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Spondyloepimetaphyseal Dysplasia With Joint Laxity Type 1

Disease ID: disease_node_15666

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DbxrefMIM:271640
SubclassofDOID_0050737, DOID_0112197
Data SourceDOID
SynonymsSEMDJL1, spondyloepimetaphyseal dysplasia with joint laxity, type 1, with or without fractures
Doid Labelspondyloepimetaphyseal dysplasia with joint laxity type 1
Doid DescriptionA spondyloepimetaphyseal dysplasia with joint laxity characterized by vertebral abnormalities and ligamentous laxity that result in spinal misalignment and progressive severe kyphoscoliosis, thoracic asymmetry, and respiratory compromise that has_material_basis_in homozygous or compound heterozygous mutation in the B3GALT6 gene on chromosome 1p36.33.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_15666
Doid IdDOID_0112198
LabelSpondyloepimetaphyseal Dysplasia With Joint Laxity Type 1