Results 141 to 150 of about 9,391,147 (295)

Quality Control Gone Wrong: Mitochondria, Lysosomal Storage Disorders and Neurodegeneration. [PDF]

open access: yes, 2013
The eukaryotic cell possesses specialized pathways to turnover and degrade redundant proteins and organelles. Each pathway is unique and responsible for degradation of distinctive cytosolic material.
Duchen, MR, Osellame, LD
core   +1 more source

Effect of a Spray Containing Occlusive Agents, Humectants and Physiological Lipids on Skin Hydration of Healthy Dogs When Applied After Bathing With a Chlorhexidine 2%/Miconazole 2% Shampoo

open access: yesVeterinary Dermatology, Volume 37, Issue 1, Page 140-147, February 2026.
Background: Normal hydration of the canine epidermis is imperative for cutaneous homeostasis. Xerosis may be encountered in canine atopic dermatitis and may be aggravated by topical antiseptics. Hypothesis and Objectives: To evaluate the hydrating properties and the safety of a spray (Sensiderm spray; MP Labo) when applied after shampooing healthy dogs
Adamantia Pseftogka   +3 more
wiley   +1 more source

Diagnosing neuronopathic Gaucher disease: New considerations and challenges in assigning Gaucher phenotypes.

open access: yesMolecular Genetics and Metabolism, 2021
Emily C Daykin, Emory Ryan, E. Sidransky
semanticscholar   +1 more source

No evidence for substrate accumulation in Parkinson brains with GBA mutations [PDF]

open access: yes, 2015
To establish whether Parkinson's disease (PD) brains previously described to have decreased glucocerebrosidase activity exhibit accumulation of the lysosomal enzyme's substrate, glucosylceramide, or other changes in lipid ...
Gegg, ME   +5 more
core   +1 more source

Simultaneous Grafting of 3,4,5‐Trihydroxypiperidine Iminosugars Onto Multivalent Scaffolds via Double Reductive Amination Provides New GCase Inhibitors

open access: yesChemistry – A European Journal, Volume 32, Issue 2, 9 January 2026.
A small set of low‐valency multivalent 3,4,5‐trihydroxypiperidines was prepared through double reductive amination (DRA) of a sugar‐derived dialdehyde. Among them, trivalent derivative 37 emerged as a potent β‐glucocerebrosidase (GCase) inhibitor displaying an unexpected mixed‐type kinetic profile. The integration of kinetic analysis, NMR, docking, and
Maria Giulia Davighi   +10 more
wiley   +1 more source

GAUCHER'S DISEASE [PDF]

open access: yesMedical Journal Armed Forces India, 1999
C, Vidyashankar   +4 more
openaire   +2 more sources

Treatment options for patients with Gaucher disease

open access: yesEgyptian Journal of Medical Human Genetics, 2016
Gaucher disease is the most common lysosomal storage disorder due to deficiency of ß-glucocerebrosidase. Since the introduction of Ceredase in 1991, enzyme replacement therapy has been the mainstay of treatment with its major disadvantage of long life ...
Rabah M. Shawky, Solaf M. Elsayed
doaj   +1 more source

Gene expression with corresponding pathways analysis in Gaucher disease

open access: green, 2021
Łukasz Pawliński   +9 more
openalex   +2 more sources

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