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Autosomal recessive spastic paraplegia type 44

ORPHA:320401DiseaseAutosomal recessiveAdolescent, Adult

Ассоциированные гены (1)

GJC2
gap junction protein gamma 2
Disease-causing germline mutation(s) in
OMIM: 608803

Фенотипы (16)

Очень частый (80–99%)10
HP:0000486Strabismus
HP:0000649Abnormality of visual evoked potentials
HP:0001251Ataxia
HP:0001260Dysarthria
HP:0001761Pes cavus
HP:0002061Lower limb spasticity
HP:0003429CNS hypomyelination
HP:0006958Abnormal auditory evoked potentials
HP:0007377Abnormality of somatosensory evoked potentials
HP:0012896Abnormal motor evoked potentials
Частый (30–79%)5
HP:0000407Sensorineural hearing impairment
HP:0001250Seizure
HP:0001288Gait disturbance
HP:0002839Urinary bladder sphincter dysfunction
HP:0003474Somatic sensory dysfunction
Периодический (5–29%)1
HP:0002194Delayed gross motor development

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