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 | total |
---|---|---|---|---|---|
164 | 240 | 189 | 5 | 0 | 597 |
Gene and significance breakdown #
Gene or gene combination | pathogenic | likely pathogenic | uncertain significance | likely benign | total |
---|---|---|---|---|---|
PKD1 | 7 | 8 | 7 | 0 | 22 |
COL4A5 | 5 | 11 | 2 | 0 | 18 |
FBN1 | 5 | 8 | 5 | 0 | 18 |
KMT2E | 6 | 10 | 0 | 0 | 15 |
COL1A1 | 6 | 4 | 1 | 0 | 11 |
COL1A2 | 4 | 7 | 0 | 0 | 11 |
COL4A4 | 1 | 8 | 2 | 0 | 11 |
PKD2 | 1 | 1 | 5 | 0 | 7 |
TTN | 0 | 5 | 1 | 1 | 7 |
CASR | 1 | 0 | 5 | 0 | 6 |
COL4A3, MFF-DT | 1 | 5 | 0 | 0 | 6 |
MACF1 | 0 | 0 | 6 | 0 | 6 |
NF1 | 1 | 3 | 2 | 0 | 6 |
ARID1B | 3 | 2 | 0 | 0 | 5 |
CFH | 1 | 3 | 1 | 0 | 5 |
CHD7 | 2 | 3 | 0 | 0 | 5 |
AARS1 | 0 | 3 | 1 | 0 | 4 |
GBF1 | 0 | 4 | 0 | 0 | 4 |
IGHMBP2 | 2 | 1 | 1 | 0 | 4 |
LMX1B | 2 | 1 | 1 | 0 | 4 |
MFN2 | 2 | 2 | 0 | 0 | 4 |
SMAD3 | 0 | 2 | 2 | 0 | 4 |
TSC2 | 2 | 0 | 2 | 0 | 4 |
UMOD | 0 | 2 | 2 | 0 | 4 |
ACTA2 | 0 | 2 | 1 | 0 | 3 |
COL2A1 | 1 | 2 | 0 | 0 | 3 |
COL5A1 | 1 | 1 | 1 | 0 | 3 |
CTNS | 0 | 1 | 2 | 0 | 3 |
DNAJB11 | 0 | 1 | 2 | 0 | 3 |
GARS1 | 1 | 0 | 2 | 0 | 3 |
GDAP1 | 3 | 0 | 0 | 0 | 3 |
KMT2D | 3 | 0 | 0 | 0 | 3 |
MPZ | 1 | 1 | 1 | 0 | 3 |
PIEZO2 | 1 | 0 | 2 | 0 | 3 |
PKHD1 | 0 | 2 | 1 | 0 | 3 |
PLEKHG5 | 1 | 1 | 1 | 0 | 3 |
SETX | 1 | 0 | 2 | 0 | 3 |
ADNP | 1 | 0 | 0 | 1 | 2 |
AGTPBP1 | 1 | 1 | 0 | 0 | 2 |
ARHGEF10 | 0 | 0 | 2 | 0 | 2 |
BMPR2 | 1 | 1 | 0 | 0 | 2 |
CAPN3 | 1 | 0 | 1 | 0 | 2 |
CFTR | 2 | 0 | 0 | 0 | 2 |
CNOT1 | 0 | 1 | 1 | 0 | 2 |
COL12A1 | 1 | 0 | 1 | 0 | 2 |
COL3A1 | 0 | 0 | 2 | 0 | 2 |
COL9A3 | 1 | 0 | 1 | 0 | 2 |
DHTKD1 | 1 | 0 | 1 | 0 | 2 |
DSG1 | 0 | 2 | 0 | 0 | 2 |
ECEL1 | 0 | 2 | 0 | 0 | 2 |
EFTUD2 | 0 | 2 | 0 | 0 | 2 |
FANCA | 1 | 1 | 0 | 0 | 2 |
FLCN | 1 | 0 | 1 | 0 | 2 |
FUS | 1 | 0 | 1 | 0 | 2 |
GPR143 | 2 | 0 | 0 | 0 | 2 |
HNF1B | 1 | 1 | 0 | 0 | 2 |
IHH | 0 | 1 | 1 | 0 | 2 |
JAG1 | 0 | 1 | 1 | 0 | 2 |
KIF5C | 0 | 0 | 1 | 1 | 2 |
LGI4 | 1 | 1 | 0 | 0 | 2 |
LIFR | 0 | 1 | 1 | 0 | 2 |
LRP2 | 2 | 0 | 0 | 0 | 2 |
MDFIC | 1 | 1 | 0 | 0 | 2 |
MMP21 | 0 | 1 | 1 | 0 | 2 |
MYLK | 0 | 0 | 2 | 0 | 2 |
NFKB1 | 0 | 2 | 0 | 0 | 2 |
NPR2 | 0 | 2 | 0 | 0 | 2 |
OTOG | 2 | 0 | 0 | 0 | 2 |
PBX1 | 1 | 1 | 0 | 0 | 2 |
PGAP2 | 0 | 2 | 0 | 0 | 2 |
PHEX, PTCHD1 | 0 | 1 | 1 | 0 | 2 |
PNPLA8 | 0 | 1 | 1 | 0 | 2 |
PRX | 2 | 0 | 0 | 0 | 2 |
PUF60 | 0 | 1 | 1 | 0 | 2 |
SCN2A | 0 | 1 | 1 | 0 | 2 |
SCN8A | 0 | 2 | 0 | 0 | 2 |
SEC24D | 0 | 2 | 0 | 0 | 2 |
SEPSECS | 0 | 1 | 1 | 0 | 2 |
SLC12A3 | 1 | 0 | 1 | 0 | 2 |
SLC52A2 | 1 | 1 | 0 | 0 | 2 |
SLC7A9 | 1 | 0 | 1 | 0 | 2 |
SMARCAL1 | 0 | 2 | 0 | 0 | 2 |
SOD1 | 2 | 0 | 0 | 0 | 2 |
SPG7 | 0 | 0 | 2 | 0 | 2 |
TGFB2 | 0 | 0 | 2 | 0 | 2 |
TGFBR1 | 0 | 2 | 0 | 0 | 2 |
TMEM147 | 0 | 1 | 1 | 0 | 2 |
TNXB | 0 | 1 | 1 | 0 | 2 |
TRPV4 | 1 | 0 | 1 | 0 | 2 |
USH1C | 1 | 1 | 0 | 0 | 2 |
VAC14 | 0 | 0 | 2 | 0 | 2 |
VPS13D | 1 | 1 | 0 | 0 | 2 |
WDR19 | 0 | 0 | 2 | 0 | 2 |
ZFYVE26 | 1 | 1 | 0 | 0 | 2 |
ACTA1 | 1 | 0 | 0 | 0 | 1 |
ACTN4 | 0 | 0 | 1 | 0 | 1 |
ADAR | 0 | 1 | 0 | 0 | 1 |
ADGRL1 | 0 | 0 | 1 | 0 | 1 |
ADPRS | 1 | 0 | 0 | 0 | 1 |
AEBP1 | 0 | 0 | 1 | 0 | 1 |
AFF2 | 0 | 1 | 0 | 0 | 1 |
AGO1 | 0 | 0 | 1 | 0 | 1 |
AGPAT2 | 0 | 0 | 1 | 0 | 1 |
AGXT | 0 | 1 | 0 | 0 | 1 |
ALG1, EEF2KMT | 0 | 1 | 0 | 0 | 1 |
ANK1 | 0 | 1 | 0 | 0 | 1 |
ANKRD11 | 1 | 0 | 0 | 0 | 1 |
ANLN | 0 | 0 | 1 | 0 | 1 |
AQP2, AQP5 | 0 | 1 | 0 | 0 | 1 |
ARHGEF9 | 0 | 0 | 1 | 0 | 1 |
ARSA | 1 | 0 | 0 | 0 | 1 |
ARX | 0 | 0 | 1 | 0 | 1 |
ASXL3 | 0 | 1 | 0 | 0 | 1 |
ATAD2B | 0 | 0 | 1 | 0 | 1 |
ATAD3A | 0 | 0 | 1 | 0 | 1 |
ATOH7 | 0 | 1 | 0 | 0 | 1 |
ATP6V0C | 0 | 1 | 0 | 0 | 1 |
ATP7A | 0 | 1 | 0 | 0 | 1 |
ATXN7L1, KMT2E, PUS7, RINT1, SRPK2 | 1 | 0 | 0 | 0 | 1 |
AUTS2 | 0 | 1 | 0 | 0 | 1 |
AVPR2 | 1 | 0 | 0 | 0 | 1 |
B4GAT1 | 0 | 0 | 1 | 0 | 1 |
BBS10 | 1 | 0 | 0 | 0 | 1 |
BCL11A | 0 | 1 | 0 | 0 | 1 |
BCOR, LOC126863239 | 0 | 0 | 1 | 0 | 1 |
BICD2 | 1 | 0 | 0 | 0 | 1 |
BTK | 1 | 0 | 0 | 0 | 1 |
C1GALT1C1 | 0 | 1 | 0 | 0 | 1 |
C1R | 0 | 0 | 1 | 0 | 1 |
C3 | 0 | 0 | 1 | 0 | 1 |
CACNA1H | 0 | 0 | 1 | 0 | 1 |
CAD | 0 | 0 | 1 | 0 | 1 |
CAD, LOC126806172 | 0 | 0 | 1 | 0 | 1 |
CDK19 | 0 | 0 | 1 | 0 | 1 |
CDK5RAP2 | 0 | 1 | 0 | 0 | 1 |
CEP104 | 0 | 0 | 1 | 0 | 1 |
CHD1L | 0 | 0 | 1 | 0 | 1 |
CHD4 | 0 | 1 | 0 | 0 | 1 |
CHD8 | 0 | 1 | 0 | 0 | 1 |
CHKB, CHKB-CPT1B | 0 | 1 | 0 | 0 | 1 |
CHRNG, TIGD1 | 1 | 0 | 0 | 0 | 1 |
CHST3 | 0 | 1 | 0 | 0 | 1 |
CLCN1 | 0 | 0 | 1 | 0 | 1 |
CLCN4 | 0 | 1 | 0 | 0 | 1 |
CNKSR2 | 0 | 1 | 0 | 0 | 1 |
COL11A1 | 0 | 1 | 0 | 0 | 1 |
COL11A2 | 1 | 0 | 0 | 0 | 1 |
COL1A1, LOC126862586 | 1 | 0 | 0 | 0 | 1 |
COL5A1, LOC101448202 | 0 | 0 | 1 | 0 | 1 |
COL5A2 | 0 | 1 | 0 | 0 | 1 |
COL6A1 | 1 | 0 | 0 | 0 | 1 |
COL6A2 | 0 | 1 | 0 | 0 | 1 |
COL6A3 | 0 | 0 | 1 | 0 | 1 |
CRTAP | 0 | 0 | 1 | 0 | 1 |
CTNND1, TMX2-CTNND1 | 1 | 0 | 0 | 0 | 1 |
CUL3 | 0 | 1 | 0 | 0 | 1 |
CYP24A1 | 0 | 1 | 0 | 0 | 1 |
DEPDC5 | 0 | 1 | 0 | 0 | 1 |
DHDDS | 0 | 1 | 0 | 0 | 1 |
DLG4 | 1 | 0 | 0 | 0 | 1 |
DLL4 | 0 | 0 | 1 | 0 | 1 |
DNAAF3 | 1 | 0 | 0 | 0 | 1 |
DNAI2 | 0 | 1 | 0 | 0 | 1 |
DNM1L | 1 | 0 | 0 | 0 | 1 |
DNMT3A | 1 | 0 | 0 | 0 | 1 |
DOK7 | 0 | 1 | 0 | 0 | 1 |
DSP | 0 | 0 | 1 | 0 | 1 |
DYNC1H1 | 0 | 0 | 1 | 0 | 1 |
DYRK1A | 1 | 0 | 0 | 0 | 1 |
EBP | 1 | 0 | 0 | 0 | 1 |
ECHS1 | 0 | 1 | 0 | 0 | 1 |
EGR2 | 0 | 0 | 1 | 0 | 1 |
ENO3 | 0 | 0 | 1 | 0 | 1 |
ERCC6 | 1 | 0 | 0 | 0 | 1 |
ETFDH | 0 | 0 | 1 | 0 | 1 |
EXOSC3 | 1 | 0 | 0 | 0 | 1 |
EXT1 | 1 | 0 | 0 | 0 | 1 |
EXT2 | 0 | 1 | 0 | 0 | 1 |
EYA1 | 0 | 1 | 0 | 0 | 1 |
EZH2 | 1 | 0 | 0 | 0 | 1 |
FANCA, LOC130059837 | 0 | 0 | 1 | 0 | 1 |
FARS2 | 0 | 0 | 1 | 0 | 1 |
FAT1, LOC126807255 | 0 | 1 | 0 | 0 | 1 |
FBLN5 | 0 | 0 | 1 | 0 | 1 |
FBN1, LOC126862124 | 1 | 0 | 0 | 0 | 1 |
FBXO11 | 0 | 0 | 1 | 0 | 1 |
FBXO38 | 0 | 0 | 1 | 0 | 1 |
FDXR | 0 | 1 | 0 | 0 | 1 |
FH | 1 | 0 | 0 | 0 | 1 |
FKBP10 | 1 | 0 | 0 | 0 | 1 |
FKRP | 0 | 1 | 0 | 0 | 1 |
FXN | 1 | 0 | 0 | 0 | 1 |
GAN | 1 | 0 | 0 | 0 | 1 |
GJB1 | 0 | 1 | 0 | 0 | 1 |
GLS | 0 | 0 | 1 | 0 | 1 |
GNA11 | 0 | 0 | 1 | 0 | 1 |
GNAS | 1 | 0 | 0 | 0 | 1 |
GNPTAB | 0 | 0 | 1 | 0 | 1 |
GREB1L | 0 | 0 | 1 | 0 | 1 |
GRHPR | 0 | 1 | 0 | 0 | 1 |
GRIN2A | 0 | 1 | 0 | 0 | 1 |
GRIN2B | 0 | 0 | 1 | 0 | 1 |
HINT1 | 1 | 0 | 0 | 0 | 1 |
HIVEP2 | 1 | 0 | 0 | 0 | 1 |
HNF4A | 0 | 0 | 1 | 0 | 1 |
HNRNPK | 0 | 1 | 0 | 0 | 1 |
HPDL | 0 | 1 | 0 | 0 | 1 |
HPRT1, LOC107032760, LOC129929047 | 1 | 0 | 0 | 0 | 1 |
HSPB1 | 0 | 1 | 0 | 0 | 1 |
HSPD1 | 0 | 0 | 1 | 0 | 1 |
IFIH1 | 0 | 0 | 0 | 1 | 1 |
IFT140, LOC126862260 | 0 | 1 | 0 | 0 | 1 |
IGF1R | 0 | 1 | 0 | 0 | 1 |
INPPL1 | 0 | 0 | 1 | 0 | 1 |
INSR | 0 | 1 | 0 | 0 | 1 |
IPO8 | 0 | 0 | 1 | 0 | 1 |
KCNH1 | 0 | 0 | 0 | 1 | 1 |
KCNJ1 | 0 | 0 | 1 | 0 | 1 |
KCNQ2 | 0 | 1 | 0 | 0 | 1 |
KCNT2 | 0 | 1 | 0 | 0 | 1 |
KIF1B | 0 | 0 | 1 | 0 | 1 |
KIF26A | 1 | 0 | 0 | 0 | 1 |
KMT2E, SRPK2 | 1 | 0 | 0 | 0 | 1 |
LAMA1 | 0 | 0 | 1 | 0 | 1 |
LAMB2 | 1 | 0 | 0 | 0 | 1 |
LDLR | 0 | 1 | 0 | 0 | 1 |
LEFTY2 | 0 | 0 | 1 | 0 | 1 |
LOC100507346, PTCH1 | 0 | 1 | 0 | 0 | 1 |
LOC107303340, VHL | 0 | 1 | 0 | 0 | 1 |
LOC107982234, WT1 | 1 | 0 | 0 | 0 | 1 |
LOC126859690, PKHD1 | 0 | 1 | 0 | 0 | 1 |
LOC129930352, P3H1 | 0 | 1 | 0 | 0 | 1 |
LOC129935184, TTN | 1 | 0 | 0 | 0 | 1 |
LOC132090497, PIGN | 0 | 1 | 0 | 0 | 1 |
LRP5 | 0 | 1 | 0 | 0 | 1 |
LZTR1 | 0 | 0 | 1 | 0 | 1 |
MAGEL2 | 0 | 0 | 1 | 0 | 1 |
MAP2K1 | 0 | 0 | 1 | 0 | 1 |
MARS1 | 0 | 0 | 1 | 0 | 1 |
MBD5 | 0 | 1 | 0 | 0 | 1 |
MDH2 | 0 | 1 | 0 | 0 | 1 |
MEF2C | 0 | 1 | 0 | 0 | 1 |
MEN1 | 0 | 1 | 0 | 0 | 1 |
MET | 0 | 0 | 1 | 0 | 1 |
MIAT, MN1, PITPNB, TTC28 | 0 | 0 | 1 | 0 | 1 |
MME | 0 | 0 | 1 | 0 | 1 |
MORC2 | 0 | 1 | 0 | 0 | 1 |
MPV17 | 0 | 1 | 0 | 0 | 1 |
MTMR2 | 1 | 0 | 0 | 0 | 1 |
MTRFR | 1 | 0 | 0 | 0 | 1 |
MYH3 | 0 | 0 | 1 | 0 | 1 |
MYH7 | 1 | 0 | 0 | 0 | 1 |
NAA15 | 0 | 1 | 0 | 0 | 1 |
NBAS | 0 | 1 | 0 | 0 | 1 |
NDUFS6 | 0 | 1 | 0 | 0 | 1 |
NEXMIF | 1 | 0 | 0 | 0 | 1 |
NFIX | 0 | 1 | 0 | 0 | 1 |
NIPBL | 0 | 0 | 1 | 0 | 1 |
NLGN3 | 0 | 1 | 0 | 0 | 1 |
NOTCH2 | 0 | 1 | 0 | 0 | 1 |
NOTCH3 | 0 | 1 | 0 | 0 | 1 |
NPHP3, NPHP3-ACAD11 | 0 | 1 | 0 | 0 | 1 |
NR3C2 | 1 | 0 | 0 | 0 | 1 |
NUP93 | 0 | 1 | 0 | 0 | 1 |
OBSCN | 0 | 1 | 0 | 0 | 1 |
OCA2 | 0 | 1 | 0 | 0 | 1 |
OSGEP | 0 | 0 | 1 | 0 | 1 |
P3H1 | 0 | 1 | 0 | 0 | 1 |
P4HB | 0 | 0 | 1 | 0 | 1 |
PACS2 | 0 | 0 | 1 | 0 | 1 |
PAX6 | 1 | 0 | 0 | 0 | 1 |
PAX8 | 0 | 1 | 0 | 0 | 1 |
PCDH15 | 0 | 1 | 0 | 0 | 1 |
PCDH19 | 0 | 0 | 1 | 0 | 1 |
PDGFA | 0 | 0 | 1 | 0 | 1 |
PDXK | 0 | 1 | 0 | 0 | 1 |
PEX26 | 0 | 1 | 0 | 0 | 1 |
PHIP | 0 | 1 | 0 | 0 | 1 |
PIGG | 0 | 1 | 0 | 0 | 1 |
PKD1, TSC2 | 0 | 0 | 1 | 0 | 1 |
PLCE1 | 0 | 0 | 1 | 0 | 1 |
PLOD2 | 0 | 0 | 1 | 0 | 1 |
PLS3 | 1 | 0 | 0 | 0 | 1 |
POGZ | 0 | 1 | 0 | 0 | 1 |
POLG | 1 | 0 | 0 | 0 | 1 |
POLG, POLGARF | 0 | 0 | 1 | 0 | 1 |
PPARG | 1 | 0 | 0 | 0 | 1 |
PPM1D | 0 | 0 | 1 | 0 | 1 |
PTPRO | 0 | 0 | 1 | 0 | 1 |
RAF1 | 0 | 0 | 1 | 0 | 1 |
RARB | 0 | 0 | 1 | 0 | 1 |
RNASEH2A | 0 | 1 | 0 | 0 | 1 |
ROBO4 | 0 | 0 | 1 | 0 | 1 |
RORB | 1 | 0 | 0 | 0 | 1 |
RUNX2 | 0 | 0 | 1 | 0 | 1 |
SACS | 1 | 0 | 0 | 0 | 1 |
SALL1 | 0 | 1 | 0 | 0 | 1 |
SCN10A | 0 | 0 | 1 | 0 | 1 |
SCN1A | 0 | 1 | 0 | 0 | 1 |
SDHD | 0 | 0 | 1 | 0 | 1 |
SERPINF1 | 0 | 0 | 1 | 0 | 1 |
SETD1A | 0 | 1 | 0 | 0 | 1 |
SH3TC2 | 1 | 0 | 0 | 0 | 1 |
SIN3A | 1 | 0 | 0 | 0 | 1 |
SLC12A6 | 0 | 0 | 1 | 0 | 1 |
SLC12A9 | 0 | 0 | 1 | 0 | 1 |
SLC16A2 | 1 | 0 | 0 | 0 | 1 |
SLC2A2 | 1 | 0 | 0 | 0 | 1 |
SLC34A3 | 0 | 0 | 1 | 0 | 1 |
SLC3A1 | 1 | 0 | 0 | 0 | 1 |
SLC4A4 | 0 | 1 | 0 | 0 | 1 |
SLC5A7 | 0 | 0 | 1 | 0 | 1 |
SMARCA2 | 0 | 1 | 0 | 0 | 1 |
SMN1 | 0 | 1 | 0 | 0 | 1 |
SON | 1 | 0 | 0 | 0 | 1 |
SPG11 | 1 | 0 | 0 | 0 | 1 |
SPTBN2 | 0 | 1 | 0 | 0 | 1 |
SRCAP | 0 | 0 | 1 | 0 | 1 |
STAT3 | 0 | 0 | 1 | 0 | 1 |
STRC | 1 | 0 | 0 | 0 | 1 |
SUCLA2 | 0 | 1 | 0 | 0 | 1 |
SUZ12 | 0 | 0 | 1 | 0 | 1 |
SYNE1 | 1 | 0 | 0 | 0 | 1 |
SYNGAP1 | 0 | 0 | 1 | 0 | 1 |
TAF8 | 0 | 0 | 1 | 0 | 1 |
TAPBPL, VAMP1 | 1 | 0 | 0 | 0 | 1 |
TAPT1 | 0 | 0 | 1 | 0 | 1 |
TCF20 | 1 | 0 | 0 | 0 | 1 |
TCOF1 | 1 | 0 | 0 | 0 | 1 |
TGFBR2 | 0 | 1 | 0 | 0 | 1 |
TGM5 | 0 | 1 | 0 | 0 | 1 |
TLK2 | 0 | 1 | 0 | 0 | 1 |
TNNI3 | 0 | 0 | 1 | 0 | 1 |
TRIO | 0 | 1 | 0 | 0 | 1 |
TSFM | 0 | 0 | 1 | 0 | 1 |
TUBA1A | 0 | 0 | 1 | 0 | 1 |
TUBB3 | 0 | 0 | 1 | 0 | 1 |
TUBB4A | 0 | 1 | 0 | 0 | 1 |
UBE3B | 1 | 0 | 0 | 0 | 1 |
UNC45B | 0 | 0 | 1 | 0 | 1 |
USH1G | 1 | 0 | 0 | 0 | 1 |
VCP | 1 | 0 | 0 | 0 | 1 |
VHL | 1 | 0 | 0 | 0 | 1 |
WAC | 1 | 0 | 0 | 0 | 1 |
WNK4 | 0 | 0 | 1 | 0 | 1 |
WNT1 | 0 | 1 | 0 | 0 | 1 |
ZNF469 | 0 | 0 | 1 | 0 | 1 |