If a variant has more than one submission, it may be counted in more than one significance column. If this is the
case, the total number of variants will be less than the sum of the other cells.
pathogenic |
likely pathogenic |
uncertain significance |
likely benign |
benign |
risk factor |
total |
390
|
210
|
107
|
1
|
0 |
3
|
699
|
Gene and significance breakdown #
Total genes and gene combinations: 107
Gene or gene combination |
pathogenic |
likely pathogenic |
uncertain significance |
likely benign |
risk factor |
total |
LDLR
|
44
|
41
|
2
|
0 |
0 |
79
|
BRCA2
|
61
|
4
|
1
|
0 |
0 |
66
|
BRCA1
|
32
|
5
|
0 |
0 |
0 |
35
|
RYR1
|
6
|
13
|
13
|
0 |
0 |
32
|
AHDC1
|
27
|
0 |
0 |
0 |
0 |
27
|
PMS2
|
12
|
6
|
1
|
0 |
0 |
19
|
KCNQ1
|
9
|
7
|
2
|
0 |
0 |
18
|
TP53
|
14
|
4
|
0 |
0 |
0 |
18
|
CHEK2
|
10
|
6
|
0 |
0 |
0 |
16
|
PALB2
|
10
|
5
|
1
|
0 |
0 |
16
|
MYBPC3
|
12
|
3
|
0 |
0 |
0 |
15
|
DSP
|
4
|
3
|
7
|
0 |
0 |
14
|
MSH6
|
11
|
3
|
0 |
0 |
0 |
14
|
MYH7
|
4
|
10
|
0 |
0 |
0 |
14
|
PKP2
|
10
|
2
|
2
|
0 |
0 |
14
|
ATM
|
9
|
2
|
1
|
0 |
0 |
12
|
MLH1
|
7
|
4
|
1
|
0 |
0 |
12
|
FBN1
|
2
|
4
|
5
|
0 |
0 |
11
|
SCN5A
|
3
|
6
|
2
|
0 |
0 |
11
|
ATM, C11orf65
|
6
|
4
|
0 |
0 |
0 |
10
|
BRCA1, LOC126862571
|
10
|
0 |
0 |
0 |
0 |
10
|
POLR2A
|
1
|
8
|
0 |
1
|
0 |
10
|
APOB
|
1
|
2
|
6
|
0 |
0 |
9
|
KCNH2
|
3
|
4
|
3
|
0 |
0 |
9
|
NF1
|
8
|
1
|
0 |
0 |
0 |
9
|
TSC2
|
2
|
2
|
5
|
0 |
0 |
9
|
DSG2
|
2
|
6
|
0 |
0 |
0 |
8
|
COL3A1
|
1
|
3
|
3
|
0 |
0 |
7
|
COL5A1
|
0 |
0 |
7
|
0 |
0 |
7
|
MSH2
|
6
|
1
|
0 |
0 |
0 |
7
|
PTEN
|
6
|
1
|
0 |
0 |
0 |
7
|
SDHC
|
1
|
4
|
1
|
0 |
0 |
6
|
DSC2
|
0 |
1
|
4
|
0 |
0 |
5
|
RET
|
3
|
2
|
0 |
0 |
0 |
5
|
RYR2
|
0 |
0 |
5
|
0 |
0 |
5
|
TNNI3
|
2
|
4
|
0 |
0 |
0 |
5
|
TNNT2
|
2
|
2
|
1
|
0 |
0 |
5
|
APC
|
3
|
0 |
0 |
0 |
1
|
4
|
KCNE1
|
1
|
1
|
1
|
0 |
1
|
4
|
MUTYH
|
3
|
1
|
0 |
0 |
0 |
4
|
PCSK9
|
0 |
1
|
3
|
0 |
0 |
4
|
SDHA
|
2
|
2
|
0 |
0 |
0 |
4
|
CACNA1A
|
1
|
0 |
2
|
0 |
0 |
3
|
CACNA1S
|
0 |
1
|
2
|
0 |
0 |
3
|
HNF1A
|
1
|
0 |
2
|
0 |
0 |
3
|
LMNA
|
1
|
2
|
0 |
0 |
0 |
3
|
LOC107303340, VHL
|
2
|
1
|
0 |
0 |
0 |
3
|
LPL
|
2
|
1
|
0 |
0 |
0 |
3
|
MC4R
|
1
|
2
|
0 |
0 |
0 |
3
|
MYH11
|
0 |
0 |
3
|
0 |
0 |
3
|
MYLK
|
0 |
1
|
2
|
0 |
0 |
3
|
SDHB
|
2
|
1
|
0 |
0 |
0 |
3
|
TGFBR2
|
1
|
1
|
1
|
0 |
0 |
3
|
TTN
|
1
|
2
|
0 |
0 |
0 |
3
|
UMOD
|
0 |
3
|
0 |
0 |
0 |
3
|
CBL
|
1
|
1
|
0 |
0 |
0 |
2
|
DICER1
|
2
|
0 |
0 |
0 |
0 |
2
|
F11
|
2
|
0 |
0 |
0 |
0 |
2
|
GLA, RPL36A-HNRNPH2
|
2
|
0 |
0 |
0 |
0 |
2
|
HNF1B
|
0 |
1
|
1
|
0 |
0 |
2
|
HRAS, LRRC56
|
2
|
0 |
0 |
0 |
0 |
2
|
KCNJ2
|
0 |
0 |
2
|
0 |
0 |
2
|
LOC126861898, MYH7
|
1
|
1
|
0 |
0 |
0 |
2
|
MEN1
|
0 |
1
|
1
|
0 |
0 |
2
|
MYH11, NDE1
|
0 |
0 |
2
|
0 |
0 |
2
|
TMEM43
|
0 |
0 |
2
|
0 |
0 |
2
|
TSC1
|
0 |
0 |
2
|
0 |
0 |
2
|
ABCA1
|
0 |
1
|
0 |
0 |
0 |
1
|
ACTA2
|
0 |
0 |
1
|
0 |
0 |
1
|
ALK
|
0 |
1
|
0 |
0 |
0 |
1
|
ATP1A2
|
0 |
1
|
0 |
0 |
0 |
1
|
BMPR1A
|
0 |
1
|
0 |
0 |
0 |
1
|
CCDST, FLG
|
1
|
0 |
0 |
0 |
0 |
1
|
CRHR1, KANSL1, MAPT, PLEKHM1, SPPL2C, STH
|
1
|
0 |
0 |
0 |
0 |
1
|
CYP21A2, LOC106780800
|
1
|
0 |
0 |
0 |
0 |
1
|
DCTN5, PALB2
|
1
|
0 |
0 |
0 |
0 |
1
|
ELN
|
0 |
1
|
0 |
0 |
0 |
1
|
F5
|
1
|
0 |
0 |
0 |
0 |
1
|
FBN1, LOC113939944
|
0 |
0 |
1
|
0 |
0 |
1
|
HFE
|
1
|
0 |
0 |
0 |
0 |
1
|
INSL6, JAK2
|
1
|
0 |
0 |
0 |
0 |
1
|
KCNE2, LOC105372791
|
0 |
0 |
0 |
0 |
1
|
1
|
KIF1A
|
1
|
0 |
0 |
0 |
0 |
1
|
LMNA, LOC126805877
|
0 |
1
|
0 |
0 |
0 |
1
|
LOC107982234, WT1
|
0 |
0 |
1
|
0 |
0 |
1
|
LOC126806068, RYR2
|
0 |
0 |
1
|
0 |
0 |
1
|
LOC126861897, MHRT, MYH7
|
0 |
1
|
0 |
0 |
0 |
1
|
LOC126862482, POLR2A
|
1
|
0 |
0 |
0 |
0 |
1
|
LOC130062899, STK11
|
0 |
0 |
1
|
0 |
0 |
1
|
MED13L
|
1
|
0 |
0 |
0 |
0 |
1
|
MEFV
|
0 |
1
|
0 |
0 |
0 |
1
|
MYL3
|
1
|
0 |
0 |
0 |
0 |
1
|
NEXN
|
0 |
1
|
0 |
0 |
0 |
1
|
OTC
|
1
|
0 |
0 |
0 |
0 |
1
|
POLE
|
0 |
0 |
1
|
0 |
0 |
1
|
PRKAR1A
|
0 |
1
|
0 |
0 |
0 |
1
|
PTCH1
|
0 |
1
|
0 |
0 |
0 |
1
|
SDHD
|
1
|
0 |
0 |
0 |
0 |
1
|
SERPINA1
|
1
|
0 |
0 |
0 |
0 |
1
|
SMAD3
|
0 |
1
|
0 |
0 |
0 |
1
|
SMAD4
|
1
|
0 |
0 |
0 |
0 |
1
|
STK11
|
0 |
0 |
1
|
0 |
0 |
1
|
TGFB2
|
1
|
0 |
0 |
0 |
0 |
1
|
TOR1A
|
1
|
0 |
0 |
0 |
0 |
1
|
TPM1
|
0 |
0 |
1
|
0 |
0 |
1
|
TUSC3
|
1
|
0 |
0 |
0 |
0 |
1
|
WT1
|
0 |
1
|
0 |
0 |
0 |
1
|
Condition and significance breakdown #
Condition |
pathogenic |
likely pathogenic |
uncertain significance |
likely benign |
risk factor |
total |
Familial hypercholesterolemia
|
29
|
17
|
8
|
0 |
0 |
54
|
Breast-ovarian cancer, familial, susceptibility to, 2
|
46
|
3
|
1
|
0 |
0 |
50
|
Hypercholesterolemia, familial, 1
|
16
|
26
|
0 |
0 |
0 |
42
|
Rhabdomyosarcoma
|
36
|
6
|
0 |
0 |
0 |
42
|
Breast-ovarian cancer, familial, susceptibility to, 1
|
35
|
4
|
0 |
0 |
0 |
39
|
Familial cancer of breast
|
22
|
6
|
0 |
0 |
0 |
28
|
AHDC1-related intellectual disability - obstructive sleep apnea - mild dysmorphism syndrome
|
27
|
0 |
0 |
0 |
0 |
27
|
Malignant hyperthermia, susceptibility to, 1
|
4
|
5
|
13
|
0 |
0 |
22
|
Hereditary breast ovarian cancer syndrome
|
17
|
3
|
0 |
0 |
0 |
20
|
Arrhythmogenic right ventricular dysplasia 9
|
10
|
2
|
2
|
0 |
0 |
14
|
Ataxia-telangiectasia syndrome; Breast cancer, susceptibility to
|
10
|
3
|
1
|
0 |
0 |
14
|
Arrhythmogenic right ventricular dysplasia 8
|
4
|
2
|
7
|
0 |
0 |
13
|
Hypertrophic cardiomyopathy 1
|
5
|
8
|
0 |
0 |
0 |
13
|
Lynch syndrome
|
6
|
7
|
0 |
0 |
0 |
13
|
Lynch syndrome 4
|
9
|
3
|
1
|
0 |
0 |
13
|
Marfan syndrome
|
2
|
4
|
6
|
0 |
0 |
12
|
Colorectal cancer, hereditary nonpolyposis, type 2
|
7
|
3
|
1
|
0 |
0 |
11
|
Hypertrophic cardiomyopathy 4
|
9
|
2
|
0 |
0 |
0 |
11
|
Hypertrophic cardiomyopathy
|
3
|
7
|
0 |
0 |
0 |
10
|
Neurodevelopmental disorder with hypotonia and variable intellectual and behavioral abnormalities
|
2
|
8
|
0 |
0 |
0 |
10
|
Arrhythmogenic right ventricular dysplasia 10
|
2
|
7
|
0 |
0 |
0 |
9
|
Congenital multicore myopathy with external ophthalmoplegia
|
1
|
8
|
0 |
0 |
0 |
9
|
Lynch syndrome 5
|
7
|
2
|
0 |
0 |
0 |
9
|
Long QT syndrome 2
|
2
|
3
|
3
|
0 |
0 |
8
|
Tuberous sclerosis 2
|
1
|
2
|
5
|
0 |
0 |
8
|
Ehlers-Danlos syndrome, classic type, 1
|
0 |
0 |
7
|
0 |
0 |
7
|
Ehlers-Danlos syndrome, type 4
|
1
|
3
|
3
|
0 |
0 |
7
|
Long QT syndrome
|
3
|
4
|
0 |
0 |
0 |
7
|
Long QT syndrome 1
|
3
|
4
|
0 |
0 |
0 |
7
|
Long QT syndrome 1; Jervell and Lange-Nielsen syndrome 1
|
4
|
1
|
2
|
0 |
0 |
7
|
Pancreatic cancer, susceptibility to, 3; Breast cancer, susceptibility to
|
3
|
3
|
1
|
0 |
0 |
7
|
Brugada syndrome 1; Long QT syndrome 3
|
0 |
4
|
2
|
0 |
0 |
6
|
Catecholaminergic polymorphic ventricular tachycardia 1
|
0 |
0 |
6
|
0 |
0 |
6
|
Li-Fraumeni syndrome
|
4
|
2
|
0 |
0 |
0 |
6
|
Aortic aneurysm, familial thoracic 4
|
0 |
0 |
5
|
0 |
0 |
5
|
Arrhythmogenic right ventricular dysplasia 11
|
0 |
1
|
4
|
0 |
0 |
5
|
Breast cancer, susceptibility to; Prostate cancer susceptibility
|
1
|
4
|
0 |
0 |
0 |
5
|
Long QT syndrome 3
|
3
|
2
|
0 |
0 |
0 |
5
|
Paragangliomas 3
|
1
|
3
|
1
|
0 |
0 |
5
|
Familial adenomatous polyposis 2
|
3
|
1
|
0 |
0 |
0 |
4
|
Hypercholesterolemia, autosomal dominant, 3
|
0 |
1
|
3
|
0 |
0 |
4
|
Hypertrophic cardiomyopathy 7
|
2
|
2
|
0 |
0 |
0 |
4
|
Lynch syndrome 1
|
4
|
0 |
0 |
0 |
0 |
4
|
Multiple endocrine neoplasia type 2A
|
3
|
1
|
0 |
0 |
0 |
4
|
Aortic aneurysm, familial thoracic 7
|
0 |
1
|
2
|
0 |
0 |
3
|
Dilated cardiomyopathy 1A
|
0 |
3
|
0 |
0 |
0 |
3
|
Dilated cardiomyopathy 1G
|
1
|
2
|
0 |
0 |
0 |
3
|
Episodic ataxia type 2
|
1
|
0 |
2
|
0 |
0 |
3
|
Familial adenomatous polyposis 1
|
2
|
0 |
0 |
0 |
1
|
3
|
Familial juvenile hyperuricemic nephropathy type 1
|
0 |
3
|
0 |
0 |
0 |
3
|
Hyperlipidemia, familial combined, LPL related
|
2
|
1
|
0 |
0 |
0 |
3
|
Hypertrophic cardiomyopathy 2; Dilated cardiomyopathy 1D
|
0 |
2
|
1
|
0 |
0 |
3
|
Intellectual disability
|
3
|
0 |
0 |
0 |
0 |
3
|
Loeys-Dietz syndrome 2
|
1
|
1
|
1
|
0 |
0 |
3
|
Long QT syndrome 5
|
1
|
1
|
0 |
0 |
1
|
3
|
Malignant hyperthermia, susceptibility to
|
2
|
1
|
0 |
0 |
0 |
3
|
Maturity-onset diabetes of the young type 3
|
1
|
0 |
2
|
0 |
0 |
3
|
Obesity
|
1
|
2
|
0 |
0 |
0 |
3
|
PTEN hamartoma tumor syndromes
|
2
|
1
|
0 |
0 |
0 |
3
|
Paragangliomas 4
|
2
|
1
|
0 |
0 |
0 |
3
|
Von Hippel-Lindau syndrome
|
2
|
1
|
0 |
0 |
0 |
3
|
Andersen Tawil syndrome
|
0 |
0 |
2
|
0 |
0 |
2
|
Arrhythmogenic right ventricular dysplasia 5
|
0 |
0 |
2
|
0 |
0 |
2
|
Cowden syndrome 1
|
2
|
0 |
0 |
0 |
0 |
2
|
Dilated cardiomyopathy 1D
|
2
|
0 |
0 |
0 |
0 |
2
|
Fabry disease
|
2
|
0 |
0 |
0 |
0 |
2
|
Generalized juvenile polyposis/juvenile polyposis coli
|
1
|
1
|
0 |
0 |
0 |
2
|
Hypercholesterolemia, autosomal dominant, type B
|
1
|
1
|
0 |
0 |
0 |
2
|
Li-Fraumeni syndrome 1
|
1
|
1
|
0 |
0 |
0 |
2
|
Malignant hyperthermia, susceptibility to, 5
|
0 |
0 |
2
|
0 |
0 |
2
|
Multiple endocrine neoplasia, type 1
|
0 |
1
|
1
|
0 |
0 |
2
|
Paragangliomas 5
|
0 |
2
|
0 |
0 |
0 |
2
|
Peutz-Jeghers syndrome
|
0 |
0 |
2
|
0 |
0 |
2
|
Renal cysts and diabetes syndrome
|
0 |
1
|
1
|
0 |
0 |
2
|
Tuberous sclerosis 1
|
0 |
0 |
2
|
0 |
0 |
2
|
Wilms tumor 1
|
0 |
1
|
1
|
0 |
0 |
2
|
Acquired polycythemia vera
|
1
|
0 |
0 |
0 |
0 |
1
|
Alpha-1-antitrypsin deficiency
|
1
|
0 |
0 |
0 |
0 |
1
|
Aneurysm-osteoarthritis syndrome
|
0 |
1
|
0 |
0 |
0 |
1
|
Aortic aneurysm, familial thoracic 6
|
0 |
0 |
1
|
0 |
0 |
1
|
Arrhythmogenic cardiomyopathy with wooly hair and keratoderma
|
0 |
1
|
0 |
0 |
0 |
1
|
Brugada syndrome
|
1
|
0 |
0 |
0 |
0 |
1
|
Colorectal cancer susceptibility 12
|
0 |
0 |
1
|
0 |
0 |
1
|
Congenital adrenal hyperplasia
|
1
|
0 |
0 |
0 |
0 |
1
|
Dilated cardiomyopathy 1CC
|
0 |
1
|
0 |
0 |
0 |
1
|
Early-onset generalized limb-onset dystonia
|
1
|
0 |
0 |
0 |
0 |
1
|
Factor V deficiency
|
1
|
0 |
0 |
0 |
0 |
1
|
Factor XI
|
1
|
0 |
0 |
0 |
0 |
1
|
Familial Mediterranean fever
|
0 |
1
|
0 |
0 |
0 |
1
|
Familial multiple polyposis syndrome
|
1
|
0 |
0 |
0 |
0 |
1
|
Hemochromatosis type 1
|
1
|
0 |
0 |
0 |
0 |
1
|
Hereditary factor XI deficiency disease
|
1
|
0 |
0 |
0 |
0 |
1
|
Hypertrophic cardiomyopathy 3
|
0 |
0 |
1
|
0 |
0 |
1
|
Hypertrophic cardiomyopathy 8
|
1
|
0 |
0 |
0 |
0 |
1
|
Hypoalphalipoproteinemia, primary, 1
|
0 |
1
|
0 |
0 |
0 |
1
|
Ichthyosis vulgaris
|
1
|
0 |
0 |
0 |
0 |
1
|
Intellectual disability, autosomal dominant 9
|
1
|
0 |
0 |
0 |
0 |
1
|
Jervell and Lange-Nielsen syndrome 2; Long QT syndrome 5
|
0 |
0 |
1
|
0 |
0 |
1
|
Loeys-Dietz syndrome 4
|
1
|
0 |
0 |
0 |
0 |
1
|
Long QT syndrome 6
|
0 |
0 |
0 |
0 |
1
|
1
|
Migraine, familial hemiplegic, 2; Alternating hemiplegia of childhood
|
0 |
1
|
0 |
0 |
0 |
1
|
Multiple endocrine neoplasia type 2B; Multiple endocrine neoplasia type 2A
|
0 |
1
|
0 |
0 |
0 |
1
|
Multiple endocrine neoplasia, type 2
|
0 |
1
|
0 |
0 |
0 |
1
|
Ornithine carbamoyltransferase deficiency
|
1
|
0 |
0 |
0 |
0 |
1
|
PTEN hamartoma tumor syndrome
|
1
|
0 |
0 |
0 |
0 |
1
|
Paragangliomas 1
|
1
|
0 |
0 |
0 |
0 |
1
|
Primary dilated cardiomyopathy
|
1
|
0 |
0 |
0 |
0 |
1
|
Supravalvar aortic stenosis
|
0 |
1
|
0 |
0 |
0 |
1
|
Tuberous sclerosis syndrome
|
1
|
0 |
0 |
0 |
0 |
1
|
not specified
|
0 |
0 |
0 |
1
|
0 |
1
|
The information on this website is not intended for direct
diagnostic use or medical decision-making without review by a
genetics professional. Individuals should not change their
health behavior solely on the basis of information contained on
this website. Neither the University of Utah nor the National
Institutes of Health independently verfies the submitted
information. If you have questions about the information
contained on this website, please see a health care
professional.