نتایج جستجو برای: hypophosphatasia

تعداد نتایج: 591  

Journal: :Annals of the Rheumatic Diseases 2023

Background Hypophosphatasia (HPP) is a rare disease caused by mutations of the ALPL gene leading to reduced activity tissue non-specific alkaline phosphatase (AP). HPP can mimic other diseases clinically and be overseen, misdiagnosed or in worst-case inadequately treated. Objectives To identify patients with high likelihood HPP, explore frequency our rheumatologic outpatient clinic summarize cl...

2015
Álvaro Sebastián-Serrano Laura de Diego-García Carlos Martínez-Frailes Jesús Ávila Herbert Zimmermann José Luis Millán María Teresa Miras-Portugal Miguel Díaz-Hernández

Tissue-nonspecific alkaline phosphatase (TNAP) is one of the four isozymes in humans and mice that have the capacity to hydrolyze phosphate groups from a wide spectrum of physiological substrates. Among these, TNAP degrades substrates implicated in neurotransmission. Transgenic mice lacking TNAP activity display the characteristic skeletal and dental phenotype of infantile hypophosphatasia, as ...

Journal: :Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research 2014
Manisha C Yadav Carmen Huesa Sonoko Narisawa Marc F Hoylaerts Alain Moreau Colin Farquharson José Luis Millán

PHOSPHO1 and tissue-nonspecific alkaline phosphatase (TNAP) have nonredundant functions during skeletal mineralization. Although TNAP deficiency (Alpl(-/-) mice) leads to hypophosphatasia, caused by accumulation of the mineralization inhibitor inorganic pyrophosphate (PPi ), comparably elevated levels of PPi in Phospho1(-/-) mice do not explain their stunted growth, spontaneous fractures, bowed...

Journal: :Clinical Cases in Mineral and Bone Metabolism 2017

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