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Oligodontia

ORPHA:99798Morphological anomalyAutosomal dominant, Autosomal recessive, X-linked recessiveChildhood

Ассоциированные гены (12)

SUMO1
small ubiquitin like modifier 1
Candidate gene tested in
OMIM: 601912
EDA
ectodysplasin A
Disease-causing germline mutation(s) in
OMIM: 300451
EDARADD
EDAR associated via death domain
Disease-causing germline mutation(s) in
OMIM: 606603
FGFR1
fibroblast growth factor receptor 1
Disease-causing germline mutation(s) in
OMIM: 136350
IRF6
interferon regulatory factor 6
Disease-causing germline mutation(s) in
OMIM: 607199
WNT10A
Wnt family member 10A
Disease-causing germline mutation(s) in
OMIM: 606268
MSX1
msh homeobox 1
Disease-causing germline mutation(s) in
OMIM: 142983
PAX9
paired box 9
Disease-causing germline mutation(s) in
OMIM: 167416
TGFA
transforming growth factor alpha
Disease-causing germline mutation(s) in
OMIM: 190170
LRP6
LDL receptor related protein 6
Disease-causing germline mutation(s) (loss of function) in
OMIM: 603507
GREM2
gremlin 2, DAN family BMP antagonist
Disease-causing germline mutation(s) in
OMIM: 608832
WNT10B
Wnt family member 10B
Disease-causing germline mutation(s) in
OMIM: 601906

Фенотипы (23)

Облигатный (100%)1
HP:0000677Oligodontia
Очень частый (80–99%)1
HP:0005216Impaired mastication
Частый (30–79%)10
HP:0000684Delayed eruption of teeth
HP:0000687Widely spaced teeth
HP:0000689Dental malocclusion
HP:0000690Agenesis of maxillary lateral incisor
HP:0000691Microdontia
HP:0000696Delayed eruption of permanent teeth
HP:0006289Agenesis of central incisor
HP:0006336Short dental roots
HP:0011051Agenesis of premolar
HP:0011056Agenesis of first permanent molar tooth
Периодический (5–29%)11
HP:0000202Orofacial cleft
HP:0000679Taurodontia
HP:0000685Hypoplasia of teeth
HP:0006297Enamel hypoplasia
HP:0006342Peg-shaped maxillary lateral incisors
HP:0006344Abnormality of primary molar morphology
HP:0006482Abnormal dental morphology
HP:0011053Agenesis of mandibular premolar
HP:0011078Abnormality of canine
HP:0011219Short face
HP:0012472Eclabion

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы