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Angelman syndrome due to imprinting defect in 15q11-q13

ORPHA:411515Etiological subtypeNot applicableInfancy

Ассоциированные гены (3)

SNRPN
small nuclear ribonucleoprotein polypeptide N
Role in the phenotype of
OMIM: 182279
UBE3A
ubiquitin protein ligase E3A
Role in the phenotype of
OMIM: 601623
ATP10A
ATPase phospholipid transporting 10A (putative)
Role in the phenotype of
OMIM: 605855

Фенотипы (27)

Частый (30–79%)13
HP:0000748Inappropriate laughter
HP:0001010Hypopigmentation of the skin
HP:0001250Seizure
HP:0001513Obesity
HP:0001999Abnormal facial shape
HP:0002353EEG abnormality
HP:0002591Polyphagia
HP:0005599Hypopigmentation of hair
HP:0007730Iris hypopigmentation
HP:0008872Feeding difficulties in infancy
HP:0008947Floppy infant
HP:0031936Delayed ability to walk
HP:0040082Happy demeanor
Периодический (5–29%)14
HP:0000154Wide mouth
HP:0000303Mandibular prognathia
HP:0000708Atypical behavior
HP:0000710Hyperorality
HP:0000736Short attention span
HP:0000752Hyperactivity
HP:0001251Ataxia
HP:0001344Absent speech
HP:0002136Broad-based gait
HP:0002307Drooling
HP:0002465Poor speech
HP:0004485Cessation of head growth
HP:0006979Sleep-wake cycle disturbance
HP:0410263Brain imaging abnormality

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы