Differences in the predominance of lysosomal and autophagic pathologies between infants and adults with Pompe disease: implications for therapy.
about
Autophagy in lysosomal storage disordersNew insights into therapeutic options for Pompe diseaseAutophagy and mitochondria in Pompe disease: nothing is so new as what has long been forgottenFiber type conversion by PGC-1α activates lysosomal and autophagosomal biogenesis in both unaffected and Pompe skeletal muscle.Pompe disease: from pathophysiology to therapy and back againPompe disease results in a Golgi-based glycosylation deficit in human induced pluripotent stem cell-derived cardiomyocytes.Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease.Quantitative evaluation of skeletal muscle defects in second harmonic generation images.Slow, progressive myopathy in neonatally treated patients with infantile-onset Pompe disease: a muscle magnetic resonance imaging study.Skeletal muscle pathology of infantile Pompe disease during long-term enzyme replacement therapy.The value of muscle biopsies in Pompe disease: identifying lipofuscin inclusions in juvenile- and adult-onset patientsEnzyme replacement therapy for Pompe disease.Infantile Pompe disease on ERT: update on clinical presentation, musculoskeletal management, and exercise considerations.Guidelines for the diagnosis, treatment and clinical monitoring of patients with juvenile and adult Pompe disease.Toward deconstructing the phenotype of late-onset Pompe disease.Improvement of bone mineral density after enzyme replacement therapy in Chinese late-onset Pompe disease patients.36 months observational clinical study of 38 adult Pompe disease patients under alglucosidase alfa enzyme replacement therapy.A Skeletal Muscle Model of Infantile-onset Pompe Disease with Patient-specific iPS CellsHuman Pompe disease-induced pluripotent stem cells for pathogenesis modeling, drug testing and disease marker identification.Muscle fiber-type distribution, fiber-type-specific damage, and the Pompe disease phenotype.Effects of short-to-long term enzyme replacement therapy (ERT) on skeletal muscle tissue in late onset Pompe disease (LOPD).
P2860
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P2860
Differences in the predominance of lysosomal and autophagic pathologies between infants and adults with Pompe disease: implications for therapy.
description
2010 nî lūn-bûn
@nan
2010 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Differences in the predominanc ...... ase: implications for therapy.
@ast
Differences in the predominanc ...... ase: implications for therapy.
@en
Differences in the predominanc ...... ase: implications for therapy.
@nl
type
label
Differences in the predominanc ...... ase: implications for therapy.
@ast
Differences in the predominanc ...... ase: implications for therapy.
@en
Differences in the predominanc ...... ase: implications for therapy.
@nl
prefLabel
Differences in the predominanc ...... ase: implications for therapy.
@ast
Differences in the predominanc ...... ase: implications for therapy.
@en
Differences in the predominanc ...... ase: implications for therapy.
@nl
P2093
P2860
P1476
Differences in the predominanc ...... ase: implications for therapy.
@en
P2093
Cynthia Schreiner
Evelyn Ralston
Kristien J M Zaal
Nina Raben
Paul H Plotz
Rebecca Baum
Yin-Hsiu Chien
P2860
P304
P356
10.1016/J.YMGME.2010.08.001
P577
2010-08-07T00:00:00Z