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Efzimfotase Alfa Improves Respiratory Capacity in Muscle Tissue From a Mouse Model of HPP

ABSTRACT Hypophosphatasia (HPP) is an inherited metabolic disease caused by deficient tissue‐nonspecific alkaline phosphatase (ALP) activity and characterized by skeletal and nonskeletal symptoms, including muscle weakness and fatigue. We hypothesized that mitochondrial respiration is impaired in mu...

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I tiakina i:
Ngā kaituhi matua: Denise Devore, Juan Ruanova, Walter Voegtli, Derek Dunn, John Decker, Maurizio Mazzantini, Vincenzo De Tata, Maria Concetta Scavuzzo, Francesco Conti, Anna Petryk, Maria Luisa Brandi
Hōputu: Artigo
Reo:Inglês
I whakaputaina: Wiley 2026-01-01
Rangatū:JIMD Reports
Ngā marau:
efzimfotase alfa
enzyme replacement therapy
hypophosphatasia
mitochondrial dysfunction
muscle weakness
ultrastructural studies
Urunga tuihono:https://doi.org/10.1002/jmd2.70057
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