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Autosomal Recessive Distal Hereditary Motor Neuronopathy 1

Disease ID: disease_node_18016

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DbxrefICD10CM:G12.2, MIM:604320, ORDO:98920
SubclassofDOID_0111197
Data SourceDOID
SynonymsDSMA1, SIANRF, SMARD1, autosomal recessive distal spinal muscular atrophy 1, autosomal recessive spinal muscular atrophy with respiratory distress, dHMN6, diaphragmatic spinal muscular atrophy, distal hereditary motor neuropathy type 6, distal spinal muscular atrophy 1, distal-HMN type 6, severe infantile axonal neuropathy with respiratory failure type 1, spinal muscular atrophy with respiratory distress type 1
Doid Labelautosomal recessive distal hereditary motor neuronopathy 1
Doid DescriptionA spinal muscular atrophy characterized by autosomal recessive inheritance of severe respiratory distress resulting from diaphragmatic paralysis that predomitly involves the upper limbs and distal muscles that has_material_basis_in homozygous or compound heterozygous mutation in the IGHMBP2 gene on chromosome 11q13.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_18016
Doid IdDOID_0111064
LabelAutosomal Recessive Distal Hereditary Motor Neuronopathy 1