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Pseudopseudohypoparathyroidism

Disease ID: disease_node_6388

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DbxrefGARD:7860, MESH:D011556, MIM:612463, NCI:C129722, SNOMEDCT_US_2023_03_01:190867002, UMLS_CUI:C0033835
SubclassofDOID_4184
Data SourceDOID, MESH
SynonymsNormocalcemic pseudohypoparathyroidism, PPHP
Mesh IdD011556
Mesh LabelPseudopseudohypoparathyroidism
Mesh SubclassofD011547
Doid Labelpseudopseudohypoparathyroidism
Doid DescriptionA pseudohypoparathyroidism characterized by a lack of resistance to parathyroid hormone or other hormones along with the constellation of clinical features referred to as Albright hereditary osteodystrophy and that has_material_basis_in a mutation resulting in loss of function of the Gs-alpha isoform of the GNAS gene on the paternal allele. This results in expression of the Gs-alpha protein only from the maternal allele.
Disease Node Iddisease_node_6388
Doid IdDOID_4183
LabelPseudopseudohypoparathyroidism