Canavan Disease |
ACY2 deficiency, aminoacylase 2 deficiency, ASP deficiency, ASPA deficiency, aspartoacylase deficiency, Canavan-van Gogaert-Bertrand disease, spongy degeneration of central nervous system |
ASPA |
autosomal recessive |
Carey-Fineman-Ziter Syndrome |
CFZS, congenital nonprogressive myopathy with Moebius and Robin sequences |
MYMK |
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Carpenter Syndrome |
ACPS II, acrocephalopolysyndactyly type II |
RAB23 |
autosomal recessive |
Cataracts 13, Congenital, in Adult i RBC Phenotype |
CTRCT13, GCNT2 |
GCNT2 |
autosomal recessive |
Cataracts 34 |
cataract 34 multiple types with or without microcornea, CTRCT34 |
FOXE3 |
autosomal recessive |
Cataracts 43 |
CTRCT43 |
UNC45B |
autosomal dominant |
Cataracts 45 |
CTRCT45 |
SIPA1L3 |
autosomal recessive |
Cataracts 46, Juvenile-Onset |
CTRCT46, juvenile cataract Hutterite type |
LEMD2 |
autosomal recessive |
Cataracts and Ichthyosis |
|
? |
? |
Cataracts, Anterior Polar 2 |
CTAA2 |
17p13 locus |
autosomal dominant |
Cataracts, Anterior Polar with Guttata |
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TMCO3 |
autosomal dominant |
Cataracts, Ataxia, Short Stature, and Mental Retardation |
CASH syndrome |
Xpter-q13.1 locus |
X-linked recessive |
Cataracts, Congenital Cerulean |
blue dot congenital cataract, CCA1, CCA2, CCA3, CCA4, CCA5 |
CCA5, CRYBB2, CRYGD, MAF |
autosomal dominant |
Cataracts, Congenital Nuclear |
CATCN1, CATCN2, CATCN3 |
CRYBB1, CRYBB3 |
autosomal recessive |
Cataracts, Congenital Sutural with Punctate and Cerulean Opacities |
CSPC |
CRYBB2 |
autosomal dominant |
Cataracts, Congenital with Sclerocornea and Glaucoma |
Anterior Segment Dysgenesis 7, ASGD7 |
PXDN |
autosomal recessive |
Cataracts, Congenital Zonular Pulverulent 1 |
CAE1, CTRCT1, CZP, CZP1, Duffy-linked cataract |
GJA8 |
autosomal dominant |
Cataracts, Congenital Zonular Pulverulent 3 |
CZP3 |
GJA3 |
autosomal dominant |
Cataracts, Congenital Zonular With Sutural Opacities |
CCZS |
CRYBA1 |
autosomal dominant |
Cataracts, Congenital, and Hypomyelinating Leukodystrophy |
HCC, hypomyelination and congenital cataract |
FAM126A |
autosomal recessive |
Cataracts, Congenital, Autosomal Dominant |
lens opacities |
BFSP2, GJA3, MIP, PAX6 |
autosomal dominant |
Cataracts, Congenital, Autosomal Recessive 2 |
CATC2 |
FYCO1 |
autosomal recessive |
Cataracts, Congenital, Autosomal Recessive 3 |
CATC3 |
1p34.3-p32.2 locus |
autosomal recessive |
Cataracts, Congenital, Autosomal Recessive 4 |
CATC4 |
TDRD7 |
autosomal recessive |
Cataracts, Congenital, Autosomal Recessive 5 |
CATC5 |
AGK |
autosomal recessive |
Cataracts, Congenital, Deafness, Short Stature, Developmental Delay |
Ayme-Gripp syndrome |
MAF |
autosomal dominant |
Cataracts, Congenital, Facial Dysmorphism, and Neuropathy |
CCFDN, congenital cataracts with facial dysmorphism and neuropathy |
CTDP1 |
autosomal recessive |
Cataracts, Congenital, Intellectual Disability, Abnormal Striatum, and ADHD |
|
KCNA4 |
autosomal recessive |
Cataracts, Congenital, Posterior Polar |
CTPA, CTPP, CTPP1, posterior polar cataract |
EPHA2 |
autosomal dominant |
Cataracts, Congenital, Volkmann Type |
CCV, Volkmann type congenital cataract |
1pter-p36.13 locus |
autosomal dominant |
Cataracts, Congenital, with Brain Hemorrhage and Subependymal Calcification |
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JAM3 |
autosomal recessive |
Cataracts, Congenital, with Cleft Palate |
|
? |
autosomal dominant |
Cataracts, Congenital, with Intellectual Disability |
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STX3 |
autosomal recessive |
Cataracts, Congenital, With Short Stature and Minor Skeletal Anomalies |
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BRD4 |
autosomal dominant |
Cataracts, Congenital, X-Linked |
CCT, congenital total cataract with posterior sutural opacities in heterozygotes, CXN |
NHS |
X-linked recessive |
Cataracts, Coppock-Like |
CCL, Coppock-like cataract |
CRYBB2, CRYGC, GJA3 |
autosomal dominant |
Cataracts, CRYAA Mutations |
autosomal dominant cataract with microcornea, autosomal dominant nuclear cataract, autosomal dominant nuclear with iris coloboma, autosomal recessive congenital cataract 1, CATC1, CRYAA, crystallin alpha-1, zonular central nuclear cataract |
CRYAA |
autosomal dominant, autosomal recessive |
Cataracts, Growth Hormone Deficiency, and Skeletal Dysplasia |
CAGSSS |
IARS2 |
autosomal recessive |
Cataracts, Hearing Loss, and Neurodegeneration |
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SLC33A1 |
autosomal recessive |
Cataracts, Lamellar |
CAM, CTM, Marner cataract, perinuclear cataract, zonular cataract |
HSF4 |
autosomal dominant |
Cerebellar Atrophy, Visual Impairment, and Psychomotor Retardation |
CAVIPMR |
EMC1 |
autosomal recessive |
Cerebral Amyloid Angiopathy |
familial Danish dementia, FDD |
ITM2B |
autosomal dominant |
Cerebral Atrophy, Autosomal Recessive |
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TMPRSS4 |
autosomal recessive |
Cerebral Cavernous Malformations |
CAM, cavernous angiomatous malformations, CCM, cerebral capillary malformations, familial cavernous angioma |
CCM2/malcavernin, KRIT1, PDCD10 |
autosomal dominant |
Cerebral Palsy, Spastic Quadriplegic, 3 |
CPSQ3 |
ADD3 |
autosomal recessive |
Cerebrooculofacioskeletal Syndrome |
COFS syndrome, COFS1, CS II, optic atrophy, Pena-Shokeir syndrome, type II Cockayne syndrome |
ERCC6 |
autosomal recessive |
Cerebrotendinous Xanthomatosis |
cerebral cholesterinosis, CTX |
CYP27A1 |
autosomal recessive |
Charcot-Marie-Tooth Disease with Glaucoma |
Charcot-Marie-Tooth disease type 4B2 with early-onset glaucoma, CMT4B2 |
SBF2 |
autosomal recessive |
Charcot-Marie-Tooth Disease(s) |
CMT |
multiple |
autosomal dominant, autosomal recessive, X-linked dominant, X-linked recessive |
CHARGE Syndrome |
CHARGE association, Hall-Hittner syndrome, HHS |
CHD7, SEMA3E |
autosomal dominant |
Chédiak-Higashi Syndrome |
CHS |
LYST |
autosomal recessive |
Chondrodysplasia Punctata 2 |
CDPX2, CDPXD, Conradi-Hünermann syndrome, CPXD, Happle syndrome |
EBP |
X-linked dominant |
CHOPS Syndrome |
CHOPS, cognitive impairment coarse facies heart defects obesity airway anomalies short stature and skeletal dysplasia syndrome |
AFF4 |
autosomal dominant |
Chorioretinal dysplasia, lymphedema, and microcephaly |
lymphedema and retinal folds with microcephaly and microphthalmos, microcephaly with lymphedema and chorioretinal dysplasia syndrome |
KIF11 |
autosomal dominant |
Chorioretinal dysplasia, microcephaly, and mental retardation |
chorioretinal dysplasia-microcephaly-mental retardation syndrome |
? |
autosomal dominant |
Chorioretinopathy with Microcephaly 1 |
chorioretinal dysplasia-microcephaly-mental retardation syndrome, MCCRP1, TUBGCP6 |
TUBGCP6 |
autosomal recessive |
Chorioretinopathy with Microcephaly 2 |
MCCRP2 |
PLK4 |
autosomal recessive |
Chorioretinopathy with Microcephaly 3 |
MCCRP3 |
TUBGCP4 |
autosomal recessive |
Chorioretinopathy, Ataxia, and Hypogonadism |
BNHS, Boucher-Neuhauser syndrome |
PNPLA6 |
autosomal recessive? |
Choroidal Dystrophy, Central Areolar 1 |
CACD, CACD1, choroidal macular dystrophy, choroidal sclerosis |
GUCY2D |
autosomal dominant |
Choroidal Dystrophy, Central Areolar 2 |
CACD2, progressive macular dystrophy |
PRPH2 |
autosomal dominant |
Choroideremia |
choroidal sclerosis, tapetochoroidal dystrophy, TCD |
CHM |
X-linked recessive |
Cleft Palate, Psychomotor Retardation, and Distinctive Facial Features |
CPRF |
KDM1A |
autosomal dominant |
Coats Plus Syndrome |
cerebroretinal microangiopathy with calcifications and cysts, CRMCC |
CTC1 |
autosomal recessive |
Cockayne Syndrome, Type A |
CKN1, CSI |
ERCC8 |
autosomal recessive |
Cockayne Syndrome, Type B |
CKN2, CSII |
ERCC6 |
autosomal recessive |
CODAS Syndrome |
cerebral ocular dental auricular skeletal anomalies syndrome |
LONP1 |
autosomal recessive |
Cohen Syndrome |
COH1, Pepper syndrome |
COH1, VPS13B |
autosomal recessive |
Cole-Carpenter Syndrome 1 |
bone fragility with craniosynostosis ocular proptosis hydrocephalus and distinctive facial features, CLCRP1 |
P4HB |
autosomal dominant |
Cole-Carpenter Syndrome 2 |
CLCRP2 |
SEC24D |
autosomal recessive |
Coloboma of the Optic Nerve |
morning glory disc, optic nerve pits |
PAX6 |
autosomal dominant |
Coloboma, Isolated |
ocular coloboma, uveal coloboma, uveoretinal coloboma |
ABCB6, PAX6, SHH |
autosomal dominant |
Coloboma, Microphthalmia, Albinism, and Deafness |
COMMAD |
MITF |
autosomal recessive? |
Coloboma, Ptosis, Hypertelorism, and Global Delay |
Baraitser-Winter syndrome-1 |
ACTG1 |
autosomal dominant? |
Color Blindness, Red-Green, Partial |
DCB, deutan color blindness, deuteranomaly, deuteranopia, green color blindness, protanomaly, protanopia, red color blindness |
OPN1LW, OPN1MW |
X-linked recessive |
Colorblindness-Achromatopsia 2 |
ACHM2, RMCH2, rod monochromatism |
CNGA3 |
autosomal recessive |
Colorblindness-Achromatopsia 3 |
ACHM3, achromatopsia with myopia, Pingelapese blindness, rod monochromatism |
CNGB3 |
autosomal recessive |
Colorblindness-Achromatopsia 4 |
ACHM4, achromatopsia 4 |
GNAT2 |
autosomal recessive |
Colorblindness-Achromatopsia 5 |
ACHM5, COD4, cone dystrophy 4 |
PDE6C |
autosomal recessive |
Colorblindness-Tritanopia |
blue colorblindness, CBT, tritan colorblindness, tritanopic colorblindness |
OPN1SW |
autosomal dominant |
Combined Oxidative Phosphorylation Deficiency 32 |
COXPD32 |
MRPS34 |
autosomal recessive |
Cone Dystrophy 3 |
COD3, cone-rod dystrophy 14, CORD14, retinal cone dystrophy |
GUCA1A (GCAP1) |
autosomal dominant |
Cone Dystrophy, Peripheral |
|
? |
autosomal recessive? |
Cone-Rod Dystrophies, AD and AR |
Cone-rod dystrophy, CORD, CRD |
|
autosomal dominant, autosomal recessive |
Cone-Rod Dystrophies, X-Linked |
COD1, CORDX1, CORDX2, CORDX3 |
CACNA1F, RPGR |
X-linked dominant, X-linked recessive |
Cone-Rod Dystrophy With Decreased Male Fertility |
CORD19 |
TTLL5 |
|
Cone-Rod Dystrophy with Hearing Loss |
CRDHL |
CEPL78 |
autosomal recessive |
Congenital Disorder of Glycosylation, Type Ia |
CDG Ia, CDGIa, Jaeken syndrome, phosphomannomutase 2 deficiency |
PMM2 |
autosomal recessive |
Congenital Disorder of Glycosylation, Type Ij |
CDG-1j, CDG-Ij, DPAGT1-CDG (CDG-lj) |
DPAGT1 |
autosomal recessive |
Congenital Disorder of Glycosylation, Type Iq |
carbohydrate-deficient glycoprotein syndrome, CDG syndrome, CDG1Q, CDGIq |
SRD5A3 |
autosomal recessive |
Congenital Heart Defects, Dysmorphic Facies, and Intellectual Developmental Disorder |
CHDFIDD |
CDK13 |
autosomal dominant |
Conjunctivitis, Ligneous |
dysplasminogenemia, plasminogen deficiency type I |
PLG |
autosomal recessive |
Cornea Plana |
CNA 1, CNA 2 |
KERA |
autosomal dominant, autosomal recessive |
Cornea, Ring Dermoid |
RDC, ring dermoid of cornea |
PITX2 |
autosomal dominant |
Corneal Dystrophy, Avellino Type |
ACD, Avellino corneal dystrophy, CDA, combined granular-lattice corneal dystrophy, GCD2, granular corneal dystrophy |
TGFBI |
autosomal dominant |
Corneal Dystrophy, Band-Shaped |
band keratopathy |
? |
autosomal dominant?, autosomal recessive |
Corneal Dystrophy, Congenital Endothelial 1 |
CHED1, congenital hereditary endothelial dystrophy, Maumenee corneal dystrophy |
20p11.2-q11.2 locus |
autosomal dominant |
Corneal Dystrophy, Congenital Endothelial 2 |
CHED2, congenital hereditary endothelial dystrophy of cornea, Maumenee corneal dystrophy |
SLC4A11 |
autosomal recessive |
Corneal Dystrophy, Congenital Stromal |
congenital hereditary stromal dystrophy, congenital stromal corneal dystrophy, CSCD, Witschel dystrophy |
DCN |
autosomal dominant |
Corneal Dystrophy, Endothelial X-Linked |
X-linked endothelial corneal dystrophy, XECD |
Xq25 locus |
X-linked dominant, X-linked recessive |
Corneal Dystrophy, Epithelial Basement Membrane |
anterior basement membrane dystrophy, Cogan corneal dystrophy, EBMD, map-dot-fingerprint corneal dystrophy, microcystic corneal dystrophy |
TGFBI |
autosomal dominant |
Corneal Dystrophy, Fleck |
fleck corneal dystrophy, Francois-Neetens fleck dystrophy |
(PIP5K3), PIKFYVE |
autosomal dominant |
Corneal Dystrophy, Fuchs Endothelial, Early Onset |
early onset endothelial corneal dystrophy, FECD1 |
COL8A2 |
autosomal dominant |
Corneal Dystrophy, Fuchs Endothelial, Late Onset |
FCD1, FECD2, late onset corneal endothelial dystrophy |
ZEB1 |
autosomal dominant |
Corneal Dystrophy, Fuchs Endothelial, Late Onset 2 |
FCD2 locus, FECD3 |
TCF4 |
autosomal dominant |
Corneal Dystrophy, Gelatinous Drop-like |
amyloid corneal dystrophy, CDGDL, corneal amyloidosis, GDLD, lattice corneal dystrophy type III |
M1S1 (TACSTD2) |
autosomal recessive |
Corneal Dystrophy, Granular |
CDGG1, GCD1, granular corneal dystrophy type I, Groenouw type I, punctate or nodular corneal dystrophy |
TGFBI |
autosomal dominant |
Corneal Dystrophy, Lattice Type I |
Biber-Haab-Dimmer dystrophy, CDL1, lattice corneal dystrophy, LCD, LCD1 |
TGFBI |
autosomal dominant |
Corneal Dystrophy, Lattice Type II |
amyloidosis V, Finnish type amyloidosis, gelsolin amyloidosis, GSN lattice dystrophy, Meretoja type lattice corneal dystrophy |
GSN |
autosomal dominant |
Corneal Dystrophy, Lisch Epithelial |
LECD, Lisch epithelial corneal dystrophy, whorled microcystic dystrophy |
None identified |
X-linked dominant |
Corneal Dystrophy, Macular |
Fehr corneal dystrophy, Groenouw type II corneal dystrophy, MCDC1 |
CHST6 |
autosomal recessive |
Corneal Dystrophy, Meesmann |
juvenile hereditary corneal dystrophy, MECD, Meesmann corneal dystrophy |
KRT12, KRT3 |
autosomal dominant |
Corneal Dystrophy, Posterior Amorphous |
PACD, posterior amorphous corneal dystrophy, posterior amorphous stromal dystrophy |
12q21.33 deletion |
autosomal dominant |
Corneal Dystrophy, Posterior Polymorphous 1 |
Maumenee corneal dystrophy, posterior polymorphous corneal dystrophy 1, PPCD1 |
OVOL2 |
autosomal dominant |
Corneal Dystrophy, Posterior Polymorphous 2 |
PPCD2 |
COL8A2 |
autosomal dominant |
Corneal Dystrophy, Posterior Polymorphous 3 |
PPCD3 |
ZEB1 |
autosomal dominant |
Corneal Dystrophy, Posterior Polymorphous 4 |
PPCD4 |
GRHL2 |
autosomal dominant |
Corneal Dystrophy, Recurrent Epithelial Erosions |
epithelial recurrent erosion dystrophy, ERED, Franceschetti hereditary recurrent corneal erosion, hereditary corneal erosions, recurring corneal erosions |
? |
autosomal dominant |
Corneal Dystrophy, Reis-Bücklers |
CDB type I, corneal dystrophy of Bowman layer type I, GCD type III, geographic corneal dystrophy, RBCD, Reis-Bücklers corneal dystrophy, superficial granular corneal dystrophy |
TGFBI |
autosomal dominant |
Corneal Dystrophy, Schnyder |
crystalline stromal dystrophy, SCD, Schnyder corneal dystrophy, Schnyder crystalline corneal dystrophy |
UBIAD1 |
autosomal dominant |
Corneal Dystrophy, Stocker-Holt |
juvenile hereditary epithelial dystrophy |
KRT12 |
autosomal dominant |
Corneal Dystrophy, Subepithelial Mucinous |
SMCD, subepithelial mucinous corneal dystrophy |
unknown |
autosomal dominant |
Corneal Dystrophy, Thiel-Behnke |
CDB type II, CDB2, CDTB, corneal dystrophy of Bowman layer type II, honeycomb corneal dystrophy, TBCD, Thiel-Behnke corneal dystrophy |
TGFBI |
autosomal dominant |
Cornelia de Lange Syndrome |
BDLS, Brachmann-de Lange syndrome, CDL, CDLS1, de Lange syndrome |
HDAC8, NIPBL, RAD21, SMC1A, SMC3 |
autosomal dominant, autosomal recessive, X-linked |
Corpus Callosum Agenesis with Facial Anomalies and Cerebellar Ataxia |
Birk-Flusser syndrome, CCAFCA |
FRMD4A |
autosomal recessive |
Cranial Dysinnervation Disorders with Strabismus and Arthrogryposis |
DA5, DA5D, distal arthrogryposis type 5D, oculomelic amyoplasia |
ECEL1, PIEZO2 |
autosomal dominant, autosomal recessive |
Craniofacial-Deafness-Hand Syndrome |
CDHS |
PAX3 |
autosomal dominant |
Crouzon Syndrome |
CFD1, craniofacial dysostosis type I, Crouzon craniofacial dysostosis |
FGFR2 |
autosomal dominant |
Cryptophthalmos |
simple ankyloblepharon |
|
autosomal dominant |
Cystinosis |
cystinosin defect, deficiency of lysosomal cystine transport protein |
CTNS |
autosomal recessive |