Impaired axon transport is one of the earliest pathological manifestations of several neurodegenerative diseases, and mutations in motor proteins can exacerbate or cause degeneration (Williamson and Cleveland, 1999; Gunawardena and Goldstein, 2004; Stokin and Goldstein, 2006). Compromised function in lysosomes and other degradative organelles that intersect with the lysosomal pathway are also s...

The Harvard community has made this article openly available. Please share how this access benefits you. Your story matters. Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in \(hSOD1^{G93A}\) transgenic ALS mice. Amyotrophic lateral sclerosis (ALS) is characterized by predominant vulnerability and central degeneration of both ...

Amyotrophic lateral sclerosis (ALS) is characterized by predominant vulnerability and central degeneration of both corticospinal/corticobulbar motor neurons (CSMN; "upper motor neurons") in cerebral cortex, and spinal/bulbar motor neurons (SMN; "lower motor neurons") in spinal cord and brainstem. Increasing evidence indicates broader cerebral cortex pathology in cognitive, sensory, and associat...

Methylmercury (MeHg) exposure from occupational, environmental, and food sources is a significant threat to public health. MeHg poisonings in adults may result in severe psychological and neurological deficits, and in utero exposures can confer embryonic defects and developmental delays. Recent epidemiological and vertebrate studies suggest that MeHg exposure may also contribute to dopamine (DA...

BACKGROUND Amyotrophic lateral sclerosis (ALS), the most frequent adult onset motor neuron disease, is associated with hypermetabolism linked to defects in muscle mitochondrial energy metabolism such as ATP depletion and increased oxygen consumption. It remains unknown whether muscle abnormalities in energy metabolism are causally involved in the destruction of neuromuscular junction (NMJ) and ...

Motor neurons typically have very long axons, and fine-tuning axonal transport is crucial for their survival. The obstruction of axonal transport is gaining attention as a cause of neuronal dysfunction in a variety of neurodegenerative motor neuron diseases. Depletions in dynein and dynactin-1, motor molecules regulating axonal trafficking, disrupt axonal transport in flies, and mutations in th...

Increasing evidence has suggested an important role for environmental factors such as exposure to pesticides in the pathogenesis of Parkinson's disease. In experimental animals the exposure to a common herbicide, rotenone, induces features of parkinsonism; mechanistically, rotenone-induced destruction of dopaminergic neurons has been attributed to its inhibition of the activity of neuronal mito...

BACKGROUND AND PURPOSE Delayed paraplegia remains a devastating complication after ischemic spinal cord injury associated with aortic surgery and trauma. Although apoptosis has been implicated in the pathogenesis of delayed neurodegeneration, mechanisms responsible for the delayed paraplegia remain incompletely understood. The aim of this study was to elucidate the role of apoptosis in delayed ...

Objective(s) Motor deficit and neuron degeneration is seen after nerve transection. The aim of this study is to determine whether a poled polyvinelidene fluoride (PVDF) tube with other supportive strategies can protect the neuronal morphology and motor function after sciatic nerve transaction in rats. Materials and Methods After transection of the left sciatic nerve in 60 male Wistar rats (2...

Amyotrophic lateral sclerosis is a neurodegenerative disorder that results in the selective death of motor neurons in the central nervous system. This progressive motor neuron degeneration leads to death of the patient on average three to five years after onset of the disease. To date, no therapy is available. Many hypotheses have been formulated to explain the selective degeneration of motor n...