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 |
not provided |
total |
117
|
90
|
1900
|
839
|
323
|
7
|
3209
|
Gene and significance breakdown #
Total genes and gene combinations: 109
Gene or gene combination |
pathogenic |
likely pathogenic |
uncertain significance |
likely benign |
benign |
not provided |
total |
GJB1
|
17
|
6
|
353
|
10
|
4
|
0 |
377
|
MFN2
|
7
|
5
|
173
|
35
|
11
|
0 |
225
|
SH3TC2
|
12
|
7
|
121
|
55
|
14
|
0 |
203
|
PRX
|
6
|
3
|
115
|
62
|
16
|
0 |
200
|
MPZ
|
19
|
10
|
163
|
7
|
2
|
0 |
194
|
DYNC1H1
|
0 |
3
|
30
|
105
|
44
|
1
|
182
|
IGHMBP2
|
1
|
4
|
71
|
45
|
26
|
0 |
145
|
TRPV4
|
3
|
1
|
49
|
47
|
23
|
0 |
122
|
KIF1B
|
0 |
1
|
45
|
41
|
30
|
0 |
116
|
FIG4
|
3
|
1
|
39
|
37
|
15
|
0 |
94
|
PMP22
|
6
|
7
|
62
|
9
|
2
|
0 |
84
|
GDAP1
|
5
|
3
|
57
|
9
|
4
|
6
|
76
|
LRSAM1
|
2
|
6
|
31
|
27
|
11
|
0 |
76
|
SBF2
|
0 |
1
|
35
|
30
|
8
|
0 |
74
|
GARS1
|
2
|
1
|
26
|
29
|
13
|
0 |
70
|
MED25
|
0 |
0 |
20
|
39
|
11
|
0 |
70
|
MARS1
|
0 |
0 |
28
|
25
|
8
|
0 |
61
|
LMNA
|
2
|
3
|
22
|
25
|
7
|
0 |
58
|
NEFL
|
5
|
4
|
32
|
15
|
2
|
0 |
57
|
FGD4
|
0 |
0 |
21
|
16
|
12
|
0 |
49
|
MTMR2
|
0 |
0 |
24
|
17
|
8
|
0 |
49
|
HSPB1
|
8
|
2
|
28
|
13
|
1
|
0 |
48
|
NDRG1
|
1
|
0 |
18
|
20
|
3
|
0 |
42
|
SPTLC1
|
2
|
0 |
20
|
9
|
7
|
0 |
37
|
AARS1
|
1
|
0 |
32
|
0 |
0 |
0 |
33
|
LITAF
|
2
|
2
|
23
|
5
|
4
|
0 |
33
|
EGR2
|
0 |
0 |
16
|
14
|
1
|
0 |
30
|
NTRK1
|
0 |
0 |
29
|
1
|
0 |
0 |
30
|
TTR
|
5
|
2
|
7
|
10
|
5
|
0 |
29
|
DNAJB2
|
0 |
0 |
7
|
14
|
3
|
0 |
24
|
LOC101928008, SBF2
|
0 |
0 |
7
|
8
|
6
|
0 |
21
|
LOC105369149, SBF2
|
0 |
0 |
6
|
12
|
2
|
0 |
20
|
AIFM1, RAB33A
|
0 |
0 |
4
|
8
|
6
|
0 |
18
|
INF2
|
0 |
0 |
17
|
0 |
0 |
0 |
17
|
SEPTIN9
|
0 |
0 |
16
|
0 |
0 |
0 |
16
|
RAB7A
|
0 |
0 |
7
|
6
|
2
|
0 |
15
|
GDAP1, LOC130000622
|
2
|
1
|
5
|
4
|
1
|
0 |
13
|
DNM2
|
0 |
2
|
8
|
0 |
0 |
0 |
10
|
IGHMBP2, LOC126861245
|
0 |
0 |
3
|
5
|
2
|
0 |
10
|
LOC130064454, PRX
|
3
|
2
|
5
|
0 |
0 |
0 |
10
|
PDK3
|
0 |
0 |
4
|
5
|
1
|
0 |
10
|
LOC126860531, NDRG1
|
0 |
0 |
2
|
5
|
2
|
0 |
9
|
MORC2
|
0 |
0 |
9
|
0 |
0 |
0 |
9
|
SCN11A
|
0 |
0 |
8
|
0 |
0 |
0 |
8
|
SETX
|
0 |
0 |
8
|
0 |
0 |
0 |
8
|
SLC12A6
|
0 |
0 |
8
|
0 |
0 |
0 |
8
|
DYNC1H1, LOC126862060
|
0 |
0 |
1
|
4
|
1
|
0 |
6
|
WNK1
|
0 |
0 |
5
|
0 |
0 |
0 |
5
|
DCTN1
|
0 |
1
|
3
|
0 |
0 |
0 |
4
|
DNMT1
|
0 |
0 |
4
|
0 |
0 |
0 |
4
|
GAN
|
0 |
0 |
4
|
0 |
0 |
0 |
4
|
HINT1
|
0 |
0 |
4
|
0 |
0 |
0 |
4
|
HSPB8
|
0 |
0 |
4
|
0 |
0 |
0 |
4
|
KIF1B, LOC126805614
|
0 |
0 |
2
|
2
|
0 |
0 |
4
|
LOC126860330, NEFL
|
0 |
0 |
2
|
2
|
0 |
0 |
4
|
RETREG1
|
0 |
0 |
4
|
0 |
0 |
0 |
4
|
ARHGEF10
|
0 |
1
|
2
|
0 |
0 |
0 |
3
|
ELP1
|
0 |
0 |
3
|
0 |
0 |
0 |
3
|
NGF
|
0 |
0 |
3
|
0 |
0 |
0 |
3
|
REEP1
|
0 |
0 |
3
|
0 |
0 |
0 |
3
|
ARF1, LOC126806039
|
0 |
0 |
2
|
0 |
0 |
0 |
2
|
ATL1
|
0 |
0 |
2
|
0 |
0 |
0 |
2
|
ATP7A
|
0 |
0 |
2
|
0 |
0 |
0 |
2
|
BICD2
|
0 |
0 |
2
|
0 |
0 |
0 |
2
|
BSCL2, HNRNPUL2-BSCL2
|
0 |
1
|
1
|
0 |
0 |
0 |
2
|
DST
|
0 |
1
|
1
|
0 |
0 |
0 |
2
|
KARS1
|
0 |
0 |
2
|
0 |
0 |
0 |
2
|
KIF1B, LOC129388446
|
0 |
0 |
1
|
1
|
0 |
0 |
2
|
LMNA, LOC126805877
|
0 |
0 |
0 |
1
|
1
|
0 |
2
|
LMNA, LOC129931597
|
0 |
0 |
1
|
0 |
1
|
0 |
2
|
LOC129929426, MFN2
|
0 |
0 |
1
|
1
|
0 |
0 |
2
|
MIR6841, NEFL
|
0 |
0 |
0 |
1
|
1
|
0 |
2
|
PLD3, PRX
|
0 |
1
|
2
|
0 |
1
|
0 |
2
|
POLR2F, SOX10
|
0 |
0 |
2
|
0 |
0 |
0 |
2
|
SBF1
|
0 |
2
|
1
|
0 |
0 |
0 |
2
|
SCN1A, SCN9A
|
0 |
0 |
2
|
0 |
0 |
0 |
2
|
YARS1
|
0 |
0 |
2
|
0 |
0 |
0 |
2
|
AIFM1, LOC130068679, RAB33A
|
0 |
0 |
0 |
1
|
0 |
0 |
1
|
ARHGEF10, LOC126860281
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
CNTNAP2
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
CTDP1
|
1
|
0 |
0 |
0 |
0 |
0 |
1
|
DCTN2
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
DHX9
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
DRP2
|
0 |
0 |
0 |
0 |
1
|
0 |
1
|
FBXO38
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
GAN, LOC130059498
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
GNB4
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
HADHB
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
HARS1
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
IFRD1
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
KIF1B, LOC129388447
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
LAMA2
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
LOC112872299, RAB7A
|
0 |
0 |
0 |
1
|
0 |
0 |
1
|
LOC126862651, SEPTIN9
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
LOC129390683, SLC12A6
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
LOC129993734, RETREG1
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
MED25, MIR6800
|
0 |
0 |
0 |
1
|
0 |
0 |
1
|
MME
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
MPZ, SDHC
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
MT-ATP6
|
1
|
0 |
0 |
0 |
0 |
0 |
1
|
MYH14
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
NAGLU
|
1
|
0 |
0 |
0 |
0 |
0 |
1
|
POLG
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
SEMA5A
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
SLC25A46
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
SLC5A7
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
SPG11
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
SPTLC2
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
TFG
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
Submitter and significance breakdown #
Submitter |
pathogenic |
likely pathogenic |
uncertain significance |
likely benign |
benign |
not provided |
total |
Molecular Genetics Laboratory, London Health Sciences Centre
|
62
|
59
|
638
|
833
|
317
|
0 |
1909
|
Inherited Neuropathy Consortium
|
45
|
18
|
1049
|
4
|
1
|
0 |
1117
|
Genesis Genome Database
|
0 |
0 |
207
|
0 |
0 |
0 |
207
|
Natera, Inc.
|
5
|
0 |
18
|
5
|
3
|
0 |
31
|
Dept. of Medical Genetics, Telemark Hospital Trust, Telemark Hospital Trust
|
4
|
6
|
17
|
0 |
1
|
0 |
27
|
Molecular Genetics, Royal Melbourne Hospital
|
0 |
1
|
6
|
0 |
1
|
0 |
8
|
GeneReviews
|
1
|
0 |
0 |
0 |
0 |
6
|
7
|
Al Jalila Children’s Genomics Center, Al Jalila Childrens Speciality Hospital
|
2
|
3
|
0 |
0 |
0 |
0 |
5
|
Laboratory for Molecular Medicine, Mass General Brigham Personalized Medicine
|
1
|
1
|
0 |
0 |
0 |
0 |
2
|
Victorian Clinical Genetics Services, Murdoch Childrens Research Institute
|
1
|
0 |
1
|
0 |
0 |
0 |
2
|
Illumina Laboratory Services, Illumina
|
1
|
0 |
1
|
0 |
0 |
0 |
2
|
ClinVar Staff, National Center for Biotechnology Information (NCBI)
|
0 |
0 |
0 |
0 |
0 |
1
|
1
|
Genomic Medicine Center of Excellence, King Faisal Specialist Hospital and Research Centre
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
CSER _CC_NCGL, University of Washington
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
Kariminejad - Najmabadi Pathology & Genetics Center
|
0 |
0 |
1
|
0 |
0 |
0 |
1
|
Department of Human Genetics, University Hospital Magdeburg
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
Institute of Medical Genetics and Applied Genomics, University Hospital Tübingen
|
0 |
1
|
0 |
0 |
0 |
0 |
1
|
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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
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