Results 11 to 20 of about 10,629 (179)

Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation [PDF]

open access: yes, 2014
Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine, have positive effects on mouse models of human cystic fibrosis (CF). These measures
Salvadori L   +112 more
core   +1 more source

SERS Spectral Bands of L-Cysteine, Cysteamine and Homocysteine fitted by Tsallis q-Gaussian functions

open access: yes, 2023
Tsallis q-Gaussian functions are probability distributions possessing a shape which can be stretched from a Lorentzian to a Gaussian profile, according to the q-index parameter of their generalized exponentials.
Amelia Carolina, Sparavigna
core   +1 more source

Rotational spectroscopic study of cysteamine

open access: yes, 2023
Cysteamine (NH2CH2CH2SH) is a molecule that is potentially of interest to astrobiology, but it has not yet been detected in the interstellar medium. Its rotational spectrum was investigated in the 18-40 GHz frequency region and two conformers and their ...
Song, Wentao
core   +1 more source

Total Chemical Synthesis of Interleukin‐15 and Interleukin‐2: Taming Protein Hydrophobicity and Aggregation by a Versatile Solubilizing Strategy

open access: yesAngewandte Chemie, EarlyView.
A reducible solubilizing tag strategy (RST‐2.0) enables the synthesis, purification and ligation of difficult peptides while being readily removable just during folding, which overcomes the common challenges from protein hydrophobicity and aggregation. With this method, we achieved the first total synthesis of native and N‐glycosylated IL‐15 as well as
Jingwen Zeng   +4 more
wiley   +2 more sources

Three Component Thio‐ and Carboboration of Alkynes: A Modular Route to Functionalised Bicyclic Boronates

open access: yesAngewandte Chemie, EarlyView.
Three‐component thio‐ and carbo‐boration reactions to form functionalised benzoxaborinines and benzazaborinines are reported. The nucleophile scope includes thioethers, thiols, and (hetero)arenes. Mechanistic studies revealed a disparity between thioboration using thioethers and using thiols.
Laura Winfrey   +4 more
wiley   +2 more sources

A comparison of immediate release and delayed release cysteamine in 17 patients with nephropathic cystinosis

open access: yes, 2021
Background Nephropathic cystinosis is a rare and severe metabolic disease leading to an accumulation of cystine in lysosomes which especially harms kidney function.
Grebe, Jürgen   +15 more
core   +1 more source

Investigation Of A Novel Mammalian Thiol Dioxygenase Structure: Human Cysteamine Dioxygenase

open access: yes, 2018
In 2007, a gene homolog of CDO encoded by the gene Gm237 in the DUF164 family was identified as cysteamine dioxygenase (ADO). ADO is one of the only known thiol dioxygenases found in mammals.
Xiong, Tseng
core   +1 more source

Effect of cysteamine on mutant ASL proteins with cysteine for arginine substitutions

open access: yes, 2016
INTRODUCTION Cysteamine is used to treat cystinosis via the modification of cysteine residues substituting arginine in mutant proteins. OBJECTIVES We investigated the effect of cysteamine on mutant argininosuccinate lyase (ASL), the second most common ...
Rüfenacht, Véronique   +9 more
core   +1 more source

Intrinsic Photoactive Star ZnPc–Poly(glutamate) Nanoplatforms for Multimodal Glioblastoma Therapy and Brain‐Targeted Delivery

open access: yesAdvanced Functional Materials, EarlyView.
An intrinsic photoactive star‐shaped zinc phtalocyanine‐poly(L‐glutamic acid) (ZnPc‐PGA) nanoplatform for multimodal glioblastoma (GBM) therapy and brain‐targeted elivery. A ZnPc‐PGA‐based multifunctional theranostic nanocarrier platform enables image‐guided, multimodal GBM therapy. ZnPc‐PGA nanocarriers support the integration of fluorescence imaging,
Amina Benaicha‐Fernández   +14 more
wiley   +1 more source

Packed Hydrogel Microfibers as Scaffolds Supporting Dynamic Cellular Behavior and Biomaterial Inks in 3D Printing

open access: yesAdvanced Healthcare Materials, EarlyView.
Packed hydrogel microfiber (PHM) materials consist of flexible and high aspect ratio hydrogel components that, as a bulk material, are simultaneously mechanically robust and dynamic. Cells cultured in or on PHM scaffolds can be influenced by topographical cues or interact with a dynamic environment that permits cell spreading and multicellular ...
M. Gregory Grewal   +7 more
wiley   +1 more source

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