Results 31 to 40 of about 20,747 (199)

Experimental gene therapies for the NCLs

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2020
The neuronal ceroid lipofuscinoses (NCLs), also known as Batten disease, are a group of rare monogenic neurodegenerative diseases predominantly affecting children. All NCLs are lethal and incurable and only one has an approved treatment available. To date, 13 NCL subtypes (CLN1-8, CLN10-14) have been identified, based on the particular disease-causing ...
Liu, Wenfei, kleine-Holthaus, Sophia Martha, Herranz-Martin, Saul, Aristorena, Mikel, Mole, Sara E., Smith, Alexander J., Ali, Robin R., Rahim, Ahad A.   +7 more
openaire   +4 more sources

Photoelectron Photoion Coincidence Spectroscopy of NCl₃ and NCl₂ [PDF]

ChemPhysChem, 2021
AbstractWe investigate NCl3 and the NCl2 radical by photoelectron‐photoion coincidence spectroscopy using synchrotron radiation. The mass selected threshold photoelectron spectrum (ms‐TPES) of NCl3 is broad and unstructured due to the large geometry change. An ionization energy of 9.7±0.1 eV is estimated from the spectrum and supported by computations.
Marius Gerlach, Sophie Monninger, Domenik Schleier, Patrick Hemberger, James T. Goettel, Holger Braunschweig, Ingo Fischer   +6 more
openaire   +2 more sources

Repression of Virulence in Staphylococcus aureus by 2‐Aminothiol‐Mediated Quorum Quenching

Angewandte Chemie, EarlyView.
Simple analogues of cysteine that can eliminate the native autoinducing peptides (AIPs) of staphylococci extracellularly, before they activate their receptor, and turn off quorum sensing in different Staphylococcus aureus. The elimination of AIPs occurs through a mechanism similar to native chemical ligation (NCL). Further, this strategy showed promise
Bengt H. Gless, Riccardo Milazzo, Martin S. Bojer, Benjamin S. Sereika‐Bejder, Hanne Ingmer, Christian A. Olsen   +5 more
wiley   +2 more sources

NCLcomparator: systematically post-screening non-co-linear transcripts (circular, trans-spliced, or fusion RNAs) identified from various detectors

BMC Bioinformatics, 2019
Background Non-co-linear (NCL) transcripts consist of exonic sequences that are topologically inconsistent with the reference genome in an intragenic fashion (circular or intragenic trans-spliced RNAs) or in an intergenic fashion (fusion or intergenic ...
Chia-Ying Chen, Trees-Juen Chuang
doaj   +1 more source

Contribution of patient organisations to the NCLs

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2020
This review will summarise the achievements of Patient Organisations and Foundations started by affected Batten disease families, with an emphasis on their recent contribution to Batten disease, examine the benefits and pitfalls of their interactions with scientists and propose a way forward.
Heather, Band, Frank, Stehr, Noreen, Murphy   +2 more
openaire   +2 more sources

Methylation as a key regulator of Tau aggregation and neuronal health in Alzheimer’s disease

Cell Communication and Signaling, 2021
Neurodegenerative diseases like Alzheimer’s, Parkinson’s and Huntington’s disease involves abnormal aggregation and accumulation of toxic proteins aggregates. Post-translational modifications (PTMs) of the causative proteins play an important role in the
Abhishek Ankur Balmik, Subashchandrabose Chinnathambi   +1 more
doaj   +1 more source

Blockage of Orai1-Nucleolin interaction meditated calcium influx attenuates breast cancer cells growth

Oncogenesis, 2022
As an important second messenger, calcium (Ca2+) regulates a wide variety of physiological processes. Disturbance of intracellular calcium homeostasis implicated in the occurrence of multiple types of diseases.
Chunming Gu, Wenhao Zhang, Enze Yang, Congyou Gu, Zhaoyang Zhang, Jing Ke, Xiong Wang, Shengying Wu, Shan Li, Fuyun Wu   +9 more
doaj   +1 more source

NCLs and ER: A stressful relationship

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2017
The Neuronal Ceroid Lipofuscinoses (NCLs, Batten disease) are a group of inherited neurodegenerative disorders with variable age of onset, characterized by the lysosomal accumulation of autofluorescent ceroid lipopigments. The endoplasmic reticulum (ER) is a critical organelle for normal cell function. Alteration of ER homeostasis leads to accumulation
Marotta, Davide, Tinelli, Elisa, Mole, Sara E.   +2 more
openaire   +3 more sources

One dimensional normal consolidation line equation

Journal of the Mechanical Behavior of Materials, 2022
Many empirical equations have been proposed in the past to predict the parameters of the one-dimensional normal consolidation line (1D-NCL) for soils. However, applications of these equations are limited to specific range of pressure and plasticity soils.
Al-Badran Yasir M., Baille Wiebke, Schanz Tom   +2 more
doaj   +1 more source

Study on Influencing Factors Analysis of Gastric Tube Insertion Length and Construction of Estimation Method

Frontiers in Surgery, 2022
BackgroundInfluenced by individual differences, the depth of gastric tube placement is often different. Clinically, it is necessary to seek a simple and accurate gastric tube insertion scheme to improve the clinical efficacy of indwelling gastric tube ...
Hua Zhang, Huaqin Wang, Huaqin Wang, Xiaoyu Fan, Xiangqun Cao, Wan Su, Bo Yang   +6 more
doaj   +1 more source