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The Neuronal Channel NALCN Contributes Resting Sodium Permeability and Is Required for Normal Respiratory Rhythm
Sodium plays a key role in determining the basal excitability of the nervous systems through the resting "leak" Na(+) permeabilities, but the molecular identities of the TTX- and Cs(+)-resistantExpand
Extracellular Calcium Controls Background Current and Neuronal Excitability via an UNC79-UNC80-NALCN Cation Channel Complex
In contrast to its extensively studied intracellular roles, the molecular mechanisms by which extracellular Ca(2+) regulates the basal excitability of neurons are unclear. One mechanism is believedExpand
Peptide neurotransmitters activate a cation channel complex of NALCN and UNC-80
Several neurotransmitters act through G-protein-coupled receptors to evoke a ‘slow’ excitation of neurons. These include peptides, such as substance P and neurotensin, as well as acetylcholine andExpand
A novel human embryonic stem cell‐derived Huntington's disease neuronal model exhibits mutant huntingtin (mHTT) aggregates and soluble mHTT‐dependent neurodegeneration
  • B. Lu, J. Palacino
  • Medicine, Biology
  • FASEB journal : official publication of the…
  • 1 May 2013
Most neurodegenerative diseases are linked to aberrant accumulation of aggregation‐prone proteins. Among them, Huntington's disease (HD) is caused by an expanded polyglutamine repeat stretch in the NExpand
Identification of NUB1 as a suppressor of mutant Huntingtin toxicity via enhanced protein clearance
Huntington's disease is caused by expanded CAG repeats in HTT, conferring toxic gain of function on mutant HTT (mHTT) protein. Reducing mHTT amounts is postulated as a strategy for therapeuticExpand
A toxic mutant huntingtin species is resistant to selective autophagy.
Protein misfolding is a common theme in neurodegenerative disorders including Huntington's disease (HD). The HD-causing mutant huntingtin protein (mHTT) has an expanded polyglutamine (polyQ) stretchExpand
TR-FRET Assays of Huntingtin Protein Fragments Reveal Temperature and PolyQ Length-Dependent Conformational Changes
Time-Resolved Fluorescence Resonance Energy Transfer (TR-FRET) technology is a widely used immunoassay that enables high-throughput quantitative measurements of proteins of interest. One of the wellExpand
Allele-selective lowering of mutant HTT protein by HTT–LC3 linker compounds
Compounds that interact with mutant huntingtin and an autophagosomal protein are able to reduce cellular levels of mutant Huntingtin by targeting it for autophagic degradation, demonstrating an approach that may have potential for treating proteopathies. Expand
A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity
Huntington's disease (HD) represents an important model for neurodegenerative disorders and proteinopathies. It is mainly caused by cytotoxicity of the mutant huntingtin protein (Htt) with anExpand
Cytoplasmic DAXX drives SQSTM1/p62 phase condensation to activate Nrf2-mediated stress response
Autophagy cargo recognition and clearance are essential for intracellular protein quality control. SQSTM1/p62 sequesters intracellular aberrant proteins and mediates cargo delivery for theirExpand