Reduced numbers and intense anti-ubiquitin immunostaining of bovine motor neurons affected with spinal muscular atrophy

Hiraga, Takeo; Leipold, H. W.; Cash, W. C.; Troyer, Deryl L.

doi:10.1016/0022-510x(93)90244-s

Cited by 8 publications

(3 citation statements)

References 22 publications

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“…Ubiquitin has been implicated in playing a role in several neurodegenerative diseases such as frontotemporal lobar degeneration [31]–[32], schizophrenia [33], Parkinson’s [34], spinal muscular atrophy [35], motor neuron disease/amyotrophic lateral sclerosis [36]–[40], often associated with filamentous inclusion bodies found in these types of disorder [31],[38]. In spinal cord injury ubiquitin has been detected in axonal swellings [41]; however in this study there was no evidence of ubiquitinated neuronal inclusions.…”

Section: Discussionmentioning

confidence: 50%

Expression and Cellular Distribution of Ubiquitin in Response to Injury in the Developing Spinal Cord of Monodelphis domestica

et al. 2013

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Ubiquitin, an 8.5 kDa protein associated with the proteasome degradation pathway has been recently identified as differentially expressed in segment of cord caudal to site of injury in developing spinal cord. Here we describe ubiquitin expression and cellular distribution in spinal cord up to postnatal day P35 in control opossums (Monodelphis domestica) and in response to complete spinal transection (T10) at P7, when axonal growth through site of injury occurs, and P28 when this is no longer possible. Cords were collected 1 or 7 days after injury, with age-matched controls and segments rostral to lesion were studied. Following spinal injury ubiquitin levels (western blotting) appeared reduced compared to controls especially one day after injury at P28. In contrast, after injury mRNA expression (qRT-PCR) was slightly increased at P7 but decreased at P28. Changes in isoelectric point of separated ubiquitin indicated possible post-translational modifications. Cellular distribution demonstrated a developmental shift between earliest (P8) and latest (P35) ages examined, from a predominantly cytoplasmic immunoreactivity to a nuclear expression; staining level and shift to nuclear staining was more pronounced following injury, except 7 days after transection at P28. After injury at P7 immunostaining increased in neurons and additionally in oligodendrocytes at P28. Mass spectrometry showed two ubiquitin bands; the heavier was identified as a fusion product, likely to be an ubiquitin precursor. Apparent changes in ubiquitin expression and cellular distribution in development and response to spinal injury suggest an intricate regulatory system that modulates these responses which, when better understood, may lead to potential therapeutic targets.

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Section: Discussionmentioning

confidence: 50%

Expression and Cellular Distribution of Ubiquitin in Response to Injury in the Developing Spinal Cord of Monodelphis domestica

et al. 2013

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“…16) (8, 81, 267). The cytoskeleton of the neurons disintegrates through this process (131,132). Wallerian-type degeneration of axons within the spinal cord white matter, radix ventralis and peripheral nerves, briefly characterised by axonal swelling, loss of axons, infiltration by macrophages and distension of myelin sheets, is seen (8,267,293).…”

Section: Spinal Muscular Atrophymentioning

confidence: 99%

Inherited Disorders in Danish Cattle

Agerholm¹

2007

Apmis

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“…16) (8, 81, 267). The cytoskeleton of the neurons disintegrates through this process (131, 132). Wallerian‐type degeneration of axons within the spinal cord white matter, radix ventralis and peripheral nerves, briefly characterised by axonal swelling, loss of axons, infiltration by macrophages and distension of myelin sheets, is seen (8, 267, 293).…”

Section: Inherited Disorders In Danish Cattlementioning

confidence: 99%

Inherited Disorders in Danish Cattle

Agerholm

2007

APMIS

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Reduced numbers and intense anti-ubiquitin immunostaining of bovine motor neurons affected with spinal muscular atrophy

Cited by 8 publications

References 22 publications

Expression and Cellular Distribution of Ubiquitin in Response to Injury in the Developing Spinal Cord of Monodelphis domestica

Expression and Cellular Distribution of Ubiquitin in Response to Injury in the Developing Spinal Cord of Monodelphis domestica

Inherited Disorders in Danish Cattle

Inherited Disorders in Danish Cattle

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