Genes associated with “hypertrichosis

250 genes foundHPO: HypertrichosisOpen Targets: hypertrichosis2139 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:
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hover for details

Strong Candidates

15 genes
38
score
ClinGen: DefinitiveP2G #5GTR ↑

Cornelia de Lange syndrome 3

Frequency
78%
n=18
P/LP Variants
20
OT Score
-
37
score
ClinGen: DefinitiveP2G #1GTR ↑

Cornelia de Lange syndrome 5

Frequency
100%
n=5
P/LP Variants
21
OT Score
-
37
score
ClinGen: DefinitiveP2G #4GTR ↑

developmental and epileptic encephalopathy, 85, with or without midline brain defects

Frequency
100%
n=1
P/LP Variants
40
OT Score
-
4
ARID1B

AT-rich interaction domain 1B

34
score
ClinGen: DefinitiveP2G #30GTR ↑

Coffin-Siris syndrome 1

Frequency
-
P/LP Variants
73
OT Score
0.41
34
score
ClinGen: DefinitiveP2G #7GTR ↑

Cornelia de Lange syndrome 4

Frequency
60%
n=5
P/LP Variants
12
OT Score
-
33
score
ClinGen: DefinitiveP2G #9GTR ↑
Frequency
-
P/LP Variants
101
OT Score
-
31
score
ClinGen: DefinitiveP2G #25GTR ↑

intellectual disability, autosomal dominant 16

Frequency
100%
n=12
P/LP Variants
2
OT Score
-
8
ASXL1

ASXL transcriptional regulator 1

29
score
ClinGen: DefinitiveGTR ↑

Bohring-Opitz syndrome

Frequency
86%
n=7
P/LP Variants
3
OT Score
0.34
29
score
ClinGen: DefinitiveGTR ↑

Wiedemann-Steiner syndrome

Frequency
100%
n=1
P/LP Variants
92
OT Score
-
25ABCC9
Def

hypertrichotic osteochondrodysplasia Cantu type

23KCNK4
Mod

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

twist family bHLH transcription factor 2

22BRD4
Def

Cornelia de Lange syndrome 6

21UROD
Def

familial porphyria cutanea tarda

21SMARCC2
Def

Coffin-Siris syndrome 8

Consider

40 genes
19ARID2
Def
19SMARCB1
Def

intellectual disability, autosomal dominant 15

19CREBBP
Def

Rubinstein-Taybi syndrome due to CREBBP mutations

19ASXL2
Def

Shashi-Pena syndrome

18NFIX
Def

Marshall-Smith syndrome

17RAI1
Def#14

Smith-Magenis syndrome

17BICRA
Def
17DPF2
Def

gingival fibromatosis-hypertrichosis syndrome

16PAX3
Def#8

Waardenburg syndrome type 3

16ARID1A
Def

Warburg micro syndrome 1

14EP300
Def

Rubinstein-Taybi syndrome due to CREBBP mutations

13EHMT1
Def#12

Kleefstra syndrome 1

FACIAL DYSMORPHISM, HYPERTRICHOSIS, EPILEPSY, INTELLECTUAL/DEVELOPMENTAL DELAY, AND GINGIVAL OVERGROWTH SYNDROME; FHEIG

?Hypertrichosis universalis congenita, Ambras type

Hypertrichosis, congenital generalized

Hypertrichosis terminalis, generalized, with or without gingival hyperplasia

13SLC29A3
Def
13UROS
Def#13

cutaneous porphyria

12PPOX
Str

Fontaine progeroid syndrome

11BLM
Def#20

Bloom syndrome

11GNE
Def

sialuria

10NAGLU
Def

mucopolysaccharidosis type 3B

10NOTCH2
Def

acroosteolysis dominant type

intellectual developmental disorder with microcephaly and with or without ocular malformations or hypogonadotropic hypogonadism

10KCNJ11
Def

potassium inwardly rectifying channel subfamily J member 11

9ZNF462
Def
9GP1BA
Def

glycoprotein Ib platelet subunit alpha

9GP9
Def

glycoprotein IX platelet

9SOX3
Mod

SRY-box transcription factor 3

8TMEM94
Def

intellectual developmental disorder with cardiac defects and dysmorphic facies

8MITF
Def

Waardenburg syndrome type 2A

KBG syndrome

8SETD5
Def

intellectual disability-facial dysmorphism syndrome due to SETD5 haploinsufficiency

mitochondrial complex I deficiency, nuclear type 23

Possible

104 genes — click to expand
8USP9X
Def

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

8UBE2A
Def

syndromic X-linked intellectual disability Nascimento type

8PHF8
Def

syndromic X-linked intellectual disability Siderius type

7SMS
Def

syndromic X-linked intellectual disability Snyder type

intellectual disability, X-linked 21

Okur-Chung neurodevelopmental syndrome

7ACTB
Def
7PRR12
Def

neuroocular syndrome 1

7GNS
Def

mucopolysaccharidosis type 3D

7GLB1
Def

GM1 gangliosidosis type 1

7AHDC1
Def

AHDC1-related intellectual disability - obstructive sleep apnea - mild dysmorphism syndrome

7HGSNAT
Def

mucopolysaccharidosis type 3C

7ASH1L
Def

intellectual disability, autosomal dominant 52

7GPC3
Def

Simpson-Golabi-Behmel syndrome type 1

KINSSHIP syndrome

6SETBP1
Def

intellectual disability, autosomal dominant 29

6OTC
DefSF

ornithine transcarbamylase

6RAC1
Def

intellectual disability, autosomal dominant 48

6CDC42
Def

macrothrombocytopenia-lymphedema-developmental delay-facial dysmorphism-camptodactyly syndrome

6NUS1
Def

congenital disorder of glycosylation, type IAA

6ANO6
Mod
6ASXL3
Def

severe feeding difficulties-failure to thrive-microcephaly due to ASXL3 deficiency syndrome

6KDM6B
Def

neurodevelopmental disorder with coarse facies and mild distal skeletal abnormalities

6NANS
Def

spondyloepimetaphyseal dysplasia, Genevieve type

6ALG9
Def

Gillessen-Kaesbach-Nishimura syndrome

craniofacial dysmorphism, skeletal anomalies, and impaired intellectual development 1

intellectual disability, autosomal dominant 30

6EBF3
Def

hypotonia, ataxia, and delayed development syndrome

desmoglein 4

5NSDHL
Mod

NAD(P) dependent 3-beta-hydroxysteroid dehydrogenase NSDHL

5ARX
Def

corpus callosum agenesis-abnormal genitalia syndrome

COFFIN-SIRIS SYNDROME 1; CSS1

5IDS
Def

mucopolysaccharidosis type 2

TRICHOMEGALY; TCMGLY

ZIMMERMANN-LABAND SYNDROME 3; ZLS3

5CDH11
Def

Elsahy-Waters syndrome

5PACS2
Def

developmental and epileptic encephalopathy, 66

5TRIO
Def

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

autosomal recessive spinocerebellar ataxia 17

5PLCB4
Def

auriculocondylar syndrome 2B

5BAP1
Def

Kury-Isidor syndrome

5TRIM8
Def

focal segmental glomerulosclerosis and neurodevelopmental syndrome

4ZFX
Str

intellectual developmental disorder, X-linked, syndromic 37

Hengel-Maroofian-Schols syndrome

Zimmermann-Laband syndrome 2

4FOXN1
Def

forkhead box N1

4FBXL4
Def

mitochondrial DNA depletion syndrome 13

Noonan syndrome 13

4AIFM1
Def

apoptosis inducing factor mitochondria associated 1

4FA2H
Def

fatty acid 2-hydroxylase

4RHBDF2
Def

rhomboid 5 homolog 2

transmembrane serine protease 6

4SPOP
Def

neurodevelopmental disorder with microcephaly and dysmorphic facies

4VPS33A
Def

mucopolysaccharidosis-plus syndrome

4COG7
Def

COG7-congenital disorder of glycosylation

4DHX30
Def

neurodevelopmental disorder with severe motor impairment and absent language

intellectual disability-sparse hair-brachydactyly syndrome

inositol 1,4,5-trisphosphate receptor type 3

arterial tortuosity-bone fragility syndrome

4KCNMA1
Def

Liang-Wang syndrome

4SCN4A
Def

congenital myopathy 22A, classic

cerebellar, ocular, craniofacial, and genital syndrome

4TBCD
Def

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

cortical dysplasia-focal epilepsy syndrome

3TBCK
Def

hypotonia, infantile, with psychomotor retardation and characteristic facies 3

3JARID2
Def

developmental delay with variable intellectual disability and dysmorphic facies

trichohepatoneurodevelopmental syndrome

microcephaly 4, primary, autosomal recessive

Alazami-Yuan syndrome

Suleiman-El-Hattab syndrome

transcriptional repressor GATA binding 1

GLI family zinc finger 2

holoprosencephaly 5

fibroblast growth factor 5

intellectual disability, autosomal recessive 45

neurodevelopmental disorder with hypotonia and characteristic brain abnormalities

3B3GLCT
Def

Peters plus syndrome

3KCNH1
Def

Zimmermann-Laband syndrome 1

3KIFBP
Def

Goldberg-Shprintzen syndrome

3MAN2B1
Def

alpha-mannosidosis

3MED13
Def

intellectual developmental disorder 61

3NSUN2
Def

intellectual disability, autosomal recessive 5

3SGSH
Def

mucopolysaccharidosis type 3A

3SMCHD1
Def

arhinia, choanal atresia, and microphthalmia

3SRD5A3
Def

SRD5A3-congenital disorder of glycosylation

3TLK2
Def

intellectual disability, autosomal dominant 57

3WAC
Def

DeSanto-Shinawi syndrome due to WAC point mutation

3ZBTB20
Def

Primrose syndrome

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