Results 111 to 120 of about 460,260 (336)

Suppression of lung adenocarcinoma migration through organelle alkalization by human lactoferrin – albumin fusion

FEBS Open Bio, EarlyView.
This paper reveals how human lactoferrin–albumin fusion (hLF‐HSA) potently suppresses lung adenocarcinoma cell migration. hLF‐HSA upregulates NHE7, leading to Golgi alkalization, disruption of the Golgi secretome, downregulation of MMP1, and reversal of EMT. These findings suggest a novel Golgi‐targeting strategy to suppress cancer cell migration.
Hana Nopia, Masahiro Kimura, Daisuke Kurimoto, Atsushi Sato   +3 more
wiley   +1 more source

Enzyme replacement therapy for Pompe disease [PDF]

, 2010
Pompe Disease is a rare genetic lysosomal storage disease resulting from mutations in the gene for acid alpha- glucosidase. Mutations in this gene cause a buildup of glycogen within lysosomes, leading to lysosomal engorgement and a disruption of cellular
Burris, Ryan Jonathan William
core  

Symptomatic increase in intracranial pressure following pancreatic enzyme replacement therapy for cystic fibrosis [PDF]

, 1995
A newly diagnosed 5-month-old infant with cystic fibrosis (CF) developed signs and symptoms of increased intracranial pressure (ICP) within days of starting pancreatic enzyme replacement therapy. Symptoms promptly resolved on two occasions after stopping
Denise Schaffert, Samya Z. Nasr, Nasr, Samya Z., Schaffert, Denise   +3 more
core   +1 more source

Enzyme replacement therapy in paediatric patients affected by Anderson-Fabry disease leads to improvement in arterial elasticity, but not normalization

Journal of Pediatric and Neonatal Individualized Medicine, 2017
Introduction: Increase in blood pressure, probably due to an impairment in arterial elasticity, is frequent in patients affected by Anderson-Fabry disease (FD).
Pier Paolo Bassareo, Claudio Barbanti, Andrea Raffaele Marras, Giuseppe Mercuro   +3 more
doaj   +1 more source

Identifying transcription factors controlling the basal expression of human MRP4 highlights a substantial role for Sp1

FEBS Open Bio, EarlyView.
The MRP4 transporter exports several drugs and signaling molecules. Here, we identified key promoter elements regulating basal MRP4 expression. Using reporter assays, we defined a conserved region with essential Sp1 and contributory Ets sites, which controlled basal MRP4 expression.
Debora Singer, Angelique Kragl, Katrin Ziems, Juliane Glaubitz, Sophie Grammbauer, Susanne Hildebrandt, Mladen V. Tzvetkov, Gabriele Jedlitschky   +7 more
wiley   +1 more source

Enzyme replacement therapy for Gaucher disease in Australia [PDF]

, 2005
Gaucher disease; enzyme replacement; inherited; metabolic.AimTo study the effectiveness of a specific national programme of enzyme replacement therapy (ERT) for patients with severe forms of Gaucher disease, a disorder of sphingolipid metabolism ...
McGill, J., Szer, J., Wilson, M., Fletcher, J., Rowell, J., Goldblatt, J.   +5 more
core   +1 more source

Engineering of GlcNAc-1-Phosphotransferase for Production of Highly Phosphorylated Lysosomal Enzymes for Enzyme Replacement Therapy

Molecular Therapy: Methods & Clinical Development, 2017
Several lysosomal enzymes currently used for enzyme replacement therapy in patients with lysosomal storage diseases contain very low levels of mannose 6-phosphate, limiting their uptake via mannose 6-phosphate receptors on the surface of the deficient ...
Lin Liu, Wang-Sik Lee, B. Doray, S. Kornfeld   +3 more
semanticscholar   +1 more source

One size does not fit all: An in vitro evaluation of the effects of bezafibrate and medroxyprogesterone acetate on human SH‐SY5Y and U‐87 MG cancer cells

FEBS Open Bio, EarlyView.
Drugs previously repurposed to target blood cancers reduced neuroblastoma and glioblastoma cell growth and viability. However, their levels of anticancer activity were different and their clinical application may be problematic due to side effects at effective doses.
Abhishek Kharawatkar, Francesco Michelangeli, Farhat Latif Khanim, Christopher Martin Bunce, William Eustace Basil Johnson   +4 more
wiley   +1 more source

MAPK dysregulation in the brain pathology of mucopolysaccharidosis IIIB disease [PDF]

, 2010
The accumulation of heparan sulfate (HS) in lysosomes is the primary consequence of the enzyme defect (α-N-acetylglucosaminidase) in Mucopolysaccharidosis type IIIB.
Cecere, Francesca
core  

Hypophosphatasia in childhood: Diagnosis to management

Osteoporosis and Sarcopenia
Hypophosphatasia (HPP) is a rare inherited metabolic bone disorder caused by loss-of-function mutations in the ALPL gene, leading to deficient activity of tissue-nonspecific alkaline phosphatase (TNSALP).
Minji Im, Sung Yoon Cho
doaj   +1 more source