Molecular Genetics

 

Disease OMIM gene OMIM Disease gene
46XY complete gonadal dysgenesis 605423 233420 DHH
46XY gonadal dysgenesis, complete or partial, with or without adrenalfailure

NR5A1
Abetalipoproteinemia
MTP
Adipositas

LEP
Adrenal hyperplasia, congenital
202010 CYP11B1
Adrenal hyperplasia, congenital 609300 202110 CYP17A1
Adrenal hyperplasia, congenital 210910 201910 CYP21A2
Adrenal hyperplasia, congenital 201810
HSD3B2
Afibrinogenemia, congenital 134820 202400 FGA
Afibrinogenemia, congenital 134830 202400 FGB
Afibrinogenemia, congenital 134850 202400 FGG
Alagille syndrome 1 (ALGS1)
118450 JAG1
Alagille syndrome 2 (ALGS2) 600275 610205 Notch 2
Albinism, oculocutaneous, type IA
203100 TYR
Albinism, oculocutaneous, type II 611409 203200 OCA2
Alpers-Huttenlocher syndrome 174763 203700 POLG
Alpha-1-Antitrypsin deficiency
107400 PI
Amelogenesis imperfecta 606585 204650 ENAM
Amelogenesis imperfecta type IIA1 603767 204700 KLK4
Amyotrophic lateral sclerosis ALS1 147450 105400 SOD1
Amyotrophic lateral sclerosis ALS10 612069 605078 TARDBP
Amyotrophic lateral sclerosis ALS4 608465 602433 SETX
Amyotrophic lateral sclerosis ALS6
608030 FUS
Angelman syndrome (AS) 601623 105830 15q11-q13 UBE3A
Angioedema, hereditary 606860 106100 C1NH
Aniridia, type II
106210 PAX6
Apolipoprotein E deficiency
107741 APOE
arrayCGH 105k
arrayCGH 244k
arrayCGH 44k
arrayCGH validation
Arthrogryposis, distal, type 1 (DA1) 190990 108120 TPM2
Arthrogryposis, distal, type 2A 160720 193700 MYH3
Arthrogryposis, distal, type 2B 191043 601680 TNNI2
Arthrogryposis, distal, type 2B 600692 601680 TNNT3
Asthma, susceptibility to 605238 600807 HNMT
Ataxia with Oculomotor Apraxia 1 606350 208920 APTX
Ataxia with Oculomotor Apraxia 2 608465 606002 SETX
Ataxia with Vitamin-E deficiency (VED) 600415 277460 TTPA
Bardet Biedl syndrome 1 BBS1
Bardet Biedl syndrome 10
BBS10
Bardet Biedl syndrome 12
BBS12
Bardet Biedl syndrome 2
BBS2
Bardet Biedl syndrome 6
BBS6 (=MKKS)
Basal Ganglia Disease 134790 606159 FTL
Beare-Stevenson syndrome 176943 123790 FGFR2
Beckwith-Wiedemann 600856 130650 CDKN1C
Beckwith-Wiedemann 103280; 604115 130650 different
Bernard-Soulier syndrome 606672/138720/173515 231200 GP1BA; GP1BB; GP9
Beta-Thalassemia (sickle cell anaemia) 141900 141900 HBB
Brachydactyly type B1, BDB1 602337 11300 ROR2
Brachydactyly type B2, BDB2 602991 611337 NOG
Branchiootorenal syndrome (BOR) 601653 113650 EYA1
Branchiootorenal syndrome (BOR) 601205 113650 SIX1
Branchiootorenal syndrome (BOR) 2 600963 610896 SIX5
Bruck syndrome type 2 601865 609220 PLOD2
Budd-Chiari 147796 600880 JAK2
C syndrome 606037 211750 CD96
CADASIL 600276 177400 NOTCH3
Campomelic Dysplasia 313430 114290 SOX9
Cardiofaciocutaneous syndrome 164757 115150 BRAF
Cardiofaciocutaneous syndrome 190070 115150 KRAS
Carnitine Palmitoyltransferase II Deficiency 600650 255110 CPT2
Cerebrotendinous Xanthomatosis 606530 213700 CYP27A1
Charcot-Marie-Tooth disease, type 1A (CMT1A) 601097 118220 PMP22
Charcot-Marie-Tooth disease, type 1B (CMT1B) 159440 118200 MPZ
Charcot-Marie-Tooth disease, type 1C (CMT1C) 603795 601098 LITAF
Charcot-Marie-Tooth disease, type 1D (CMT1D) 129010 607678 EGR2
Charcot-Marie-Tooth disease, type 2A (CMT2A) 608507 609260 MFN2
Charcot-Marie-Tooth disease, type 2C (CMT2C) 605427 606071 TRPV4
Charcot-Marie-Tooth disease, X-linked (CMTX1) 304040 302800 GJB1
Charcot-Marie-Tooth type 1E 601097 118220 PMP22
CHARGE 608892 214800 CHD7
Cherubism 602104 118400 SH3BP2
Chondrodysplasia punctata (CDPX2) 608674 302960 EBP
Chondrodysplasia punctata (CDPX2) 300180 300180 ARSE
Chondrodysplasia punctata rhicomelic 1 601757 302960 PEX7
Chondrodysplasia punctata, rhicomelic 2 602744 222765 GNPAT
Choroideremia 300390 303100 CHM
Cockayne syndrome A 609412 21640 ERCC8
Cockayne syndrome B 609413 133540 ERCC6
Colorectal cancer, hereditary nonpolyposis, type 1 (HNPCC1) 609309 120435 MSH2
Colorectal cancer, hereditary nonpolyposis, type 2 (HNPCC2) 120436 609310 MLH1
COPROPORPHYRIA 612732 121300 CPOX
Cornelia-de-Lange syndrome (CDL) 608667 122470 NIPBL
Cornelia-de-Lange syndrome (CDL)
122470 SMC1L1 (SMC1A)
Cornelia-de-Lange syndrome (CDL)

SMC3
Costello syndrome
218040 HRAS
cowden 176300 105210 TTR
Cowden Disease 601728 158350 PTEN
CRASH syndrome 308840 303350 L1CAM
Crigler-Najjar syndrome 191740 218800 UGT1A1
Crouzon syndrome 176943 123500 FGFR2
Currarino syndrome 142994 176450 HLXB9 (MNX1)
Cutaneous Malignant Melanoma

CDKN2A
Cystic Fibrosis (CF) 602421 219700 CFTR
Cystinuria 104614 220100 SLC3A1
Deafness, neurosensory, autosomal recessive (DFNB1) 121011
GJB2
Deafness, neurosensory, autosomal recessive (DFNB1) 604418 220290 GJB6
Dementia, frontotemporal, with or without parkinsonism

MAPT
Denys-Drash syndrome 607102 194080 WT1
Diastrophic dysplasia 606718 222600 SLC26A2
DiGeorge syndrome Velocardialfacial syndrome (VCFS)
188400, 192430 22q11.2 and other regions
Dravet syndrome 182389 604233 SCN1A
Duane-radial ray syndrome 607343 607323 SALL4
Dysautonomia,familial

IKBKAP
Dysfibrinogenemia 134850 202400 FGA
Dysfibrinogenemia 134830 202400 FGB
Dysfibrinogenemia 134850 202400 FGG
Dystonia 1
128100 DYT1
Dystonia, dopa-responsive (Segawa syndrome, DRD) 600225 128230 GCH1
Dystrophia myotonica I 605377 160900 DMPK
Dystrophia myotonica II 116955 602668 ZNF9
ectodermal dysplasia 602273
TP63
Ectodermal dysplasia 3, anhidrotic (ED3)
129490 EDAR
Ectodermal dysplasia, anhidrotic 606603 224900 EDARADD
Ectodermal dysplasia, X-linked (ED1) 300451 305100 ED1
Emery-Dreifuss muscular dystrophy (EMD2, EMD3) 150330 181350, 604929 LMNA
Encephalopathy, epileptic early infantile 182810 613477 SPTAN1
EPILEPSY OF UNVERRICHT AND LUNDBORG 601145 254800 CSTB
Epilepsy, benign neonatal, 1 (EBN1) 602235 121200 KCNQ2
EPILEPSY, BENIGN NEONATAL, 2; EBN2 602232 121201 KCNQ3
EPILEPSY, CHILDHOOD ABSENCE, 1; ECA1 137164 600131 GABRG2
EPILEPSY, CHILDHOOD ABSENCE, 1; ECA1 600570 600131 CLCN2
Epilepsy, generalized with febrile seizures plus (GEFS+), type 1 600235 604233 SCN1B
Epilepsy, generalized with febrile seizures plus (GEFS+), type 2 182389 604233 SCN1A
Epilepsy, generalized with febrile seizures plus (GEFS+), type 3 137140
GABRG2
EPILEPSY, JUVENILE MYOCLONIC; JME 601949 606904 CACNB4
EPILEPSY, JUVENILE MYOCLONIC; JME 137160 606904 GABRA1
EPILEPSY, JUVENILE MYOCLONIC; JME 600570 606904 CLCN2
EPILEPSY, LATERAL TEMPORAL LOBE, AUTOSOMAL DOMINANT; ADLTE 604619 600512 LGI1
Epilepsy, nocturnal frontal lobe, type 1 118504 600513 CHRNA4
Epilepsy, nocturnal frontal lobe, type 2 118504 603204 CHRNA4
Epilepsy, nocturnal frontal lobe, type 3 118507 605375 CHRNB2
Epilepsy, nocturnal frontal lobe, type 4 118502 610353 CHRNA2
Epilepsy, Pyriodoxine-dependent 107323 266100 ALDH7A1
EPILEPSY. Lafora

EPM2A
EPILEPSY. Lafora 608072
EPM2B = NHLRC1
Epileptic encephalopathy, early infantile 300460 300088 PCDH19
Epiphyseal dysplasia, multiple, 1 (EDM1) 600310 132400 COMP
Episodic Ataxia (EA1) 602420 160120 KCNA1
Episodic Ataxia (EA2) 601011 108500 CACNA1A
Episodic Ataxia (EA2) 601011 108500 CACNA1A
Episodic Ataxia (EA5)

CACNB4
Epstein syndrome 160775 153650 MYH9
EXUDATIVE VITREORETINOPATHY 1 604579 133780 FZD4
Exudative vitreoretinopathy 4

LRP5
Fabry disease
301500 GLA
Factor 10 deficiency 227600 227600 F10
Factor 11 deficiency

F11
Factor 2 deficiency 187930 176930 F2
Factor 5 deficiency 612309 227400 F5
Factor II deficiency 187930 176930 F2
Factor V deficiency, Leiden
227400 FV
Factor VII deficiency
227500 F7
FGFR2-related Craniosynostosis 176943
FGFR2
FGFR3-related Craniosynostosis

FGFR3
Fichtner syndrome 160775 153640 MYH9
Fragile X syndrome, type A 309550 309550 FMR1
Fragile X syndrome, type E 300806 309548 FMR2
Friedreich Ataxia 606829 229300 FXN1
FRONTOTEMPORAL DEMENTIA; FTD 138945 600274 GRN
Fuhrmann syndrome 601570 228930 WNT7A
FUMARASE DEFICIENCY 136850 606812 FH
Galactosemia 606999 230400 GALT
Gastric Cancer 192090 137215 CDH1
GENERALIZED EPILEPSY WITH FEBRILE SEIZURES PLUS 137163 604233 GABRD
Gilbert syndrome 191740 143500 UGT1A1
Gilles-de-la-Tourette syndrome

SLITRK1
Glaucoma 1 601771 137750 CYP1B1
GLUT1 DEFICIENCY SYNDROME 138140 606777 GLUT1 = SLC2A1
Glycogen storage disease 607839 607839 GBE1
GM1-Gangliosidiosis Type 1 611458 230500 GLB1
Growth Hormone Receptor (GHR) 600946 600946 GHR
Growth Hormone Releasing Hormone Receptor (GHRHR) 139191 139191 GHRHR
HAD deficiency
231530 HAD
Hallervorden-Spatz-Syndrom 606157 234200 PANK2
Hemochromatosis (HFE) 235200 235200 HFE
Hemochromatosis (TFR2)

TFR2
Hemophagocytic Lymphohistiocytosis, familial FHL2 170280 603553 PRF1
Hemophagocytic Lymphohistiocytosis, familial FHL3 608897 608898 UNC13D
Hemophagocytic Lymphohistiocytosis, familial FHL4 605014 603552 STX11
Hemophagocytic Lymphohistiocytosis, familial FHL5 613101 601717 STXBP2
Hepatitis C Virus Treatment Response

IL28B
HEREDITARY LEIOMYOMATOSIS WITH RENAL CELL CANCER (HLRCC) 136850 606812, 150800 FH
Hermansky-Pudlak Syndrome 604982/606118/607145 203300 HPS1, HPS3, HPS7
Holoprosencephaly 3 (HPE2) 600725 142945 SHH
Holoprosencephaly 5 (HPE5) 603073 609637 ZIC2
Holt-Oram syndrome (HOS) 601620 142900 TBX5
Huntington disease (HD) 613004 143100 HD
Hutchinson-Gilford progeria syndrome (HGPS) 150330 176670 LMNA
Hydrocephalus 308840 307000 L1CAM
Hyper-IgD syndrome (HIDS) 251170 260920 MVK
Hyper-IgE-syndrome

STAT3
Hypercalcemia 126065 143880 Cyp24A1
Hypercholesterolemia 143890 606945 LDLR
Hyperekplexia 138491
GLRA1
Hyperekplexia 138492
GLRB
Hyperferritinemia-cataract syndrome
600886 FTL
Hyperparathyroidism 601199 239200 CASR
Hypochondroplasia (HCH) 134934 146000 FGFR3
Hypokalemic periodic paralysis (HOKPP) 114208/603967 170400 CACNA1S, SCN4A
Hypolaemic periodic paralysis (HOKPP) 0 0 KCNE3
Hypoparathyroidism 603716 146200 GCM2
HYPOPHOSPHATEMIC RICKETS 300550 307800 PHEX
Incontinentia pigmentosa 300248 0 IKBKG/NEMO
Infantile-Onset Spinocerebellar Ataxia 612640 271245 C10ORF2
Ivemark syndrome 121014 208531 GJA1 (Cx43)
Jackson-Weiss syndrome (JWS) 176943 123150 FGFR2
Jewish Panel Carrier testing 21 most common mutations 0 0 0
Kallmann syndrome 1 (KAL1) 308700 308700 KAL1
Kallmann syndrome 2 (KAL2) 136350 147950 FGFR1
Kallmann syndrome 3 (KAL3) 607123 244200 PROKR2
Kallmann syndrome 4 (KAL4) 607002 610628 PROK2
Kallmann syndrome 5 (Kal5) 6088925 0 CHD7
Kallmann syndrome 6 (Kal6) 600483 0 FGF8
Kartagener syndrome 0 0 DNAI1/DNAH5
Kearns-Sayre syndrome (KSS) 0 530000 mit
Keutel syndrome 154870 245150 MGP
Langer-Giedion syndrome 604386 190350 TRPS1
LARON SYNDROME 600946 262500 GHR
Larson syndrome 603381 150250 FLNB
LEBER CONGENITAL AMAUROSIS 1; LCA1 600179 204000 GUCY2D
Leber Optic Atrophie 0 535000 mit
Leigh syndrome (LS) 0 256000 MTATP6
Leigh syndrome (LS) 185620 256000 SURF1
LEOPARD syndrome 1 176876 151100 PTPN11
LERI-WEILL DYSCHONDROSTEOSIS; LWD 312865 127300 SHOX/SHOY
Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation, LBSL 610956 611105 DARS2
Lissencephaly 605066 607432 247200 YWHAE
Lissencephaly 2 (Norman-Roberts type) 600514 257320 RELN
Lissencephaly-1 601545 607432 PAFAH1B1 = Lis1
Lissencephaly-3 602529 611603 TUBA1A
Long-QT-syndrome 2 152427 613688 KCNH2
LOWE SYNDROME 300535 309000 OCRL1
Lujan Fryns syndrome 0 0 MED12
Mannose-binding protein deficiency (MBL2) 154545 154545 MBL2
Marfan syndrome (MFS) 134797 154700 FBN1
Marfan syndrome type 2/LOEYS-DIETZ SYNDROME 190182 190182 TGFBR2
Marfan syndrome type 2/LOEYS-DIETZ SYNDROME 190181 608967 TGFBR1
Marple Syrup Urine Disease 608348 0 BCKDHA
Marple Syrup Urine Disease 248611 0 BCKDHB
Marple Syrup Urine Disease 248610 0 DBT
MASA syndrome 308840 303350 L1CAM
May-Hegglin Anomaly 160775 155100 MYH9
McArdle Disease (Glycogen storage disease V) 608455 232600 pygm
Meckel syndrome type 1 609883 249000 MKS1
Mediterranean fever, familial (FMF) 608107 249100 MEFV
Medullary cystic kidney disease 2 191845 603860 UMOD
MEGALENCEPHALIC LEUKOENCEPHALOPATHY WITH SUBCORTICAL CYSTS 605908 604004 MLC1
MELAS syndrome 0 540000 mit
METACHROMATIC LEUKODYSTROPHY 0 250100 ARSA
Methylmalonic aciduria and homocystinuria, cbIF type 612625 277380 LMBRD1
Migraine, familial hemiplegic, 3 (FHM3) 182389 609634 SCN1A
Miller-Dieker syndrome 0 247200 17p13.3
Mitochondrial DNA Deletion Syndrome Type 5 603921 612073 SUCLA2
MODY 1 600281 606391 HNF4A
MODY 2 138079 125851 GCK
MODY 3 0 600496 TCF1
MODY 4 600733 606392 IPF1
MODY 5 189907 137920 HNF1B
MODY 6 606394 0 NeuroD1
Morbus Osler 0 0 ENG, ALK1
Mowat-Wilson 605802 235730 ZFHX1B
MTHFR deficiency 607093 236250 MTHFR
Mucopolysaccharidosis type 1 (Hurler-Scheie syndrome) 252800 607015 IDUA
MUCOPOLYSACCHARIDOSIS TYPE IVA 612222 253000 GALNS
Muenke syndrome 134934 602849 FGFR3
Mulibrey nanism 605073 605073 TRIM37
MULTIPLE CUTANEOUS AND UTERINE LEIOMYOMATA (MCUL) 136850 606812 FH
Multiple endocrine Neoplasia, type 2A 0 171400 RET
Multiple endocrine Neoplasia, type 2B 0 162300 RET
Multiples Pterygium syndrome 100690 0 CHRNA1
Multiples Pterygium syndrome 100730 0 CHRNG
Muscle-eye-brain disease (MEB) 606822 253280 POMGNT1
Muscular dystrophy Becker (BMD) 300377 300376 BMD
Muscular dystrophy Duchenne (DMD) 0 310200 DMD
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 1A; LGMD1A 604103 159000 MYOT
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 1B; LGMD1B 150330 159001 LMNA
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 1C; LGMD1C 601253 607801 CAV3
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2A; LGMD2A 114240 253600 CAPN3
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2B; LGMD2B 603009 253601 DYSF
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2C; LGMD2C 608896 253700 SGCG
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2D; LGMD2D 600119 608099 SGCA
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2E; LGMD2E 600900 604286 SGCB
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2F; LGMD2F 601411 601411 SGCD
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2I; LGMD2I 606596 607155 FKRP
MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2K; LGMD2K 607423 609308 POMT1
Myasthenic syndrome, asso. with acetycholinreceptor deficiency 601592 608931 RAPSN
Myasthenic syndrome, asso. with episodic apnea 118490 254210 CHAT
MYASTHENIC SYNDROME, CONGENITAL 100725 608931 CHRNE
MYOADENYLATE DEAMINASE DEFICIENCY 0 102770 AMPD1
Myoclonic epilepsy juvenile susceptibility to 608815 254770 EFHC1
Myoclonic epilepsy with red ragged fibers (MERRF) 0 545000 MTTK
Myotonia congenita Becker (Myotonia autosomal recessive) 118425 255700 CLCN1
Myotonia congenita Thomson (Myotonia autosomal dominant) 118425 160800 CLCN1
NARP syndrome 0 551500 MTATP6
NEUROAXONAL DYSTROPHY, INFANTILE 605630 256600 PLA2G6
NEUROFIBROMATOSIS TYPE 1-LIKE SYNDROME; NFLS, Legius syndrome 611431 609291 SPRED1
Neurofibromatosis Type II 607379 0 NF2
NEUROFIBROMATOSIS, TYPE I 613113 162200 NF1
Neuropathy, hereditare sensory and autonomic, type II (HSAN2) 605232 201300 HSN2
Neuropathy, hereditary sensory and autonomic, HSAN1 0 0 HSN1(=SPTLC1)
NEUROPATHY, HEREDITARY SENSORY AND AUTONOMIC, TYPE V; HSAN5 162030 608654 NGFB= NGF
Neuropathy, hereditary, with liability to pressure palsies (HNPP) 601097 162500 PMP22
NEUTROPENIA, SEVERE CONGENITAL 0 300299 WAS
Neutropenia, severe congenital, x-linked (XLN) 0 300299 WAS
Niiemann Pick disease NPC1 607107 257220 NPC1
Niiemann Pick disease NPC2 601015 607625 NPC2
Noonan syndrome (NS1) 190070 163950 KRAS
Noonan syndrome (NS1) 176876 163950 PTPN11
Noonan syndrome (NS1) 164760 611553 RAF1
Noonan syndrome (NS1) 182530 163950 SOS1
Obesity, morbid 601007 0 LEPR
Obesity, severe 164160 0 LEP
Oculopharyngeale muskeldystrophy 164300 0 PABP2
Oligodontia 142983/604025/167416 0 MSX1, Axin2, Pax9
Ophthalmoplegia, progressive external 174763 0 POLG1
Ophthalmoplegia, progressive external type 3 606075 609286 C10ORF2, twinkle
Opitz syndrome 300552 300000 MID1
Osteogenesis imperfecta 120150/120160 0 COL1A1/COL1A2
PAI 0 173360 PAI
Pancreatitis, hereditary (PCTT) 606709 167800 PRSS1
Pancreatitis, hereditary (PCTT) 167790 167800 SPINK1
Paramyotonia congenita 603967 168300 SCN4A
Parkinson disease 2 (PARK2) 602544 600116 PARK2
Parkinson disease 8 (PARK8) 609007 607060 LRRK2
Partington syndrome 300382 309510 ARX
Pelizaeus Merzbacher 300401 0 PLP1
Periodic Fever, familial, autosomal-dominant 191190 142680 TNFRSF1A
Permanent Neonatal Diabetes Mellitus (PNDM), KCNJ11-Related 600937 606176 KCNJ11
Permanent Neonatal Diabetes Mellitus (PNDM), NEUROD1 606394 0 NEUROD1
Petras anomaly 601771 604229 CYP1B1
Petras-Plus syndrome 0 261540 B3GALTL
Peutz-Jeghers syndrome (PJS) 0 175200 0
Pfeiffer syndrome 0 101600 FGFR1
Pfeiffer syndrome 176943 101600 FGFR2
Pitt-Hopkins syndrome 602272 0 TCF4
Pituary hormone deficiency 601802 262600 HESX1
Pituary hormone deficiency 173110 262600 PIT1 = POU1F1
Pituary hormone deficiency 601538 262600 PROP1
Porphyria, acute intermittent 609806 176000 HMBS
Porphyria, CONGENITAL ERYTHROPOIETIC 606938 0 UROS
Prader-Willi syndrome (PW) 0 176270 15q11-q13
Progressive pseudorheumatoid arthropathy of childhood (PPAC) 603400 208230 WISP3
Propionicacidemia 232000 606054 PCCA
Propionicacidemia 232050 606054 PCCB
prph2 139320 174800 GNAS
Pseudoachondroplasia (PSACH) 0 177170 COMP
PSEUDOHYPOALDOSTERONISM, TYPE I 600983 177735 NR3C2
Pyridoxamine-5′-Phospahte Oxidase Deficiency 603287 610090 PNPO
Refsum disease 602026 266500 PHYH
Retinitis pigmentosa 1 (RP1) 0 180100 RP1
Retinitis pigmentosa 13 (RP13) 0 600059 PRPF8
Retinitis pigmentosa 14 (RP14) 179605 600132 PRPH2
Retinitis pigmentosa 18 (RP18) 607301 601414 HPRP3
Retinitis pigmentosa 3 (RP3) X-linked 312610 300029 RPGR
Retinitis pigmentosa 4 (RP4) 0 180380 RHO
Retinoschisis1, X-linked, juvenile (RS1) 601966 312700 RS1
Rett syndrome (RTT) 300005 312750 MECP2
Rett-like syndrome 300203 0 CDKL5
Rickets, Vitamin D-dependent 609506 264700 CYP27B1
Rickets, vitamin D-resistant, type IIA 0 0 VDR
Rieger syndrome (RIEG1) 601542 180500 PITX2
Robinow syndrome 268310 602337 ROR2
Rubinstein-Taybi syndrome (RSTS) 600140 600140 CREBBP
Sanfillippo syndrome (Mucopolysaccharidosis type IIIB) 609701 252920 NAGLU
SCHAD deficiency (HADH deficiency) 600890 231530 HADH
Schinzel phocomelia (Al-Awadi-Raas-Rothschild syndrome) 601570 276820 WNT7A
Sebastian syndrome 160775 605249 MYH9
Seizures, benign familial neonatal-infantile 182390 607745 SCN2A
Septooptic Dysplasia (SOD) 601802 182230 HESX1
Sever myoclonic epilepsy of infancy (SMEI) 182389 607208 SCN1A
Silver-Russell-Syndrome 0 0 0
Simpson-Golabi-Behmel syndrome (SGBS1) 300037 312870 GPC3
Smith-Lemli-Opitz syndrome 602858 270400 DHCR7
Smith-Magenis syndrome (SMS) 606586 182290 17p11.2; RAI1
Sotos syndrome 606681 117550 NSD1
Spastic Paraplegia 11 (SPG11) 0 0 SPG11
Spastic Paraplegia 2 (SPG2) 0 0 PLP1
Spastic Paraplegia 3A (SPG3A) 606439 182600 SPG3A
Spastic Paraplegia 4 (SPG4) 604277 182601 SPG4
Spastic Paraplegia 7 (SPG7) 602783 607259 SPG7
Spermatogenic failure, nonobstructive, Y-linked 0 415000 AZF
Spherocytosis, type 4 0 612653 SLC4A1
Spina bifida 610132 182940 VANGL1
Spina bifida 600533 182940 VANGL2
Spinal and bulbar muscular atrophy, Kennedy disease (SMAX1) 0 313200 AR
Spinal muscular atrophy, type1 (SMA1) 600354 253300 SMN1
Spinal muscular atrophy, type2 (SMA2) 600354 253550 <+
Spinal muscular atrophy, type3 (SMA3) 600354 253400 SMN1
Spinocerebellar Ataxia 1 (SCA1) 601556 164400 ATXN1
Spinocerebellar Ataxia 11 (SCA11) 611695 604432 TTBK2
Spinocerebellar Ataxia 12 (SCA12) 604325 604326 PPP2R2B
Spinocerebellar Ataxia 13 (SCA13) 176264 605259 KCNC3
Spinocerebellar Ataxia 14 (SCA14) 176980 605361 PRKCG
Spinocerebellar Ataxia 17 (SCA17) 600075 607136 TBP
Spinocerebellar Ataxia 2 (SCA2) 601517 183090 ATXN2
Spinocerebellar Ataxia 3 (Machado-Joseph, SCA3) 607047 109150 ATXN3
Spinocerebellar Ataxia 6 (SCA6) 601011 183086 CACNA1A
Spinocerebellar Ataxia 8 (SCA8) 603680 608768 ATXN8OS
Spinocerebellar Ataxia with Axonal Neuropathy, Autosomal Recessive (SCAN1) 607198 607250 TDP1
Stuve-Wiedemann 151443 601559 LIFR
subtelomeric screening 0 div 0
Swyer syndrome 480000 400044 SRY
Thanatophoric dysplasia (TD1) 134934 187600 FGFR3
THIOPURINE S-METHYLTRANSFERASE DEFICIENCY 187680 610460 TPMT
Thrombasthenia (Glanzmann and Naegli) 0 0 ITGB3
THROMBOCYTOPENIA 1 300392 313900 WAS
Thrombocytopenia 1 (THC1) 300392 313900 WAS
THYROID CARCINOMA, FAMILIAL MEDULLARY; MTC 191315 155240 NTRK1
Thyroid Dyshomogenesis 601843 274400 SLC5A5
Townes-Brokes syndrome 602218 107480 SALL1
Treacher Collins-Franceschatti syndrome (TCOF) 606847 154500 TCOF1
Triorhinophalangeal syndrome I+II (see Langer-Giedion) 0 0 0
TUBEROUS SCLEROSIS 605284 191100 TSC1
TUBEROUS SCLEROSIS 191092 191100 TSC2
Usher Syndrome 1B 0 0 Myo7A
Vas deferens, congenital bilateral aplasia of (CBAVD) 602421 277180 CFTR
Von Hippel-Lindau syndrome (VHL) 608537 193300 VHL
West syndrome 300382 308350 ARX
Williams-Beuren syndrome (WBS) 0 194050 7q11.2
Wilson disease 606882 277900 ATP7B
Wiskott-Aldrich syndrome (WAS) 300392 301000 WAS
XLAG syndrome 300382 300215 ARX