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Ataxia-oculomotor apraxia type 4

ORPHA:459033DiseaseAutosomal recessiveChildhood, Infancy

Ассоциированные гены (1)

PNKP
polynucleotide kinase 3'-phosphatase
Disease-causing germline mutation(s) in
OMIM: 605610

Фенотипы (21)

Облигатный (100%)1
HP:0001251Ataxia
Частый (30–79%)5
HP:0100543Cognitive impairment
HP:0001513Obesity
HP:0000657Oculomotor apraxia
HP:0009830Peripheral neuropathy
HP:0001332Dystonia
Периодический (5–29%)14
HP:0003474Somatic sensory dysfunction
HP:0003560Muscular dystrophy
HP:0001761Pes cavus
HP:0009053Distal lower limb muscle weakness
HP:0002442Dyscalculia
HP:0010522Dyslexia
HP:0000736Short attention span
HP:0007141Sensorimotor neuropathy
HP:0002751Kyphoscoliosis
HP:0000570Abnormal saccadic eye movements
HP:0001260Dysarthria
HP:0002172Postural instability
HP:0001780Abnormality of toe
HP:0008955Progressive distal muscular atrophy
Исключён (0%)1
HP:0001009Telangiectasia

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