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Prader-Willi syndrome due to imprinting mutation

ORPHA:177910Etiological subtypeNot applicableAntenatal, Neonatal

Ассоциированные гены (5)

SNORD115@
small nucleolar RNA, C/D box 115 cluster
Role in the phenotype of
OMIM: 609837
SNRPN
small nuclear ribonucleoprotein polypeptide N
Role in the phenotype of
OMIM: 182279
MAGEL2
MAGE family member L2
Role in the phenotype of
OMIM: 605283
NDN
necdin, MAGE family member
Role in the phenotype of
OMIM: 602117
SNORD116@
small nucleolar RNA, C/D box 116 cluster
Role in the phenotype of
OMIM: 605436

Фенотипы (18)

Частый (30–79%)11
HP:0000708Atypical behavior
HP:0001263Global developmental delay
HP:0001319Neonatal hypotonia
HP:0001513Obesity
HP:0001558Decreased fetal movement
HP:0001773Short foot
HP:0001999Abnormal facial shape
HP:0002591Polyphagia
HP:0004322Short stature
HP:0008872Feeding difficulties in infancy
HP:0200055Small hand
Периодический (5–29%)2
HP:0004039Abnormality of ulnar metaphysis
HP:0004283Narrow palm
Очень редкий (1–4%)5
HP:0000044Hypogonadotropic hypogonadism
HP:0001010Hypopigmentation of the skin
HP:0003241External genital hypoplasia
HP:0005599Hypopigmentation of hair
HP:0007730Iris hypopigmentation

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы