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Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

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Gene expression profiling of resistant and vulnerable motor neuron subtypes in amyotrophic lateral sclerosis

ABSTRACT: A consistent clinical feature of amyotrophic lateral sclerosis (ALS) is the sparing of eye movements. Pathological studies have confirmed that there is relative sparing of the cranial motor nuclei of the oculomotor, trochlear and abducens nerves, although pathological changes resembling those seen in anterior horn cells are present to a lesser degree. The aim of the present study is to combine LCM and microarray analysis to study the differences between motor neurons that are selectively resistant (oculomotor neurons) and those that are vulnerable (lumbar spinal motor neurons) to the disease process in amyotrophic lateral sclerosis. We used microarray analysis to determine the differences in gene expression between oculomotor and lumbar spinal motor neurons, isolated by laser capture microdissection from the midbrain and spinal cord of neurologically normal human controls.

ORGANISM(S): Homo sapiens

SUBMITTER: Paul Heath

PROVIDER: E-GEOD-40438 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Gene expression profiling of resistant and vulnerable motor neuron subtypes in amyotrophic lateral sclerosis

Project description:A consistent clinical feature of amyotrophic lateral sclerosis (ALS) is the sparing of eye movements. Pathological studies have confirmed that there is relative sparing of the cranial motor nuclei of the oculomotor, trochlear and abducens nerves, although pathological changes resembling those seen in anterior horn cells are present to a lesser degree. The aim of the present study is to combine LCM and microarray analysis to study the differences between motor neurons that are selectively resistant (oculomotor neurons) and those that are vulnerable (lumbar spinal motor neurons) to the disease process in amyotrophic lateral sclerosis.

2013-03-01 | GSE40438 | GEO

Gene expression signatures in motor neuron disease fibroblasts reveal dysregulation of metabolism, hypoxia-response and RNA processing functions

Project description:Amyotrophic lateral sclerosis and primary lateral sclerosis are two syndromic variants within the motor neurone disease spectrum. Whilst primary lateral sclerosis is associated with loss of upper motor neurons and a more benign disease course up to 17yrs, amyotrophic lateral sclerosis is caused by loss of both upper and lower motor neurons and has an average disease course of 2-3 years. The majority of cases are sporadic, thereby limiting the availability of cellular models for investigating pathogenic disease mechanisms. The aim of the present study was to evaluate fibroblasts as a cellular model for sporadic amyotrophic lateral sclerosis and primary lateral sclerosis, to establish whether disease-related dysregulated biological processes recapitulate those seen in the central nervous system and to elucidate pathways that distinguish between the two disease phenotypes. We used microarray analysis to determine the differences in gene expression between fibroblasts derived from skin biopsies taken from sporadic amyotrophic lateral sclerosis and primary lateral sclerosis neurologically normal human controls

2014-04-16 | E-GEOD-56808 | biostudies-arrayexpress

RNAseq of laser captured spinal and oculomotor neurons in post mortem material of ALS patients

Project description:Gene expression profiles of specific neuronal populations might explain differential vulnerability to neurodegeneration in the lethal disease amyotrophic lateral sclerosis (ALS). Using laser capture microscopy (LCM) and RNA sequencing (LCM-seq), we demonstrate that the molecular signature of degeneration-resistant oculomotor neurons (OMNs) is distinct from that of vulnerable spinal motor neurons (MNs).

2020-02-14 | GSE115130 | GEO

RNAseq of oculomotor (OMN) and spinal motor neurons (SC) derived from mouse embryonic stem cells and purified by FACS

Project description:Oculomotor neurons, which regulate eye movement, are resilient to degeneration in the lethal motor neuron disease amyotrophic lateral sclerosis (ALS). It would be highly advantageous if motor neuron resilience could be modeled in vitro. Towards this goal, we generated a high proportion of oculomotor neurons from mouse embryonic stem cells through temporal overexpression of Phox2a in neuronal progenitors. We demonstrate, using immunocytochemistry and RNA sequencing, that in vitro generated neurons are bona fide oculomotor neurons based on their similarity to their in vivo counterpart in rodent and man. We also show that in vitro generated oculomotor neurons display a robust activation of survival-promoting Akt signaling and are more resilient to the ALS-like toxicity of kainic acid than spinal motor neurons. Thus, we can generate bona fide oculomotor neurons in vitro which display a resilience similar to that seen in vivo.

2019-05-09 | GSE118620 | GEO

RNA-seq of laser captured oculomotor, cervical and lumbar spinal motor, and Onufs nucleus motor neurons in post mortem material of control human subjects

Project description:Oculomotor neurons, which regulate eye movement, are resilient to degeneration in the lethal motor neuron disease amyotrophic lateral sclerosis (ALS). It would be highly advantageous if motor neuron resilience could be modeled in vitro. Towards this goal, we generated a high proportion of oculomotor neurons from mouse embryonic stem cells through temporal overexpression of Phox2a in neuronal progenitors. We demonstrate, using electrophysiology, immunocytochemistry and RNA sequencing, that in vitro generated neurons are bona fide oculomotor neurons based on their similarity to their in vivo counterpart in rodent and man. We also show that in vitro generated oculomotor neurons display a robust activation of survival-promoting Akt signaling and are more resilient to the ALS-like toxicity of kainic acid than spinal motor neurons. Thus, we can generate bona fide oculomotor neurons in vitro which display a resilience similar to that seen in vivo.

2019-05-09 | GSE93939 | GEO

Co-culture of iPSC-derived microglia and spinal motor neurons as a novel model for amyotrophic lateral sclerosis

Project description:Amyotrophic lateral sclerosis is a fatal neurodegenerative disorder primarily characterized by motor neuron degeneration with additional involvement of non-neuronal cells, in particular, microglia. In our previous work, we have established protocols for the differentiation of iPSC-derived spinal motor neurons and microglia. Here, we combine both cell lineages and establish a novel co-culture of iPSC-derived motor neurons and microglia, which is compatible with motor neuron identity and function. Co-cultured microglia express key microglial markers and transcriptomically resemble primary human microglia, have highly dynamic ramifications, are phagocytic, release various cytokines and respond to stimulation. Further, they express key amyotrophic lateral sclerosis-associated genes and release disease-relevant biomarkers. This novel and authentic human model system facilitates the study of physiological motor neuron-microglia crosstalk and permits the investigation of non-cell-autonomous phenotypes in amyotrophic lateral sclerosis.

2022-07-24 | GSE200037 | GEO

Comparison of gene expression in motor pools with differential vulnerability in ALS

Project description:ALS is a uniformly fatal neurodegenerative disease in which motor neurons in the spinal cord and brain stem are selectively lost. Individual motor - groups of motor neurons innervating single muscles - show widely varying degrees of disease resistance: in the final stages of ALS, nearly all voluntary movement is lost but eye movement and eliminative and sexual functions remain relatively unimpaired. These functions are controlled by motor neurons of the oculomotor (III), trochlear (IV) and abducens (VI) nuclei in the midbrain and brainstem, and by Onuf’s nucleus in the lumbosacral spinal cord, respectively. Correspondingly, in ALS autopsies the oculomotor and Onuf’s nuclei are almost completely preserved. We used microarray profiling of isolated wildtype mouse motor neurons to identify genes whose expression was characteristic of both oculomotor and Onuf’s nuclei but not of vulnerable lumbar spinal neurons, or vice versa.

2013-11-15 | GSE52118 | GEO

Gene expression signatures in motor neuron disease fibroblasts reveal dysregulation of metabolism, hypoxia-response and RNA processing functions

2014-04-16 | GSE56808 | GEO

Age alters the gene expression profiles of rat oculomotor nucleus and spinal cord

Project description:Some of the most common neurodegenerative disorders have a strong association with aging. Curiously, these conditions tend to involve specific areas of the central and peripheral nervous system, at least initially. For example, amyotrophic lateral sclerosis preferentially targets spinal motor neurons and spares oculomotor neurons. This suggests that aging has divergent effects on brain stem and spinal cord, a factor that may explain differential susceptibility to certain neurodegenerative disorders. Therefore, for this study we asked whether aging induces a change in the evolution of the transcriptome of oculomotor nucleus (OMN) throughout the lifespan which would be different than that of the spinal cord aging. Age induced a transcriptome shift to a pattern consistent with inflammation/immune response in both OMN and spinal cord. However, the response was more dramatic in the spinal cord. On the other hand, age repressed genes coding ion channels and transporters in OMN and cytoskeletal proteins in the spinal cord. The results indicate that OMN and spinal cord transcriptomes change differently with age. The results give an initial glimpse of how tissue-specific gene expression patterns may underlie the differential susceptibility to age-related neuromuscular dysfunction. Keywords: time course, aging

2005-09-17 | GSE3305 | GEO

Transcriptome and proteome profiling of motor neuron model subjected to protein misfolding stress

Project description:To identify molecular differences between closely related motor neurons relevant for understanding of neurodegenerative diseases, such as Amyotrophic Lateral Sclerosis (ALS), we exploited induced cranial and spinal motor neuron populations from mouse embryonic stem cells. We performed a large scale time-dependent TMT-based proteomics analysis in the presence and absence of protein misfolding stress. We profiled ~8,600 proteins from two different motor neuron cell lines, measured in two replicates and three time points.

2024-03-06 | PXD018070 | Pride

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