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Aniridia-cerebellar ataxia-intellectual disability syndrome

ORPHA:1065Malformation syndromeAutosomal dominant, Autosomal recessive, Not applicableInfancy, Neonatal

Ассоциированные гены (2)

ITPR1
inositol 1,4,5-trisphosphate receptor type 1
Disease-causing germline mutation(s) in
OMIM: 147265
PAX6
paired box 6
Disease-causing germline mutation(s) in
OMIM: 607108

Фенотипы (11)

Очень частый (80–99%)5
HP:0000298Mask-like facies
HP:0000526Aniridia
HP:0001249Intellectual disability
HP:0001251Ataxia
HP:0001263Global developmental delay
Частый (30–79%)4
HP:0001252Hypotonia
HP:0002167Abnormality of speech or vocalization
HP:0002168Scanning speech
HP:0100022Abnormality of movement
Периодический (5–29%)2
HP:0000364Hearing abnormality
HP:0004414Abnormality of the pulmonary artery

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы