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Table 1 Tier 1 and tier 2 genes included in the OTOgenics™ panel (v3)

From: Comprehensive genomic diagnosis of non-syndromic and syndromic hereditary hearing loss in Spanish patients

Tier 1 (154 genes): genes with strong/moderate association with SNHL#
 ABHD12 BSND CLRN1 DIABLO FTO HSD17B4 MARVELD2 MYO3A P2RX2 PTPN11 SLC52A3 TMIE
  NM_001042472.2 NM_057176.2 NM_174878.2 NM_019887.5 NM_001080432.2 NM_000414.3 NM_001038603.2 NM_017433.4 NM_174873.2 NM_002834.3 NM_033409.3 NM_147196.2
 ACTB CABP2 COCH DIAPH1 GATA3 ILDR1 MASP1 MYO6 PAX3 PTPRQ SLITRK6 TMPRSS3
  NM_001101.3 NM_016366.2 NM_004086.2 NM_005219.4 NM_001002295.1 NM_001199799.1 NM_139125.3 NM_004999.3 NM_181457.3 NM_001145026.1 NM_032229.2 NM_024022.2
 ACTG1 CACNA1D COL2A1 DNMT1 GIPC3 KARS MIR96 MYO7A PCDH15 RAF1 SMPX TPRN
  NM_001614.3 NM_000720.3 NM_001844.4 NM_001130823.1 NM_133261.2 NM_001130089.1 NR_029512.1 NM_000260.3 NM_033056.3 NM_002880.3 NM_014332.2 NM_001128228.2
 ADGRV1 CCDC50 COL4A3 ECHS1 GJB2 KCNE1 MITF MYO15A PDZD7 RDX SNAI2 TRIOBP
  NM_032119.3 NM_178335.2 NM_000091.4 NM_004092.3 NM_004004.5 NM_000219.5 NM_000248.3 NM_016239.3 NM_001195263.1 NM_002906.3 NM_003068.4 NM_001039141.2
 AIFM1 CDH23 COL4A4 EDN3 GJB3 KCNJ10 MSRB3 NARS2 PEX1 RMND1 SOX10 TSPEAR
  NM_004208.3 NM_022124.5 NM_000092.4 NM_207034.2 NM_024009.2 NM_002241.4 NM_198080.3 NM_024678.5 NM_000466.2 NM_017909.3 NM_006941.3 NM_144991.2
 ALMS1 CEACAM16 COL4A5 EDNRB GJB6 KCNQ1 MT-CO1 NDP PEX2 SERAC1 SPATA5 USH1C
  NM_015120.4 NM_001039213.3 NM_000495.4 NM_000115.3 NM_006783.4 NM_000218.2 NC_012920.1 NM_000266.3 NM_000318.2 NM_032861.3 NM_145207.2 NM_005709.3
 ANKH CHD7 COL9A1 EPS8L2 GPSM2 KCNQ4 MT-RNR1 NLRP3 PEX3 SERPINB6 STRC USH1G
  NM_054027.4 NM_017780.3 NM_001851.4 NM_022772.3 NM_013296.4 NM_004700.3 NC_012920.1 NM_004895.4 NM_003630.2 NM_004568.5 NM_153700.2 NM_173477.4
 AP1S1 CIB2 COL11A1 ESPN GRHL2 LARS2 MT-TH OPA1 PEX5 SIX1 SYNE4 USH2A
  NM_001283.3 NM_006383.3 NM_001854.3 NM_031475.2 NM_024915.3 NM_015340.3 NC_012920.1 NM_015560.2 NM_001131025.1 NM_005982.3 NM_001039876.1 NM_206933.2
 ATP1A3 CISD2 COL11A2 ESRRB GRXCR1 LHFPL5 MT-TK OSBPL2 PEX6 SLC17A8 TBC1D24 WFS1
  NM_152296.4 NM_001008388.4 NM_080680.2 NM_004452.3 NM_001080476.2 NM_182548.3 NC_012920.1 NM_144498.2 NM_000287.3 NM_139319.2 NM_001199107.1 NM_006005.3
 ATP6V1B1 CLCNKA DCAF17 EYA1 HARS2 LHX3 MT-TL1 OTOA PEX26 SLC19A2 TECTA WHRN
  NM_001692.3 NM_004070.3 NM_025000.3 NM_000503.5 NM_012208.3 NM_014564.3 NC_012920.1 NM_144672.3 NM_017929.5 NM_006996.2 NM_005422.2 NM_015404.3
 BCAP31 CLCNKB DDX11 EYA4 HGF LOXHD1 MT-TS1 OTOF POU3F4 SLC26A4 TIMM8A XYLT2
  NM_001139441.1 NM_000085.4 NM_030653.3 NM_004100.4 NM_000601.4 NM_144612.6 NC_012920.1 NM_194248.2 NM_000307.4 NM_000441.1 NM_004085.3 NM_022167.3
 BCS1L CLDN14 DFNA5 FGF3 HOXA1 LRP2 MYH9 OTOG POU4F3 SLC33A1 TJP2  
  NM_004328.4 NM_144492.2 NM_004403.2 NM_005247.2 NM_005522.4 NM_004525.2 NM_002473.5 NM_001277269.1 NM_002700.2 NM_004733.3 NM_004817.3  
 BRAF CLPP DFNB59 FGFR3 HOXB1 LRTOMT MYH14 OTOGL PRPS1 SLC52A2 TMC1  
  NM_004333.4 NM_006012.2 NM_001042702.3 NM_000142.4 NM_002144.3 NM_001145308.4 NM_024729.3 NM_173591.3 NM_002764.3 NM_024531.4 NM_138691.2  
Tier 2 (45 genes): genes with weak/preliminary association with SNHL#
 ADCY1 COL4A6 CRYM ELMOD3 FGFR1 GTF2IRD1 KITLG MT-CO3 NDUFA13 SIX5 TK2 TP63
  NM_021116.2 NM_001847.3 NM_001888.4 NM_032213.4 NM_023110.2 NM_016328.2 NM_000899.4 NC_012920.1 NM_015965.6 NM_175875.4 NM_004614.4 NM_003722.4
 ATP2B2 COL9A2 DCDC2 EPS8 FGFR2 HMX2 MAF MT-TA NFIX SLC4A11 TMEM132E  
  NM_001683.3 NM_001852.3 NM_016356.4 NM_004447.5 NM_000141.4 NM_005519.1 NM_005360.4 NC_012920.1 NM_001271043.2 NM_032034.3 NM_001304438.1  
 ATP6V1B2 COL9A3 DIAPH3 FAM65B FOXI1 HMX3 MARS2 MT-TE PNPT1 SLC9A1 TMPRSS5  
  NM_001693.3 NM_001853.3 NM_001042517.1 NM_014722.3 NM_012188.4 NM_001105574.1 NM_138395.3 NC_012920.1 NM_033109.4 NM_003047.4 NM_030770.3  
 BDP1 COQ6 DSPP FBLN1 GRXCR2 HOMER2 MCM2 MT-TS2 SEMA3E SLC26A5 TNC  
  NM_018429.2 NM_182476.2 NM_014208.3 NM_006486.2 NM_001080516.1 NM_004839.3 NM_004526.3 NC_012920.1 NM_012431.2 NM_198999.2 NM_002160.3  
  1. #Sensorineural hearing loss