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Home › Dataset Library › Transcription profiling by array of fibroblasts derived from skin biopsies taken from sporadic amyotrophic lateral sclerosis and primary...

Dataset: Transcription profiling by array of fibroblasts derived from skin biopsies taken from sporadic amyotrophic lateral sclerosis and primary lateral sclerosis neurologically normal human controls

Amyotrophic lateral sclerosis and primary lateral sclerosis are two syndromic variants within the motor neurone disease spectrum. Whilst...

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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

Species:
human

Samples:
18

Source:
E-GEOD-56808

Updated:
Dec.12, 2014

Registered:
Sep.21, 2014


Factors: (via ArrayExpress)
Sample disease
GSM1369642 normal
GSM1369642 normal
GSM1369642 normal
GSM1369642 normal
GSM1369642 normal
GSM1369642 normal
GSM1369648 Primary lateral sclerosis
GSM1369648 Primary lateral sclerosis
GSM1369650 sporadic amyotrophic lateral sclerosis
GSM136965 Upper Motor Neuron Disease
GSM1369650 sporadic amyotrophic lateral sclerosis
GSM1369648 Primary lateral sclerosis
GSM1369650 sporadic amyotrophic lateral sclerosis
GSM1369648 Primary lateral sclerosis
GSM1369650 sporadic amyotrophic lateral sclerosis
GSM1369650 sporadic amyotrophic lateral sclerosis
GSM1369650 sporadic amyotrophic lateral sclerosis
GSM1369648 Primary lateral sclerosis

Tags

  • amyotrophic lateral sclerosis
  • central
  • disease
  • lateral
  • lateral sclerosis
  • nervous system
  • skin

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