5'-deoxy-5'-phosphonomethyladenosine phosphate

01/01/2017 - "Ppca-/- mice have proven to be a suitable model to test different therapeutic approaches, paving the way for the development of clinical trials for patients with galactosialidosis. "
01/01/2017 - "This review gives a historic overview on the discovery of PPCA, which defined galactosialidosis as a new clinical entity; the evidence for the existence of the PPCA/NEU1/β-GAL complex; the clinical forms of galactosialidosis and disease-causing CTSA mutations. "
06/01/2004 - "The capacity of recombinant PPCA and Neu1 to clear the lysosomal storage in target cells was assessed in PPCA-/- mice, a model of galactosialidosis. "
01/01/2000 - "Endothelin-1-specific immunoreactivity has been demonstrated to be markedly increased and distributed abnormally in the neurons and glial cells within autopsied brain regions, including the cerebellum, hippocampal formation, and spinal cord, of patients affected with galactosialidosis, a human PPCA deficiency. "
10/01/1998 - "Lack of PPCA expression only partially coincides with lysosomal storage in galactosialidosis mice: indirect evidence for spatial requirement of the catalytic rather than the protective function of PPCA."

01/25/2022 - "We hypothesize that patients with mPDAC will benefit from a local treatment of the pPCa by MR-guided SBRT in terms of a durable pain control with a simultaneously favorable safe toxicity profile translating into an improvement of quality-of-life. "
01/25/2022 - "However, prospective data on pain control after SBRT of pPCa is very limited. "
01/25/2022 - "This is a prospective, double-arm, randomized controlled, international multicenter study testing the added benefit of MR-guided adaptive SBRT of the pPca embedded between standard of care-chemotherapy (SoC-CT) cycles for pain control and prevention of pain in patients with mPDAC. "
01/25/2022 - "Palliative low-dose radiotherapy is a well-established local treatment option but there is some evidence for a better and longer pain response after a dose-intensified radiotherapy of the primary pancreatic cancer (pPCa). "
07/01/2012 - "Intrathecal morphine 0.3 mg is safe and effective way to improves pain control for thoracic surgery and was comparable to paravertebral patient control analgesia (PPCA) with bupivacaine for the 1(st) 48 hours post-thoracotomy."

10/01/2013 - "These results suggest that at least some of the NEU1 mutations associated with type I sialidosis may respond to PPCA-chaperone-mediated gene therapy. "
10/01/2013 - "Chaperone-mediated gene therapy with recombinant AAV-PPCA in a new mouse model of type I sialidosis."
01/01/2020 - "NEU1 is deficient in the neurodegenerative lysosomal storage disease sialidosis, and its targeting to and stability in lysosomes strictly depend on PPCA. "
06/01/2014 - "Here we review how studies of basic biological processes in the mouse models of three lysosomal storage disorders, galactosialidosis, sialidosis, and GM1-gangliosidosis, revealed new and unexpected roles for the three respective affected enzymes, Ppca, Neu1, and β-Gal, that go beyond their canonical degradative activities. "
02/01/2006 - "Apparently normal elastogenesis and EBP mRNA expression were observed for fibroblasts from Morquio B disease cases with the GLB1 gene alleles (W273L/W273L, W273L/R482H and W273L/W509C substitutions, respectively), a galactosialidosis case with the PPCA allele (IVS7+3A/IVS7+3A) and a sialidosis case with the NEU1 allele (V217M/G243R) as well as normal subject. "

10/01/2016 - "Clinical experience highlights the wide heterogeneity of primary prostate cancer (PPCa), even when potentially related to the same grade and stage. "
01/01/2020 - "This study aims to describe the pathological features and clinical outcomes in anterior-dominant prostate cancer (APCA) compared to posterior/posterolateral-dominant prostate cancer (PPCA) among men treated with radical prostatectomy for localized prostate cancer. "
06/23/2020 - "The aim of our work is to evaluate the principal differences of the pathological features in prostate cancer (PCa) lesions comparing those in the anterior region of the gland (APCa) to those in the posterior zone (PPCa) among patients who underwent to robotic-assisted radical prostatectomy (RP). "

06/01/2006 - "To study the relationship between peripapillary choroidal atrophy (PPCA) and mean defect (MD) in visual field in primary open angle glaucoma (POAG) and suspected open angle glaucoma (SOAG). "
09/10/2019 - "In all eyes were noticed a central atrophy (CA) area with absolute absence of CC that corresponded to 0 dB points at MP, a near atrophy (NA) zone of PPCA impairment that included points with decreased sensitivity at MP and a distant from atrophy (DA) zone with higher PPCA and retinal sensitivity values. "