GenTIGSA Gene Database on Rare Genetic Disorders

Select a RGD Category for details 

RGDs registry under the 'Multisystemic disorders'
Disorder Name (Total=27) Aliases Sub-Category Gene Indian Reports Link
Hypoparathyroidism, deafness, renal disease syndrome
Autosomal dominant
•Barakat syndrome
•Hypoparathyroidism-sensorineural deafness-renal disease syndrome
GATA3 GATA binding protein 3
Reports
Updated as of Mar 09, 2023
PubMed
Alstrom syndrome
Autosomal recessive
•Alström Syndrome
•Alstrom's syndrome
ALMS1 ALMS1 centrosome and basal body associated protein
Reports
Updated as of Nov 30, 2023
PubMed
Mosaic variegated aneuploidy syndrome 2
Autosomal recessive
•Mosaic variegated aneuploidy syndrome
CEP57 centrosomal protein 57
Reports
Updated as of Feb 01, 2024
PubMed
Aicardi-Goutieres syndrome 6
Autosomal recessive
•Aicardi-Goutières syndrome
•Encephalopathy with basal ganglia calcification
•Encephalopathy with intracranial calcification and chronic lymphocytosis of cerebrospinal fluid
•Pseudo-Torch syndrome
•Aicardi-Toutieres syndrome
•Type 6Aicardi-Goutieres syndrome 1
ADAR adenosine deaminase RNA specific
Reports
Updated as of Jul 20, 2024
PubMed
Aicardi-Goutieres syndrome 4
Autosomal recessive
•Aicardi-Goutières syndrome
•Encephalopathy with basal ganglia calcification
•Encephalopathy with intracranial calcification and chronic lymphocytosis of cerebrospinal fluid
•Pseudo-Torch syndrome
•Aicardi-Toutieres syndrome
RNASEH2A ribonuclease H2 subunit A
Reports
Updated as of Jul 20, 2024
PubMed
Wolcott-Rallison dysplasia
Autosomal recessive
•Multiple epiphyseal dysplasia with early-onset diabetes mellitus
•Wolcott-rallison syndrome
•MED-IDDM syndrome
•Wolcott Rallison syndrome
EIF2AK3 eukaryotic translation initiation factor 2 alpha kinase 3
Reports
Updated as of Feb 01, 2025
PubMed
Hereditary angioedema type 1
Autosomal dominant, Autosomal recessive
•Deficiency of C1 esterase inhibitor
Dermatitis SERPING1 serpin family G member 1
Reports
Updated as of Feb 01, 2025
PubMed
Costello syndrome
Autosomal dominant
•Faciocutaneoskeletal syndrome
•Fcs syndrome
•congenital myopathy with excess of muscle spindles
HRAS HRas proto-oncogene, GTPase
Reports
Updated as of Feb 01, 2025
PubMed
Zellweger spectrum disorders
•Congenital iron overload
•cerebrohepatorenal syndrome
•Zellweger syndrome
•Zellweger Spectrum
•ZS
•ZWS
PEX1 peroxisomal biogenesis factor 1
PEX6 peroxisomal biogenesis factor 6
PEX19 peroxisomal biogenesis factor 19
Reports
Updated as of Feb 01, 2025
PubMed
Autosomal recessive multiple pterygium syndrome
Autosomal recessive
•Autosomal recessive multiple pterygium syndrome
•Escobar syndrome
•Pterygium Colli syndrome
•autosomal recessive non-lethal multiple pterygium syndrome
•Escobar variant multiple pterygium syndrome
•Multiple pterygium syndrome Escobar type
•Multiple pterygium syndrome nonlethal type
CHRNG cholinergic receptor nicotinic gamma subunit
CHRNA1 cholinergic receptor nicotinic alpha 1 subunit
Reports
Updated as of Feb 01, 2025
PubMed
Lethal multiple pterygium syndrome
Autosomal recessive
CHRND cholinergic receptor nicotinic delta subunit
CHRNA1 cholinergic receptor nicotinic alpha 1 subunit
CHRNG cholinergic receptor nicotinic gamma subunit
Reports
Updated as of Feb 01, 2025
PubMed
Jervell and Lange-Nielsen syndrome
Autosomal recessive
•Jervell-Lange Nielsen syndrome
•Jervell and Lange Nielsen syndrome
•long QT interval-deafness syndrome
KCNQ1 potassium voltage-gated channel subfamily Q member 1
KCNE1 potassium voltage-gated channel subfamily E regulatory subunit 1
Reports
Updated as of Feb 01, 2025
PubMed
Cerebellar ataxia-hypogonadism syndrome
Autosomal recessive
•Gordon-Holmes syndrome
•luteinizing hormone-releasing hormone deficiency with ataxia
•Cerebellar ataxia hypogonadotropic hypogonadism
•Gordon Holmes syndrome
•Lhrh deficiency and ataxia
•Luteinizing hormone releasing hormone, deficiency of with ataxia
RNF216 ring finger protein 216
Reports
Updated as of Feb 01, 2025
PubMed
Rubinstein-Taybi syndrome due to CREBBP mutations
Autosomal dominant
•Broad thumbs and great toes, characteristic facies, and mental retardation
•Rubinstein syndrome
•Rubinstein-Taybi syndrome 1, incomplete
•Rubinstein-Taybi syndrome 1
CREBBP CREB binding protein
EP300 E1A binding protein p300
Reports
Updated as of Feb 26, 2025
PubMed
Popliteal pterygium syndrome
Autosomal dominant
•Autosomal dominant popliteal pterygium syndrome
•popliteal pterygium syndrome 1
•popliteal pterygium syndrome, autosomal dominant
•popliteal web syndrome
IRF6 interferon regulatory factor 6
Reports
Updated as of Feb 26, 2025
PubMed
Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A1
Autosomal recessive
•Cerebroocular dysgenesis
•Chemke syndrome
•COD-MD syndrome
•Cerebroocular dysplasia muscular dystrophy syndrome
•Hard +/- E syndrome
•Hydrocephalus, agyria and retinal dysplasia
•Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A, 1
•Pagon syndrome
•WALKER-WARBURG SYNDROME OR MUSCLE-EYE-BRAIN DISEASE, POMT1-RELATED
•Warburg syndrome
POMT1 protein O-mannosyltransferase 1
Reports
Updated as of Feb 26, 2025
PubMed
Cerebrooculofacioskeletal syndrome 2
Autosomal recessive
ERCC2 ERCC excision repair 2, TFIIH core complex helicase subunit
Reports
Updated as of Feb 26, 2025
PubMed
8q24.3 microdeletion syndrome
Autosomal dominant
•Verheij syndrome
•Chromosome 8q24.3 deletion syndrome
PUF60 poly(U) binding splicing factor 60
Reports
Updated as of Sep 08, 2025
PubMed
Gorlin syndrome
Autosomal dominant
•Nevoid basal cell carcinoma syndrome
•Basal cell nevus syndrome
•Gorlin syndrome
•Gorlin-Goltz syndrome
•Multiple basal cell carcinomas
•NBCCS
•Nevoid basal cell cancer syndrome
PTCH1 patched 1
PTCH2 patched 2
SUFU SUFU negative regulator of hedgehog signaling
Reports
Updated as of Jun 12, 2025
PubMed
Zimmermann-Laband syndrome
Autosomal dominant
•Gingival fibromatosis-hepatosplenomegaly-other anomalies syndrome
•Laband syndrome
•Zimmerman Laband syndrome
•Zimmermann-Laband syndrome type 1
•ZLS
KCNH1 potassium voltage-gated channel subfamily H member 1
ATP6V1B2 ATPase H+ transporting V1 subunit B2
KCNN3 potassium calcium-activated channel subfamily N member 3
Reports
Updated as of Nov 12, 2025
PubMed
X-linked lissencephaly with abnormal genitalia
X-linked recessive
•X-linked Lissencephaly with agenesis of corpus callosum and genital anomaly syndrome
•Lissencephaly, x-linked, type 2
•X-Linked Lissencephaly with ambiguous genitalia
•x-linked lissencephaly with abnormal genitalia syndrome
•x-linked lissencephaly with ambiguous genitalia
•x-linked lissencephaly-agenesis of the corpus callosum-genital anomalies syndrome
•x-linked lissencephaly-corpus callosum agenesis-genital anomalies syndrome
•xlag (x-linked lissencephaly with abnormal genitalia) syndrome
ARX aristaless related homeobox
Reports
Updated as of Nov 12, 2025
PubMed
Dyggve-Melchior-Clausen syndrome
Autosomal recessive
•DMC syndrome
•Dyggve-Melchior-Clausen disease
•Dyggve Melchior Clausen syndrome
•Dyggve-Melchior-Clausen dysplasia
•Pseudo-Morquio disease type i
•DMC disease
DYM dymeclin
Reports
Updated as of Nov 12, 2025
PubMed
X-linked intellectual disability, van Esch type
X-linked recessive
•Van Esch-O'Driscoll syndrome
•Mental retardation, x-linked, syndromic, Van Esch-O''Driscoll type
•Van Esch-O'Driscoll syndrome, x-linked recessive
•VEODS
•x-linked intellectual disability van esch type
POLA1 DNA polymerase alpha 1, catalytic subunit
Reports
Updated as of Nov 12, 2025
PubMed
Incontinentia pigmenti syndrome
X-linked dominant
•Bloch-Sulzberger syndrome
•Bloch-Siemens syndrome
•Incontinentia Pigmenti
•Incontinentia pigmenti of Bloch-Sulzberger
•Incontinentia pigmenti syndrome
•Incontinentia pigmenti, x-linked dominant
•Incontinentia pigmenti, familial male-lethal type
•Incontinentia pigmenti, type ii
•IP - Incontinentia Pigmenti
IKBKG inhibitor of nuclear factor kappa B kinase regulatory subunit gamma
Reports
Updated as of Nov 12, 2025
PubMed
Alpha thalassemia-X-linked intellectual disability syndrome
X-linked dominant
•XLMR hypotonic face syndrome
•ATR-X syndrome
•Alpha thalassemia x-linked intellectual deficit
•Alpha thalassemia x-linked intellectual disability syndrome
•Alpha thalassemia/intellectual disability syndrome x-linked
•Alpha-thalassemia x-linked intellectual disability syndrome
•Alpha-thalassemia/impaired intellectual development syndrome, x-linked
•Alpha-thalassemia/intellectual disability syndrome nondeletion type
•Alpha-thalassemia/mental retardation syndrome, x-linked dominant
•x-linked alpha-thalassemia-mental retardation syndrome
•ATR, nondeletion type
•Alpha thalassemia mental retardation syndrome, nondeletion type, X-linked
•Alpha-Thalassemia X-Linked Intellectual Disability Syndrome
•X-linked alpha-thalassemia-mental retardation syndrome
ATRX ATRX chromatin remodeler
GBA1 glucosylceramidase beta 1
Reports
Updated as of Nov 12, 2025
PubMed
Simpson-Golabi-Behmel syndrome type 1
X-linked recessive
•Bulldog syndrome
•Simpson-Golabi-Behmel syndrome
•Dysplasia gigantism syndrome, x-linked
•Golabi-Rosen syndrome
•GPC3-related Simpson-Golabi-Behmel syndrome type 1
•Simpson dysmorphia syndrome
•Sara Angers syndrome
•x-linked dysplasia gigantism syndrome
•sgb syndrome
•dgsx
•sdys
•sgbs1
GPC3 glypican 3
Reports
Updated as of Nov 12, 2025
PubMed
Holt-Oram syndrome
Autosomal dominant
•Cardiac-limb syndrome
•Heart-hand syndrome, type 1
•TBX5-Related Holt-Oram Syndrome
•Ventriculo-radial syndrome
•Atriodigital dysplasia type 1
•Holt Oram syndrome
•HOS
TBX5 T-box transcription factor 5
Reports
Updated as of Nov 12, 2025
PubMed