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Dentatorubral pallidoluysian atrophy
1 OMIM reference -
1 associated gene
56 connected diseases
No signs/symptoms info
Disease Type of connection
Autosomal recessive cutis laxa type 1
Syndromic multisystem autoimmune disease due to Itch deficiency
Acute myeloid leukemia with t(8;21)(q22;q22) translocation
Attenuated Chédiak-Higashi syndrome
Chédiak-Higashi syndrome
Pulverulent cataract
46,XY partial gonadal dysgenesis
ALG13-CDG
Acute megakaryoblastic leukemia without Down syndrome
Acute myeloid leukemia with t(8;16)(p11;p13) translocation
Alternating hemiplegia of childhood
Autosomal dominant cutis laxa
Autosomal recessive cerebellar ataxia - epilepsy - intellectual deficit
Autosomal recessive primary microcephaly
Behavioral variant of frontotemporal dementia
Blepharophimosis-intellectual deficit syndrome, SBBYS type
CLN11 disease
Carpenter syndrome
Cerebellar ataxia - areflexia - pes cavus - optic atrophy - sensorineural hearing loss
Combined pituitary hormone deficiencies, genetic forms
Cutis laxa with severe pulmonary, gastrointestinal and urinary anomalies
Desmoplastic small round cell tumor
Donnai-Barrow syndrome
Duchenne muscular dystrophy
Dyssegmental dysplasia, Silverman-Handmaker type
Ewing sarcoma
Extraskeletal Ewing sarcoma
Extraskeletal myxoid chondrosarcoma
Familial drusen
Genitopatellar syndrome
Hereditary sensorimotor neuropathy with hyperelastic skin
Hypothyroidism due to deficient transcription factors involved in pituitary development or function
Lethal arteriopathy syndrome due to FBLN4 deficiency
Lipoid proteinosis
Melanoma of soft part
Monosomy 5p
Noonan syndrome
Panhypopituitarism
Postsynaptic congenital myasthenic syndromes
Progressive non-fluent aphasia
Rapid-onset dystonia-parkinsonism
Schwartz-Jampel syndrome
Seckel syndrome
Semantic dementia
Steinert myotonic dystrophy
Synpolydactyly type 2
TARP syndrome
X-linked non-syndromic intellectual deficit
Young adult-onset Parkinsonism
Alpha-thalassemia - X-linked intellectual deficit syndrome
Alpha-thalassemia - myelodysplastic syndrome
Carpenter-Waziri syndrome
Chudley-Lowry-Hoar syndrome
Holmes-Gang syndrome
Juberg-Marsidi syndrome
Smith-Fineman-Myers syndrome
Synonym(s):
- DRPLA
- Dentatorubropallidoluysian atrophy
- Naito-Oyanagi disease

Classification (Orphanet):
- Rare genetic disease
- Rare neurologic disease

Classification (ICD10):
- Diseases of the nervous system -

Epidemiological data:
Class of prevalence: 1-9 / 1 000 000
Average age onset: variable
Average age of death: -
Type of inheritance: autosomal dominant
External references:
1 OMIM reference -
No MeSH references

Gene symbol UniProt reference OMIM reference
ATN1 P54259607462
No signs/symptoms info available.