Panele NGS

1. Padaczka (panele IMiD):

Panel EIEE v.1/2016 (49 genów)

ALDH7A1, ALG13, ARX, CACNA1A, CASK, CDKL5, CHD2, DNM1, FOXG1, GABRA1, GABRB3, GABRG2, GNAO1, GRIN1, GRIN2A, GRIN2B, HCN1, HNRNPU, IQSEC2, KCNA2, KCNB1, KCNQ2, KCNQ3, KCNT1, MBD5, MECP2, MEF2C, PCDH19, PNPO, POLG, PRRT2, SCN1A, SCN1B, SCN2A, SCN8A, SCN9A, SIK1, SLC25A22, SLC2A1, SLC35A2, SLC9A6, SPTAN1, STXBP1, SYNGAP1,TBC1D24, TBL1XR1, TCF4, UBE3A, ZEB2

Panel NBE v.1/2018 (83 geny)

ABAT, ALDH4A1, ALDH7A1, ALG13, ALPL, AMT, ARHGEF9, ARX, BTD, CACNA1A, CASK, CDKL5, CHD2, DNM1, FOLR1, FOXG1, GABRA1, GABRB3, GABRG2, GAMT, GATM, GLDC, GLRA1, GLRB, GNAO1, GPHN, GRIN1, GRIN2A, GRIN2B, HCN1, HNRNPU, IQSEC2, KCNA2, KCNB1, KCNJ10, KCNQ2, KCNQ3, KCNT1, LIAS, MBD5, MECP2, MEF2C, MOCS1, MOCS2, PCDH19, PGAP2, PHGDH, PHOX2B, PIGA, PIGO, PIGV, PMM2, PNPO, POLG, PROSC, PRRT2, PSAT1, PSPH, PURA, SCN1A, SCN1B, SCN2A, SCN8A, SCN9A, SIK1, SLC25A22, SLC2A1, SLC35A2, SLC6A5, SLC6A8, SLC9A6, SPTAN1, STXBP1, SUOX, SYNGAP1, TBC1D24, TBL1XR1, TCF4, TPP1, TSC1, TSC2, UBE3A, ZEB2

2. Encefalopatie padaczkowe

OMIM Epileptic encephalopathy, early infantile – PS308350 – 89 Entries (OMIM www.omim.org/phenotypicSeries/PS308350)

Genetic epilepsy syndromes Version 2.143 (Genomics England PanelApp; https://panelapp.genomicsengland.co.uk/panels/402/)

3. Choroba Parkinsona

OMIM Parkinson Disease Phenotypic Series – PS168600; (OMIM; https://www.omim.org/phenotypicSeries/PS168600)

Parkinson Disease and Complex Parkinsonism V 1.68; (Genomics England PanelApp; https://panelapp.genomicsengland.co.uk/panels/39/)

ADAR, ADCY5, ADH1C, ANO3 , ATM, ATP13A2 , ATP1A3, ATP7B, BCAP31, C19ORF12, CACNA1A, CHCHD2 , COASY, COL6A3 , COX20, CSF1R, DCTN1, DNAJC12, DNAJC6, EIF4G1, FA2H, FBXO7, FTL, GBA, GCDH, GCH1, GIGYF2, GLUD2, GNAL, GRN, HPCA , HTRA2 , KCNA1, KCNMA1, KCTD17, KIF1C, KMT2B, LRRK2, LYST, MAPT, MECR, MR1, OPA3, PANK2, PARK2, PARK7 (DJ1), PINK1, PLA2G6, PNKD (MR1), PRKN (PARK2), PRKRA, PRRT2 , RAB39B, SCN8A, SGCE , SLC19A3, SLC30A10, SLC2A1, SLC30A10, SLC39A14, SLC6A3, SNCA, SPG11, SPR, SYNJ1, TAF1, TH, THAP1, TOR1A, TUBB4A, UCHL1, VAC14, VPS13A, VPS13C, VPS35, WDR45

4. Dystonia

OMIM Phenotypic Series Seizures, Dystonia – PS128100 (OMIM; https://www.omim.org/phenotypicSeries/PS128100)

ADAR, ADCY5, ANO3, ATM, ATP1A3, ATP7B, BCAP31, CACNA1A, COASY, COL6A3, COX20, DNAJC12, FA2H, FBXO7, FTL, GCDH, GCH1, GNAL, HPCA, KCD17, KCNA1, KCNMA1, KIF1C, KMT2B, MECR, PANK2, PINK1, PLA2G6, PNKD (MR1 – ex1), PRKN (PARK2), PRKRA, PRRT2, SCN8A, SGCE, SLC19A3, SLC2A1, SLC30A10, SLC39A14, SLC6A3, SPR, TAF1, TH, THAP1, TOR1A, TUBB4A, VAC14, VPS13A

5. Pęcherzowe oddzielanie naskórka (Epidermolysis bullosa)

COL17A1, COL7A1, DSP, DST, EXPH5, FERMT1, ITGA6, ITGB4, JUP, KRT5, KRT14, LAMA3, LAMB3, LAMC2, PKP1, PLEC, TGM5, VPS33B, CHST8, ITGA3, CD151, KLHL24

6. Rybia łuska

AAGAB, ABCA12, ABHD5, ADAM10, ALDH3A2, ALOX12B,, ALOXE3, AP1S1, AQP5, CDSN, CLDN1, CSTA, CTSC, CYP4F22, DSG1, DSP, EBP, ENPP1, ERCC2, ERCC3, EXPH5, FERMT1, FLG (fragment sekwencji kodującej), GJA1, GJB2, GJB3, GJB4, GTF2H5, HOXC13, JUP, KANK2, KRT1, KRT10, KRT16, KRT17, KRT2, KRT9, LIPN, LOR, MBTPS2, MPLKIP, NIPAL4, NSDHL, PEX7, PHYH, PKP1, PNPLA1, POFUT1, POGLUT1, POMP, SERPINB7, SLC27A4, SLURP1, SNAP29, SPINK5, ST14, STS, SUMF1, TGM1, TRPV3, VPS33B

7.Niepłodność męska i żeńska (panel IMiD)

Analiza wybranych genów związanych z zespołem Kallmanna, hipogonadyzmem hipogonadotropowy (z anosmią lub bez)), zespołem CHARGE, zaburzeniami nasienia , niewrażliwością na androgeny, nawracającymi poronieniami/zakrzepicą, dysgenezją jajników:

ANOS1 (KAL), AR, BMP15, CFTR , CHD7, DUSP6, F2, F5, FEZF1, FGF17, FGF8, FGFR1, FLRT3, FSHB, FSHR, GNRH1, GNRHR, IL17RD, KISS1, KISS1R, LHB, NSMF, PROK2, PROKR2, SEMA3A, SOX10, SPATA16, SPRY4, WDR11

8. Wybrane choroby metaboliczne (panel IMiD)

Choroba syropu klonowego: BCKDHA, BCKDHB, DBT

Acyduria metylomalonowa/zaburzenia kobalaminy: MMUT, MCEE, MMAA, MMAB

Acyduria metylomalonowa z homocysteinurią: MMADHC, MMACHC

Fenyloketonuria, hiperfenyloalaninemia, deficyt BH4, zaburzenia neurotransmisji dopaminergicznej i serotoninergicznej: GCH1, PTS, SPR, PCBD1, QDRP, QDRP, PAH, TH, TPH, DDC, MAOA, DBH, DNAJC12

9. Niedosłuch (panel IMiD)

ACTG1, ADCY1, ADGRV1, AIFM1, ARSG, ATP2B2, ATP6V1B2, BCS1L, BSND, CATSPER2, CD164, CDC14A, CDH23, CEACAM16, CHD7, CIB2, CISD2, CLDN14, CLDN9, CLIC5, CLPP, CLRN1, CLRN2, COCH, COL11A1, COL11A2, COL4A3, COL4A4, COL4A5, COL4A6, COL9A1, COL9A2, COL9A3, CRYM, DCDC2, DIABLO, DIAPH1, DIAPH3, DMXL2, EDN3, EDNRB, ELMOD3, EPS8, EPS8L2, ERAL1, ESPN, ESRP1, ESRRB, EYA1, EYA4, FDXR, FGF3, FOXI1, GAB1, GATA3, GIPC3, GJB2, GJB3, GJB6, GPRASP2, GPSM2, GRAP, GREB1L, GRHL2, GRXCR1, GRXCR2, GSDME, HARS1, HARS2, HGF, HOMER2, HSD17B4, ILDR1, KARS1, KCNE1, KCNJ10, KCNQ1, KCNQ4, KITLG, LARS2, LHFPL5, LMX1A, LOXHD1, LRTOMT, MAP1B, MARVELD2, MET, MIR96, MITF, MPZL2, MSRB3, MYH14, MYH9, MYO15A, MYO3A, MYO6, MYO7A, OSBPL2, OTOA, OTOF, OTOG, OTOGL, P2RX2, PAX3, PCDH15, PDE1C, PDZD7, PJVK, PLS1, PNPT1, POLR1B, POLR1C, POLR1D, POU3F4, POU4F3, PPIP5K2, PRPS1, PTPRQ, RDX, REST, RIPOR2, ROR1, S1PR2, SCD5, SEMA3E, SERPINB6, SIX1, SIX5, SLC12A2, SLC17A8, SLC26A4, SLC26A5, SLC4A11, SLITRK6, SMPX, SOX10, SPNS2, STRC, SYNE4, TBC1D24, TCOF1, TECTA, TMC1, TMEM132E, TMIE, TMPRSS3, TNC, TPRN, TRIOBP, TRRAP, TSPEAR, TWNK/C10ORF2, USH1C, USH1G, USH2A, WBP2, WFS1, WHRN

10. Dysplazja szkieletowa (panel IMiD)

PTH1R, ALX1, ALX3, C21ORF2, IFT122, IFT43, IFT52, WDR35, GJA1, MAP3K7, ADAMTSL2, SOX9, FN1, TTC21B, COL9A3, ALPL, ALX4, NIN, LOXL3, NOTCH2, TWIST2, IFT80, FLNA, IFT140, WDR19, COL9A2, COL2A1, COL11A2, COL11A1, COL9A1, SCARF2

11. Dyzostozy (panel IMiD)

ZSWIM6, PDE4D, EDNRA, EFTUD2, POLR1A, PRKAR1A, CTSK, TXNL4A, EVC, EVC2, SF3B4, POLR1C, POLR1D, TCOF1

12. Exostozy (panel IMiD)

EXT1, EXT2

13. Fenotyp marfanoidalny (panel IMiD)

COL6A1, COL6A2, COL6A3, FBN2, SMAD3, TGFB2, TGFB3, TGFBR1, TGFBR2, FBN1

14. Kraniosynostoza (panel IMiD)

PHEX, MED13L, FGFR3, IHH, NOG, SIX2, EFNB1, TMCO1, SPRY4, TRIM37, IL11RA, CYP26B1, MSX2, SMAD6, TCF12, ZIC1, ERF, IDS, HUWE1, FREM1, JAG1, POR, KAT6A, ASXL1, RAB23, MEGF8, GLI3, TFAP2B, P4HB, SEC24D, FGFR2, SMO, IRX5, FGF9, ZEB2, RUNX2, MID1, SPECC1L, FGFR1, TBL1XR1, SLC25A24, FAM20C, ESCO2, RECQL4, TWIST1, SKI, SOX6, ZNF462, AHDC1

15. Niskorosłość (panel IMiD)

STAT3, BMP2, SOST, GH1, GHRHR, GHSR, BTK, GHR, GNPTAB, ARSB, IDUA, HESX1, STAT5B, RNPC3, ACAN, GSC, IGF1R, IGFALS, LHX3, LHX4, NPR2, PCNT, RPL10, SHOX, SOX3, TOP3A, POU1F1, PROP1, IGF1, CRIPT, DONSON, OTX2, AMER1, GLI1, GNAS, SOX2, B3GAT3, COLEC10, COLEC11, MASP1, AMMECR1, PITX2, ASXL3, BLM, PNPLA6, TGDS, GLI2, WNT7A, PROKR2, FAM111A, TBCE, NEK1, DYNC2H1, CDC45, CDC6, CDT1, GMNN, MCM5, ORC1, ORC4, ORC6, DHODH, SAMD9, SNRPB, LIG4, MED12, LMX1B, INSR, EIF4A3, DVL1, DVL3, FZD2, NXN, ROR2, WNT5A, RNU4ATAC, ATR, CENPJ, CEP152, CEP63, DNA2, NSMCE2, RBBP8, TRAIP, PIK3R1, H19, IGF2, GRHL3, NSD2

16. RASopatie i zespoły dysmorficzne (panel IMiD)

NF1, FBXW11, TRAF7, ACTB, ACTG1, CCDC8, OBSL1, CUL7, FGD1, HNRNPK, PHF6, RPS6KA3, ARID1A, ARID1B, ARID2, DPF2, SMARCA4, SMARCC2, SMARCD2, SMARCE1, SOX11, SOX4, SMARCB1, BRD4, HDAC8, NIPBL, RAD21, SMC1A, SMC3, HRAS, SH3PXD2B, ANKRD11, KDM6A, KMT2D, EHMT1, KCNJ2, SPRED1, SMAD4, SMARCA2, SETD5, A2ML1, KRAS, MAPK1, MRAS, NRAS, PTPN11, RAF1, RIT1, RRAS, RRAS2, SOS1, SOS2, BRAF, CDC42, LZTR1, CBL, PPP1CB, SHOC2, EP300, CREBBP, KAT6B, MAP2K1, MAP2K2, HMGA2, PLAG1, DHCR7, IRF6, KMT2A

17. Schwannomatoza

NF2, KIT

18. Wrodzona łamliwość kości (panel IMiD)

COL1A2, COL1A1, PPIB, IFITM5, FKBP10, BMP1, CREB3L1, CRTAP, MBTPS2, FAM46A, SERPINF1, P3H1, SERPINH1, SP7, TMEM38B, WNT1, SPARC

19. Zespół CHARGE (panel IMiD)

CHD7, SEMA3E

20. Zespół Ehlersa-Danlosa (panel IMiD)

SLC39A13, ADAMTS2, B3GALT6, TNXB, COL5A1, COL5A2, FKBP14, PLOD1, AEBP1, COL12A1, COL3A1, C1R, C1S, CHST14, DSE

21. Zespół Gorlina (panel IMiD)

PTCH1, PTCH2, SUFU

22. Zespół Klippel-Feila (panel IMiD)

GDF3, GDF6, MEOX1, RIPPLY2

23. Zespół nadmiernego wrostu (overgrowth) (panel IMiD)

KLLN, DNMT3A, AKT2, HERC1, AKT3, CCND2, PIK3R2, PPP2R5D, TCF20, CHD8, PDGFRB, CDKN1C, ABCC9, EED, PIK3CA, SEC23B, PTEN, AKT1, LRP2, SUZ12, GPC4, SETD2, DIS3L2, HIST1H1E, ASXL2, GPC3, OFD1, MTOR, NSD1, NFIX, RNF125, FIBP, RNF135, EZH2

24. Zespół uszno-kłykciowy (panel IMiD)

EDN1, GNAI3, PLCB4

25. Analiza genów związanych z niepełnosprawnością intelektualną na bazie listy genów  wg Genomics England PanelApp