Regulation of mammalian autophagy in physiology and pathophysiology
(Macro) autophagy is a bulk degradation process that mediates the clearance of long-lived
proteins and organelles. Autophagy is initiated by double-membraned structures, which …
proteins and organelles. Autophagy is initiated by double-membraned structures, which …
The roles of intracellular protein-degradation pathways in neurodegeneration
DC Rubinsztein - Nature, 2006 - nature.com
Many late-onset neurodegenerative diseases, including Parkinson's disease and
Huntington's disease, are associated with the formation of intracellular aggregates by toxic …
Huntington's disease, are associated with the formation of intracellular aggregates by toxic …
[HTML][HTML] Molecular motors in neurons: transport mechanisms and roles in brain function, development, and disease
The kinesin, dynein, and myosin superfamily molecular motors have fundamental roles in
neuronal function, plasticity, morphogenesis, and survival by transporting cargos such as …
neuronal function, plasticity, morphogenesis, and survival by transporting cargos such as …
Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man
LR Fischer, DG Culver, P Tennant, AA Davis… - Experimental …, 2004 - Elsevier
The SOD1 mutant mouse is the most widely used model of human amyotrophic lateral
sclerosis (ALS). To determine where and when the pathological changes of motor neuron …
sclerosis (ALS). To determine where and when the pathological changes of motor neuron …
Unraveling the mechanisms involved in motor neuron degeneration in ALS
LI Bruijn, TM Miller, DW Cleveland - Annu. Rev. Neurosci., 2004 - annualreviews.org
▪ Abstract Although Charcot described amyotrophic lateral sclerosis (ALS) more than 130
years ago, the mechanism underlying the characteristic selective degeneration and death of …
years ago, the mechanism underlying the characteristic selective degeneration and death of …
Axonal transport deficits and neurodegenerative diseases
S Millecamps, JP Julien - Nature Reviews Neuroscience, 2013 - nature.com
The intracellular transport of organelles along an axon is crucial for the maintenance and
function of a neuron. Anterograde axonal transport has a role in supplying proteins and …
function of a neuron. Anterograde axonal transport has a role in supplying proteins and …
[HTML][HTML] Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
LR Gauthier, BC Charrin, M Borrell-Pagès… - Cell, 2004 - cell.com
Polyglutamine expansion (polyQ) in the protein huntingtin is pathogenic and responsible for
the neuronal toxicity associated with Huntington's disease (HD). Although wild-type …
the neuronal toxicity associated with Huntington's disease (HD). Although wild-type …
Axonal transport and neurological disease
Axonal transport is the process whereby motor proteins actively navigate microtubules to
deliver diverse cargoes, such as organelles, from one end of the axon to the other, and is …
deliver diverse cargoes, such as organelles, from one end of the axon to the other, and is …
Molecular biology of amyotrophic lateral sclerosis: insights from genetics
P Pasinelli, RH Brown - Nature Reviews Neuroscience, 2006 - nature.com
Amyotrophic lateral sclerosis (ALS) is a paralytic disorder caused by motor neuron
degeneration. Mutations in more than 50 human genes cause diverse types of motor neuron …
degeneration. Mutations in more than 50 human genes cause diverse types of motor neuron …
Neurodegenerative diseases: a decade of discoveries paves the way for therapeutic breakthroughs
MS Forman, JQ Trojanowski, VMY Lee - Nature medicine, 2004 - nature.com
A wide variety of neurodegenerative diseases are characterized by the accumulation of
intracellular or extracellular protein aggregates. More recently, the genetic identification of …
intracellular or extracellular protein aggregates. More recently, the genetic identification of …