Genes associated with “microcephaly

385 genes foundHPO: MicrocephalyOpen Targets: microcephaly28440 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

54 genes
1
CEP152

centrosomal protein 152

51
score
ClinGen: DefinitivePanel: GreenP2G #30GTR ↑
Frequency
-
P/LP Variants
52
OT Score
0.80
2
KIF11

kinesin family member 11

50
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
1
OT Score
0.82
3
TUBGCP6

tubulin gamma complex component 6

47
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
49
OT Score
0.79
4
ASPM

assembly factor for spindle microtubules

46
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
4
OT Score
0.79
46
score
ClinGen: DefinitivePanel: GreenP2G #39GTR ↑

Cornelia de Lange syndrome 1

Frequency
85%
n=46
P/LP Variants
18
OT Score
-
6
PNKP

polynucleotide kinase 3'-phosphatase

44
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
51
OT Score
0.81
7
NDE1

nudE neurodevelopment protein 1

44
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
54
OT Score
0.81
8
WDR62

WD repeat domain 62

44
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
2
OT Score
0.82
9
MCPH1

microcephalin 1

43
score
ClinGen: DefinitivePanel: GreenGTR ↑
Frequency
-
P/LP Variants
2
OT Score
0.81
43DYRK1A
DefP:G

DYRK1A-related intellectual disability syndrome

41CDK5RAP2
DefP:G

CDK5 regulatory subunit associated protein 2

41STIL
DefP:G

STIL centriolar assembly protein

37KNL1
P:G

microcephaly 4, primary, autosomal recessive

35RNU4ATAC
DefP:G

Lowry-Wood syndrome

35UBE3A
DefP:A

Angelman syndrome

33LMNB1
DefP:G

lamin B1

32PCNT
DefP:G

pericentrin

32PPFIBP1
P:G

neurodevelopmental disorder with seizures, microcephaly, and brain abnormalities

32ANKLE2
P:G

microcephaly 16, primary, autosomal recessive

32SASS6
P:G

microcephaly 14, primary, autosomal recessive

31NBN
DefP:G#3
30SLC9A6
DefP:G

Christianson syndrome

30MECP2
DefP:G

syndromic X-linked intellectual disability Lubs type

30DHCR7
DefP:G#5
29KIF14
P:G

kinesin family member 14

29SMC1A
DefP:G#17
28ATRX
DefP:G#2

alpha thalassemia-X-linked intellectual disability syndrome

28FANCG
DefP:G

Fanconi anemia complementation group G

27QARS1
Def

diffuse cerebral and cerebellar atrophy - intractable seizures - progressive microcephaly syndrome

27HDAC8
DefP:G#15

Cornelia de Lange syndrome 5

27SLX4
DefP:G

Fanconi anemia complementation group P

26ZEB2
DefP:G
26BRIP1
DefP:G
26FANCD2
DefP:G
26PQBP1
DefP:G
26FANCC
DefP:G
26ERCC4
DefP:G

xeroderma pigmentosum group F

26FANCI
DefP:G
26RAD51C
DefP:A
25FANCA
DefP:G
25BUB1
LimP:G

microcephaly 30, primary, autosomal recessive

24SLC25A19
LimP:G

solute carrier family 25 member 19

24FANCL
DefP:G
23FANCF
DefP:G
23VARS1
Def
23ZNF335
P:G

zinc finger protein 335

22SLC1A4
DefP:G

spastic tetraplegia-thin corpus callosum-progressive postnatal microcephaly syndrome

22FANCE
DefP:G
22RTTN
DefP:G

rotatin

21ATP1A2
DefP:G

ATPase Na+/K+ transporting subunit alpha 2

21FANCM
DefP:R
21CASK
DefP:G
21NHEJ1
DefP:G
20VPS13B
Def#8

vacuolar protein sorting 13 homolog B

Consider

100 genes
20PAH
Def
19POGZ
DefP:G
18DIAPH1
DefP:G
18TRIO
DefP:G
18AKT3
DefP:A
18KMT2D
Def

Kabuki syndrome 1

18TUBGCP4
P:G

tubulin gamma complex component 4

17CDK6
P:R

cyclin dependent kinase 6

17CTNNB1
DefP:G
17MFSD2A
P:G

MFSD2 lysolipid transporter A, lysophospholipid

17CEP135
P:G

centrosomal protein 135

17LMNB2
P:G

lamin B2

17SMARCB1
Def#11

intellectual disability, autosomal dominant 15

17NCAPD3
P:G

non-SMC condensin II complex subunit D3

16MORC2
DefP:G

MORC family CW-type zinc finger 2

16NCAPD2
P:G

non-SMC condensin I complex subunit D2

16SMG8
DefP:G

SMG8 nonsense mediated mRNA decay factor

16AFG2A
Def

microcephaly-intellectual disability-sensorineural hearing loss-epilepsy-abnormal muscle tone syndrome

16GRIN2B
Def

developmental and epileptic encephalopathy, 27

16GPT2
DefP:G

glutamate pyruvate transaminase 2 deficiency

15WDR11
P:G

WD repeat domain 11

15ARF3
P:G

ARF GTPase 3

15DDX11
DefP:G
15TRMT10A
P:G
15NARS1
Mod
15RAD50
RefP:G
15AP4M1
DefP:A
15BRD4
DefP:A
15CTCF
DefP:G
15EFTUD2
DefP:G
15ASXL1
Def

angiopoietin 2

14GATA4
Def
14COPB2
P:R

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

14ASXL3
Def
14PHC1
P:R

polyhomeotic homolog 1

14CRNKL1
P:G
14IGF1R
P:G

growth delay due to insulin-like growth factor I resistance

14TNPO2
P:G

transportin 2

13RPS6KA3
Def#19

Coffin-Lowry syndrome

13WDFY3
DefP:R
13XRCC4
LimP:G
13ERCC2
Def#16

cerebrooculofacioskeletal syndrome 2

13PLK4
P:G

polo like kinase 4

13CCND2
DefP:G
13EPG5
Def

MICROCEPHALY WITH OR WITHOUT CHORIORETINOPATHY, LYMPHEDEMA, OR IMPAIRED INTELLECTUAL DEVELOPMENT; MCLMR

INTELLECTUAL DEVELOPMENTAL DISORDER WITH MICROCEPHALY AND PONTINE AND CEREBELLAR HYPOPLASIA; MICPCH

13MTR
Def
13PRUNE1
P:G

neurodevelopmental disorder with microcephaly, hypotonia, and variable brain anomalies

12KCNT1
Def
12SUOX
Def
12RPL10
DefP:G

intellectual disability, X-linked, syndromic, 35

12MCM7
P:A

minichromosome maintenance complex component 7

microcephaly 25, primary, autosomal recessive

12KMT2B
DefP:G

dystonia 28, childhood-onset

11RARS2
Def

pontocerebellar hypoplasia type 6

11PARN
Def

dyskeratosis congenita, autosomal recessive 6

11TAF13
P:A

TATA-box binding protein associated factor 13

11DYNC1H1
Def
11MTHFR
Def
11PHGDH
Def
11UGP2
P:G

developmental and epileptic encephalopathy, 83

11ACTG1
Def

Baraitser-winter syndrome 2

autosomal recessive cutis laxa type 2A

11BCOR
Def

microphthalmia, syndromic 2

10NCAPH
P:R

non-SMC condensin I complex subunit H

10PORCN
Def

focal dermal hypoplasia

neurodevelopmental disorder with microcephaly, short stature, and speech delay

10CENPF
P:G
10DYNC1I2
P:G
10SLF2
P:G
10SUCLG1
Mod
10AP4B1
DefP:A

hereditary spastic paraplegia 47

10ACO2
Def

infantile cerebellar-retinal degeneration

9FBXL4
Def

mitochondrial DNA depletion syndrome 13

9XPA
Def
9BCL11A
Def
9MEIS2
Def
9NHS
Def
9ZBTB18
Def
9CTU2
P:G

microcephaly, facial dysmorphism, renal agenesis, and ambiguous genitalia syndrome

9TCTN2
Def

Meckel syndrome, type 8

9RRP7A
P:A

microcephaly 28, primary, autosomal recessive

9CDC45
Def

Meier-Gorlin syndrome 7

9NSDHL
Mod

CK syndrome

8PPP2CA
Def

Houge-Janssens syndrome 3

8BCAP31
Def

severe motor and intellectual disabilities-sensorineural deafness-dystonia syndrome

8DNA2
P:G

Seckel syndrome 8

8GTF2E2
P:G

trichothiodystrophy 6, nonphotosensitive

cytoplasmic linker associated protein 1

8CPSF3
P:A

neurodevelopmental disorder with microcephaly, hypotonia, nystagmus, and seizures

8GMNN
ModP:G

Meier-Gorlin syndrome 6

8MED17
P:A
8POLH
Def

xeroderma pigmentosum variant type

Possible

174 genes — click to expand
8PCNA
Lim

ataxia-telangiectasia-like disorder 2

7OTUD6B
Def

intellectual developmental disorder with dysmorphic facies, seizures, and distal limb anomalies

7NUS1
Def

congenital disorder of glycosylation, type IAA

7NUP133
Mod

Galloway-Mowat syndrome 8

lysine acetyltransferase 5

7COG6
Def

COG6-congenital disorder of glycosylation

leukocyte adhesion deficiency type II

7COG5
Def
7HIBCH
Def
7KCTD7
Def
7LMBRD1
Def
7MMACHC
Def
7MTRR
Def
7PACS1
Def
7UBTF
Def
7ZNF668
P:A

neurodevelopmental disorder with poor growth, large ears, and dysmorphic facies

7ATP9A
P:A

neurodevelopmental disorder with poor growth and behavioral abnormalities

7FILIP1
P:A

neuromuscular disorder, congenital, with dysmorphic facies

6CENPE
LimP:R

microcephaly 13, primary, autosomal recessive

6EBF3
Def

hypotonia, ataxia, and delayed development syndrome

6NBEA
Def

neurodevelopmental disorder with or without early-onset generalized epilepsy

6FAT1
Str
6MED27
Str
6PSPH
Mod
6UFM1
P:G

leukodystrophy, hypomyelinating, 14

6FGD1
Def

Aarskog-Scott syndrome, X-linked

6SPTAN1
Def

developmental and epileptic encephalopathy, 5

6TBCD
Def

early-onset progressive diffuse brain atrophy-microcephaly-muscle weakness-optic atrophy syndrome

neurodevelopmental disorder with microcephaly, arthrogryposis, and structural brain anomalies

5FGF12
Def

developmental and epileptic encephalopathy, 47

5GMPPB
Def

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A14

autosomal recessive limb-girdle muscular dystrophy type R18

5KANSL1
Def

Koolen-de Vries syndrome

5PGAP3
Def

hyperphosphatasia with intellectual disability syndrome 4

SSR4-congenital disorder of glycosylation

5BCKDK
Def

branched-chain keto acid dehydrogenase kinase deficiency

5FTH1
Mod

neurodegeneration with brain iron accumulation 9

5CNOT1
Def

holoprosencephaly 12 with or without pancreatic agenesis

5EXT2
Def

seizures-scoliosis-macrocephaly syndrome

MICROCEPHALY, AMISH TYPE; MCPHA

5SHANK3
Def

Phelan-McDermid syndrome

5SOX7
Lim
5ACADVL
Def

very long chain acyl-CoA dehydrogenase deficiency

5ALG12
Def

ALG12-congenital disorder of glycosylation

5GRIK2
Def

neurodevelopmental disorder with impaired language and ataxia and with or without seizures

5SDHD
DefSF

mitochondrial complex 2 deficiency, nuclear type 3

5ZIC1
Def

structural brain anomalies with impaired intellectual development and craniosynostosis

5OTUD5
Mod

multiple congenital anomalies-neurodevelopmental syndrome, X-linked

4TPRKB
P:R

Galloway-Mowat syndrome 5

4CNNM2
Def

hypomagnesemia, seizures, and intellectual disability 1

4SATB1
Def

Kohlschutter-Tonz syndrome-like

4MBOAT7
Def

intellectual disability, autosomal recessive 57

4FKTN
Def

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

4GRIN2D
Def

developmental and epileptic encephalopathy, 46

4TANC2
Def

intellectual developmental disorder with autistic features and language delay, with or without seizures

4VPS33A
Def

mucopolysaccharidosis-plus syndrome

4BLK
Ref
4NSMCE2
ModP:R

Seckel syndrome 10

leukodystrophy and acquired microcephaly with or without dystonia;

intellectual developmental disorder, X-linked 112

4HCN1
Def

generalized epilepsy with febrile seizures plus, type 10

4IFIH1
Def

Aicardi-Goutieres syndrome 7

4AP3D1
Mod

Hermansky-Pudlak syndrome 10

Mullegama-Klein-Martinez syndrome

4TET3
Def

Beck-Fahrner syndrome

3CRELD1
Lim

Jeffries-Lakhani neurodevelopmental syndrome

hypomyelinating leukodystrophy 10

TMEM165-congenital disorder of glycosylation

neurodevelopmental disorder with motor and speech delay and behavioral abnormalities

3GFM1
Mod

hepatoencephalopathy due to combined oxidative phosphorylation defect type 1

developmental and epileptic encephalopathy, 76

neurodevelopmental disorder with ataxic gait, absent speech, and decreased cortical white matter

3ACE
Def

renal tubular dysgenesis of genetic origin

3ALG1
Def

ALG1-congenital disorder of glycosylation

3COX15
Def

cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 2

3CYB5R3
Def

methemoglobinemia due to deficiency of methemoglobin reductase

3DGUOK
Def

mitochondrial DNA depletion syndrome 3 (hepatocerebral type)

3DHX30
Def

neurodevelopmental disorder with severe motor impairment and absent language

3DLD
Def

pyruvate dehydrogenase E3 deficiency

3FLCN
Def

Potocki-Lupski syndrome

3GNAO1
Def

neurodevelopmental disorder with involuntary movements

3KIFBP
Def

Goldberg-Shprintzen syndrome

3NAA10
Def

Ogden syndrome

3NDUFS1
Def

mitochondrial complex I deficiency, nuclear type 5

3NDUFV2
Def

mitochondrial complex I deficiency, nuclear type 7

3QDPR
Def

dihydropteridine reductase deficiency

3STAC3
Def

Bailey-Bloch congenital myopathy

3TUBB4A
Def

hypomyelinating leukodystrophy 6

3UBE3B
Def

oculocerebrofacial syndrome, Kaufman type

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