatasia with intellectual disability syndrome 5

11PRICKLE2
LimP:G
11SMARCB1
DefP:G

intellectual disability, autosomal dominant 15

11AP1G1
StrP:G

Usmani-Riazuddin syndrome, autosomal recessive

11FAR1
ModP:G

fatty acyl-CoA reductase 1 deficiency

11BBS9
DefP:G

Bardet-Biedl syndrome 9

11PEX11B
DefP:G

peroxisome biogenesis disorder 14B

11MAN2C1
ModP:G

congenital disorder of deglycosylation 2

10TRMT10A
P:G

microcephaly, short stature, and impaired glucose metabolism 1

10EXTL3
ModP:G

immunoskeletal dysplasia with neurodevelopmental abnormalities

10ROBO1
LimP:G

neurooculorenal syndrome

10CENPF
P:G
10LRP2
No P:G
10SOX4
P:G
10SUZ12
P:G
10TANGO2
P:G
10RNU4ATAC
DefP:A

Lowry-Wood syndrome

10SEL1L
P:G

neurodevelopmental disorder with hypotonia, poor growth, dysmorphic facies, and agammaglobulinemia

neurodevelopmental disorder with speech impairment and with or without seizures

9TAF4
P:G

intellectual developmental disorder, autosomal dominant 73

epilepsy, early-onset, 3, with or without developmental delay

9ERI1
P:G

Hoxha-Aliu syndrome

9TIAM1
P:G

neurodevelopmental disorder with language delay and seizures

9AFG2A
Def
9PRICKLE1
LimP:R
9ARFGEF2
DefP:G

periventricular heterotopia with microcephaly, autosomal recessive

9BCKDHA
DefP:G

maple syrup urine disease type 1A

9BCS1L
DefP:G

mitochondrial complex III deficiency nuclear type 1

9CACNA1C
DefP:G

neurodevelopmental disorder with hypotonia, language delay, and skeletal defects with or without seizures

9EIF2S3
DefP:G

MEHMO syndrome

9ESCO2
DefP:G

Juberg-Hayward syndrome

9FKRP
DefP:G

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

9FKTN
DefP:G

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

9NAGA
DefP:G

alpha-N-acetylgalactosaminidase deficiency type 1

9TCTN3
DefP:G

Joubert syndrome 18

9TREX1
DefP:G

Aicardi-Goutieres syndrome 1

9ZSWIM6
DefP:G

acromelic frontonasal dysostosis

9NDUFA1
ModP:G

mitochondrial complex I deficiency, nuclear type 12

9ARV1
P:G

developmental and epileptic encephalopathy, 38

9KCNK4
ModP:A

facial dysmorphism, hypertrichosis, epilepsy, intellectual/developmental delay, and gingival overgrowth syndrome

9PRDM13
P:G

cerebellar dysfunction, impaired intellectual development, and hypogonadotropic hypogonadism

9POLA1
ModP:G

X-linked reticulate pigmentary disorder

9COPB2
P:G

osteoporosis, childhood- or juvenile-onset, with developmental delay

9CTBP1
P:G

hypotonia, ataxia, developmental delay, and tooth enamel defect syndrome

velocardiofacial syndrome

neurodevelopmental disorder with seizures and gingival overgrowth

8HNRNPC
P:G

intellectual developmental disorder, autosomal dominant 74

8ARCN1
P:G

short stature, rhizomelic, with microcephaly, micrognathia, and developmental delay

8DCHS1
ModP:G

van Maldergem syndrome 1

8DNMT3B
StrP:G

immunodeficiency-centromeric instability-facial anomalies syndrome 1

8ZFX
StrP:G

intellectual developmental disorder, X-linked, syndromic 37

Possible

75 genes — click to expand
8COG3
LimP:A

congenital disorder of glycosylation, type IIbb

8FANCB
DefP:R

Fanconi anemia complementation group B

8NUP107
P:A

Galloway-Mowat syndrome 7

8IPO8
P:A

VISS syndrome

7TCF12
DefP:R

hypogonadotropic hypogonadism 26 with or without anosmia

7WDR11
P:A

intellectual developmental disorder, autosomal recessive 78

7RPS19
DefP:R

Diamond-Blackfan anemia 1

Alfadhel syndrome

7TECR
LimP:R

intellectual disability, autosomal recessive 14

7VPS13D
Lim
7PGK1
P:G

glycogen storage disease due to phosphoglycerate kinase 1 deficiency

7ACAT1
DefP:A

beta-ketothiolase deficiency

7EYA1
DefP:R

otofaciocervical syndrome 1

7FANCA
DefP:A

Fanconi anemia complementation group A

7XPA
DefP:A

xeroderma pigmentosum group A

7KLHL7
P:G

PERCHING syndrome

7CEP63
P:A

Seckel syndrome 6

7CSTF2
P:A

intellectual developmental disorder, X-linked 113

7TOR1A
P:G

arthrogryposis multiplex congenita 5

6AP5Z1
DefP:R

hereditary spastic paraplegia 48

intellectual disability, autosomal recessive 66

6TRIM37
DefP:R

mulibrey nanism

6ATXN7
P:R

hydrocephalus, nonsyndromic, autosomal recessive 1

6ELP4
Mod

microcephaly, epilepsy, and diabetes syndrome 1

6NARS1
Mod
6NTRK2
P:G

obesity, hyperphagia, and developmental delay

microcephaly, short stature, and impaired glucose metabolism 2

6RNF135
P:R
6SLC5A6
P:G

peripheral motor neuropathy, childhood-onset, biotin-responsive

6SNAP29
P:G

CEDNIK syndrome

6WNK3
P:G

Prieto syndrome

6LRRC8C
P:A

telangiectasia, impaired intellectual development, microcephaly, metaphyseal dysplasia, eye abnormalities, and short stature

6AFG3L2
DefP:R

optic atrophy 12

5COX7B
P:A

linear skin defects with multiple congenital anomalies 1

intellectual disability, autosomal recessive 59

EPILEPSY, X-LINKED 1, WITH VARIABLE LEARNING DISABILITIES AND BEHAVIOR DISORDERS; EPILX1

5ERCC4
DefP:R

xeroderma pigmentosum group F

5LYST
DefP:R

Chediak-Higashi syndrome

5TK2
DefP:R

mitochondrial DNA depletion syndrome, myopathic form

4KLF13
Mod

chromosome 15q13.3 microdeletion syndrome

4POU1F1
P:A

pituitary hormone deficiency, combined, 1

microcephaly, developmental delay, and brittle hair syndrome

3FCSK
Lim

congenital disorder of glycosylation with defective fucosylation 2

intellectual developmental disorder, autosomal recessive 83

3YME1L1
Lim

optic atrophy 11

3GJA1
LimP:R

craniometaphyseal dysplasia, autosomal recessive

3STAC3
Def

Bailey-Bloch congenital myopathy

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