Results 91 to 100 of about 114,325,321 (319)
Advanced Science, EarlyView.This graphical abstract illustrates how chaperone‐mediated autophagy (CMA) regulates intervertebral disc degeneration (IDD). Under normal homeostasis (B), CMA degrades cytoplasmic Midnolin (MIDN) to maintain proteostasis. Under inflammatory stress (A), impaired CMA leads to cytoplasmic MIDN accumulation.
Xianglong Chen +9 more
wiley +1 more source
Cell Transplantation Combined with Recombinant Collagen Peptides for the Treatment of Fabry Disease
Cell Transplantation, 2020 Fabry disease is caused by a decrease in or loss of the activity of alpha-galactosidase, which causes its substrates globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3) to accumulate in cells throughout the body. This accumulation results
Daisuke Kami +6 more
doaj +1 more source
Advanced Science, EarlyView.The protein aggregates and gene expression in the middle temporal gyrus (MTG) and somatosensory cortex (SOM) of the postmortem brains of 13 Alzheimer's disease patients were studied in detail, revealing that small hyperphosphorylated tau aggregates increase with Braak stage driven by microglial inflammation.
Elizabeth A. English +9 more
wiley +1 more source
Identification and Clinical Characterization of a Novel Alpha-Galactosidase A Mutation
Journal of Inborn Errors of Metabolism and Screening, 2014 Fabry disease (FD) is an inborn error of metabolism characterized by deficient/absent activity of lysosomal enzyme alpha-galactosidase A, which results in systemic accumulation of glycosphingolipids and progression to renal failure, heart and ...
Nilton Salles Rosa Neto MD, PhD
doaj +1 more source
Marine Drugs, 2018 A novel wild-type recombinant cold-active α-d-galactosidase (α-PsGal) from the cold-adapted marine bacterium Pseudoalteromonas sp. KMM 701, and its mutants D451A and C494N, were studied in terms of their structural, physicochemical, and ...
Irina Bakunina +8 more
doaj +1 more source
Genetic determination of the alpha-galactosidase developmental program in mice.
, 1975 The expression of alpha-galactosidase in liver, heart, and brain during postembryonic development has been examined in several inbred mouse strains. In most strains, the developmental patterns of alpha-galactosidase are coordinate with those of two ...
Lusis, A J, Paigen, K
core +1 more source
Cardiac involvement in Anderson–Fabry disease. The role of advanced echocardiography
Frontiers in Cardiovascular MedicineAnderson–Fabry disease (AFD) is a lysosomal storage disorder, depending on defects in alpha galactosidase A activity, due to a mutation in the galactosidase alpha gene. Cardiovascular involvement represents the leading cause of death in AFD.
Letizia Spinelli +5 more
doaj +1 more source
Notes on the founding and membership of the Alpha Rho and BKΩ chapters of AKA, undated
, 1970 Notes narrating the history of the Alpha Rho and BKΩ chapters of Alpha Kappa Alpha, along with memberships lists from various years.Archival master image scanned in reflective mode as 48-bit RGB color TIFF using Epson 11000 XL. Display image is 8-bit 150
Alpha Kappa Alpha
core
Diagnostic Pathology, 2010 Background Lysosomal storage diseases (LSD) are inherited disorders caused by deficiency of lysosomal enzymes in which early diagnosis is essential to provide timely treatment. This study reports interval values for the activity of lysosomal enzymes that
Martins Ana M +4 more
doaj +1 more source
β-galactosidase-specific band shifts were not observed in monocots and basal angiosperms samples.
, 2023 (A) Tomato fruits produced GBGM oligo after AnGH5 digestion, which migrated to GAGM after the following β-galactosidase treatment. M: marker lane (standard oligo saccharides).
Alberto Echevarría-Poza (17692296) +7 more
core +1 more source