PTH resistance

Giovanna Mantovani; Francesca Marta Elli

doi:10.1016/j.mce.2021.111311

PTH resistance

Giovanna Mantovani, Francesca Marta Elli

Fondazione IRCCS Ca' Granda- Ospedale Maggiore Policlinico

Research output: Contribution to journal › Article › peer-review

Abstract

Parathyroid hormone (PTH), which is primarily regulated by extracellular calcium changes, controls calcium and phosphate homeostasis. Different diseases are derived from PTH deficiency (hypoparathyroidism), excess (hyperparathyroidism) and resistance (pseudohypoparathyroidism, PHP). Pseudohypoparathyroidism was historically classified into subtypes according to the presence or not of inherited PTH resistance associated or not with features of Albright's hereditary osteodystrophy and deep and progressive ectopic ossifications. The growing knowledge on the PTH/PTHrP signaling pathway showed that molecular defects affecting different members of this pathway determined distinct, yet clinically related disorders, leading to the proposal of a new nomenclature and classification encompassing all disorders, collectively termed inactivating PTH/PTHrP signaling disorders (iPPSD).

Original language	English
Article number	111311
Journal	Molecular and Cellular Endocrinology
Volume	531
DOIs	https://doi.org/10.1016/j.mce.2021.111311
Publication status	Published - Jul 1 2021

Keywords

Albright's hereditary osteodystrophy
Differential diagnosis
Hormone resistance
Inactivating PTH/PTHrP signaling disorders
Parathyroid hormone
Pseudohypoparathyroidism

ASJC Scopus subject areas

Biochemistry
Molecular Biology
Endocrinology

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10.1016/j.mce.2021.111311

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title = "PTH resistance",

abstract = "Parathyroid hormone (PTH), which is primarily regulated by extracellular calcium changes, controls calcium and phosphate homeostasis. Different diseases are derived from PTH deficiency (hypoparathyroidism), excess (hyperparathyroidism) and resistance (pseudohypoparathyroidism, PHP). Pseudohypoparathyroidism was historically classified into subtypes according to the presence or not of inherited PTH resistance associated or not with features of Albright's hereditary osteodystrophy and deep and progressive ectopic ossifications. The growing knowledge on the PTH/PTHrP signaling pathway showed that molecular defects affecting different members of this pathway determined distinct, yet clinically related disorders, leading to the proposal of a new nomenclature and classification encompassing all disorders, collectively termed inactivating PTH/PTHrP signaling disorders (iPPSD).",

keywords = "Albright's hereditary osteodystrophy, Differential diagnosis, Hormone resistance, Inactivating PTH/PTHrP signaling disorders, Parathyroid hormone, Pseudohypoparathyroidism",

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AU - Elli, Francesca Marta

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KW - Albright's hereditary osteodystrophy

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PTH resistance

Abstract

Keywords

ASJC Scopus subject areas

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