Peripherin partially localizes in Bunina bodies in amyotrophic lateral sclerosis

Mizuno, Yuji; Fujita, Yukio; Takatama, Masamitsu; Okamoto, Koichi

doi:10.1016/j.jns.2010.12.023

Cited by 27 publications

(14 citation statements)

References 36 publications

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“…They are rarely seen in Betz cells, neurons of oculomotor nuclei, and Onuf’s nuclei 35-37 . Immunohistochemically, Bunina bodies are positive for cystatin c (Figure 2K, L) and transferrin and partially colocalize with peripherin 38, 39 , but they are negative for a variety of proteins commonly associated with neurodegeneration, including tau, alpha and beta tubulin, synaptophysin, amyloid precursor protein, glial fibrillary acidic protein, alpha synuclein, and p62 38, 40-42 . Interestingly, it remains controversial whether or not Bunina bodies are positive for ubiquitin 10, 43 .…”

Section: Classic Als Neuropathologymentioning

confidence: 99%

Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants

et al. 2015

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Summary Amyotrophic lateral sclerosis (ALS) is a clinical syndrome named for its neuropathological hallmark: degeneration of motor neurons in the spinal anterior horn and motor cortex and loss of axons in the lateral columns of the spinal cord. The signature neuropathological molecular signature common to almost all sporadic ALS and most familial ALS is TDP-43 immunoreactive neuronal cytoplasmic inclusions. The neuropathological and molecular neuropathological features of ALS variants primarly lateral sclerosis and progressive muscular atrophy are less certain, but also appear to share the primary features of ALS. A number of genetic causes including mutations in SOD1, FUS, and C9orf72 comprise a disease spectrum and all demonstrate distinctive molecular and neuropathological signatures. Neuropathology will continue to play to a key role in solving the puzzle of ALS pathogenesis.

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Section: Classic Als Neuropathologymentioning

confidence: 99%

Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants

et al. 2015

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“…Bunina bodies are comprised of cystatin C, transferrin, peripherin, and sortilin-related receptor CNS expressed 2 (SORCS2) and are found in the surviving lower motor neurons within the brain stem and spinal cord (Okamoto et al, 1993; Piao et al, 2003; Mizuno et al, 2006, 2011; Mori et al, 2015). They also contain small organelle fragments such as vesicles and ER (Okamoto et al, 2008; Kimura et al, 2014).…”

Section: Loss Of Protein Homeostasis In Alsmentioning

confidence: 99%

Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?

Webster

Smith

Shaw

et al. 2017

Front. Mol. Neurosci.

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Protein homeostasis (proteostasis), the correct balance between production and degradation of proteins, is essential for the health and survival of cells. Proteostasis requires an intricate network of protein quality control pathways (the proteostasis network) that work to prevent protein aggregation and maintain proteome health throughout the lifespan of the cell. Collapse of proteostasis has been implicated in the etiology of a number of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), the most common adult onset motor neuron disorder. Here, we review the evidence linking dysfunctional proteostasis to the etiology of ALS and discuss how ALS-associated insults affect the proteostasis network. Finally, we discuss the potential therapeutic benefit of proteostasis network modulation in ALS.

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“…It acts as a cytoskeletal protein and is present in the neurons of the peripheral nervous system [101]. PRPH expression is increased in the spinal motor neurons after neuronal injury, indicating its role in axonal regeneration [101].…”

Section: Genetics Of Alsmentioning

confidence: 99%

Genetics of amyotrophic lateral sclerosis: an update

Chen

Sayana

Zhang

et al. 2013

Mol Neurodegeneration

297

230

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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving both upper motor neurons (UMN) and lower motor neurons (LMN). Enormous research has been done in the past few decades in unveiling the genetics of ALS, successfully identifying at least fifteen candidate genes associated with familial and sporadic ALS. Numerous studies attempting to define the pathogenesis of ALS have identified several plausible determinants and molecular pathways leading to motor neuron degeneration, which include oxidative stress, glutamate excitotoxicity, apoptosis, abnormal neurofilament function, protein misfolding and subsequent aggregation, impairment of RNA processing, defects in axonal transport, changes in endosomal trafficking, increased inflammation, and mitochondrial dysfunction. This review is to update the recent discoveries in genetics of ALS, which may provide insight information to help us better understanding of the disease neuropathogenesis.

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Peripherin partially localizes in Bunina bodies in amyotrophic lateral sclerosis

Cited by 27 publications

References 36 publications

Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants

Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants

Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?

Genetics of amyotrophic lateral sclerosis: an update

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