Results 51 to 60 of about 25,632 (110)

Enzyme Replacement Therapy Clears Gb3 Deposits from a Podocyte Cell Culture Model of Fabry Disease but Fails to Restore Altered Cellular Signaling.

Cellular Physiology and Biochemistry, 2019
BACKGROUND/AIMS Fabry disease (FD) is a lysosomal storage disorder characterized by impaired alpha-galactosidase A (α-Gal A) enzyme activity due to mutations in the GLA gene. While virtually all tissues are affected, renal damage is particularly critical
Fabian Braun, Linda Blomberg, S. Brodesser, M. Liebau, B. Schermer, T. Benzing, C. Kurschat   +6 more
semanticscholar   +1 more source

Lyso-Gb3 Increases αvβ3 Integrin Gene Expression in Cultured Human Podocytes in Fabry Nephropathy

Journal of Clinical Medicine, 2020
Background: Podocyturia in Fabry nephropathy leads to glomerulosclerosis and kidney disease progression. Integrins are involved in podocyte attachment to the glomerular basement membrane. We hypothesized that in Fabry nephropathy, lyso-Gb3 could modulate
H. Trimarchi, A. Ortiz, M. Sánchez-Niño   +2 more
semanticscholar   +1 more source

Geochemical and C-O isotopic study of ∼2-1.9 Ga phosphate-bearing marine sedimentary rocks of the Sonrai Formation, Bijawar Group, Lalitpur district, UP, India: Implications for paleoredox, paleoclimate, and paleosalinity

Geosystems and Geoenvironment
The Sonrai Formation of Bijawar Group, Lalitpur, Uttar Pradesh, India hosts phosphorite deposits. This study has been carried out using geochemical and stable isotope approaches to decipher the paleo-redox, paleo-climate, and paleo-salinity conditions of
Shamim A. Dar, K.F. Khan, Akhtar R. Mir, Tsuyoshi Komiya, Nurul Absar, Mohd Shuaib, Waseem Raza   +6 more
doaj   +1 more source

Detecting sub-basalt Mesozoic sediments and active tectonics in the Narmada-Tapti Rift Zone, central India

Geosystems and Geoenvironment, 2022
To obtain structural information below basalt cover using conventional seismic reflection has always been a challenge due to the high impedance of the basaltic layer. This study enumerates P-wave refraction tomography to image the shallow structure along
Biswajit Mandal, M.M. Dixit, Sanjay Kumar, P. Karuppannan, K. Laxminaryana, R.D. Catchings, Laxmidhar Behera, Prakash Kumar   +7 more
doaj   +1 more source

Reconstructing ancient vegetation, climate, depositional setting and ecology of the West Bokaro Coalfield, India: Insights from megafloristics and palynofacies

Geosystems and Geoenvironment
This study investigates the ancient vegetation, climate, depositional setting, and ecology of the West Bokaro Coalfield, with a focus on megafloral and palynofacies analyses from the Parej East Open Cast Project (OCP).
Soumyashree Nanda, Shreerup Goswami, Neha Aggarwal, Debashish Das   +3 more
doaj   +1 more source

Temperature effect on the mechanical behavior of shale: Implication for shale gas production

Geosystems and Geoenvironment, 2022
Deformational shale attributes such as fracture toughness, tensile strength, and axial point load strength (PLS) index under elevated temperatures are crucial for the petroleum and gas industries. Here, an attempt has been made to investigate the thermal
Vikram Vishal, Mohd. Rizwan, Bankim Mahanta, S.P. Pradhan, T.N. Singh   +4 more
doaj   +1 more source

Gb3‐cSrc complex in glycosphingolipid‐enriched microdomains contributes to the expression of p53 mutant protein and cancer drug resistance via β‐catenin–activated RNA methylation

FASEB bioAdvances, 2020
Glucosylceramide synthase (GCS) is a key enzyme catalyzing ceramide glycosylation to generate glucosylceramide (GlcCer), which in turn serves as the precursor for cells to produce glycosphingolipids (GSLs).
K. Roy, M. Uddin, S. Roy, R. A. Hill, J. Marshall, Yu-Teh Li, J. Chamcheu, Hua Lu, Yong-Yu Liu   +8 more
semanticscholar   +1 more source

Characterization of Java Island's volcanoes based on volcano density and quantification of volcanic products using Landsat imagery

Geosystems and Geoenvironment
Java Island has 34 volcanoes that the twenty of them are the most active termed as type-A. The process of volcano generation and the characteristics of Java Island as part of the Sunda Arc produce Quarternary volcanoes from the western to the eastern ...
Supriyadi, Asep Saepuloh, Harry Mahardika, Wahyu Srigutomo   +3 more
doaj   +1 more source

Plasma lyso-Gb3: a biomarker for monitoring fabry patients during enzyme replacement therapy

Clinical and Experimental Nephrology, 2018
BackgroundRecently, globotriaosylsphingosine (lyso-Gb3) has attracted interest as a biomarker of Fabry disease. However, little is known regarding its utility for the evaluation of the therapeutic efficacy.MethodWe measured plasma lyso-Gb3 concentration ...
H. Sakuraba, T. Togawa, T. Tsukimura, H. Kato   +3 more
semanticscholar   +1 more source

13C NMR Relaxation Analysis of Protein GB3 for the Assessment of Sidechain Dynamics Predictions by Current AMBER and CHARMM Force Fields.

Journal of Chemical Theory and Computation, 2020
Molecular simulations with seven current AMBER- and CHARMM-based force fields yield markedly differing internal bond vector autocorrelation function predictions for many of the 223 methine and methylene H-C bonds of the 56-residue protein GB3.
Janet S. Anderson, G. Hernández, D. LeMaster   +2 more
semanticscholar   +1 more source