[Pseudohypoparathyroidism: a generalized and multiple disorder in the adenyl cyclase system].
Pseudohypoparathyroidism (PHP) is caused by non response of target organs (kidney and bone) to parathyroid hormone because of defects in adenylate cyclase-system. The often seen combination of other endocrine abnormalities with PHP speak for a generalized defect of hormonsensitive adenylate cyclases, but not for isolated resistance to parathyroid hormone. At least two defects are known: in Typ I-PHP the defect is before the formation of cyclic AMP (cAMP) because of a reduced activity of receptor-cyclase coupling protein. In Typ II-PHP the defect lies behind cAMP-formation (defect in the reception of cAMP-signal?).
D000262:Adenylyl Cyclases / Q000378:metabolism*; D002648:Child; D000242:Cyclic AMP / Q000378:metabolism; D019204:GTP-Binding Proteins; D006801:Humans; D010281:Parathyroid Hormone / Q000097:blood; D011547:Pseudohypoparathyroidism / Q000201:enzymology*; D011956:Receptors, Cell Surface / Q000378:metabolism; D013963:Thyroid Hormones / Q000097:blood
D010281:Parathyroid Hormone; D011956:Receptors, Cell Surface; D013963:Thyroid Hormones; D000242:Cyclic AMP; D019204:GTP-Binding Proteins; D000262:Adenylyl Cyclases
Pseudohypoparathyreoidismus: eine generalisierte und multiple Störung im hormonsensitiven Adenylzyklasesystem.