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Cited by 51Neurogenetic and Muscular Disorders ResearchAmyotrophic Lateral Sclerosis ResearchHereditary Neurological Disorders

Protein expression changes in spinal muscular atrophy revealed with a novel antibody array technology

Anderson, K.·2003·Brain
DOI 10.1093/brain/awg208
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Abstract

Autosomal recessive proximal spinal muscular atrophy (SMA) is a severe neurodegenerative disease of childhood causing weakness and wasting secondary to motor neuron dysfunction. Over 97% of cases are caused by deletions or mutations within the survival motor neuron (SMN) gene. The SMN protein is highly expressed within brain, spinal cord and muscle, and is decreased in SMA patients. It has been shown to have an important role in RNA metabolism, but the reason for the specific motor neuron loss is still unclear. We have used a novel antibody array technology to look for differences in the expression patterns of primary muscle cultures from a type II SMA patient and a normal control. A relatively small number of differences were found within a group of proteins that function as both RNA binding proteins and transcription factors. Interactions between a number of these proteins are well established, and three of them bind in turn to p53 which interacts with SMN. A number of the changes were confirmed with western blot analysis both in the primary muscle cultures and in skeletal muscle samples from SMA patients and controls. Changes at the mRNA level were also confirmed with oligonucleotide arrays. These results suggest that a common transcription pathway may be altered in the disease state, and suggests that down-regulation of transcription factors contributes to SMA pathogenesis.

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Crossref · 10.1093/brain/awg208

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  • The Clontech antibody array has been used in a variety of settings since 2003.

    “The Clontech antibody array has been used in a variety of settings since 2003 (Anderson, et al., 2003,Hatjiharissi, et al., 2007).”

    From Cellular, synaptic, and biochemical features of resilient cognition in Alzheimer’s disease (2013) · cites this paper as Anderson, et al., 2003

  • Independent publications report very high validity of differential protein expression on the Clontech antibody array, verified with Western blotting, and our preliminary findings were consistent with this.

    “Independent publications report very high validity of differential protein expression, verified with Western blotting, and our preliminary study findings were consistent with this.”

    From Cellular, synaptic, and biochemical features of resilient cognition in Alzheimer’s disease (2013) · cites this paper as Anderson, et al., 2003

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Research works printed but not linked: 2

  1. Braun S, Croizat B, Lagrange MC, Poindron P, Warter JM. Degeneration of cocultures of spinal muscular atrophy muscle cells and rat spinal cord explants is not due to secreted factors and cannot be prevented by neurotrophins. Muscle Nerve 1997; 20: 953±60.
  2. Hua Xi Yi Ke Da Xue Xue Bao 1999; 30: 318±20, 323.

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Authors
Anderson, K.
Affiliations
University of Oxford (GB)
Corresponding author
Anderson, K.
ORCID
Anderson, K.
Venue
Brain
Publisher
Oxford University Press (OUP)
Published
2003-09-01
Volume
126
Issue
9
Pages
2052-2064
ISSN
  • 0006-8950 (ISSN-L)
  • 1460-2156
DOI
10.1093/brain/awg208
OpenAlex
W2060036094
Cited by
51
References
43
Citations by year
2026: 1 · 2025: 1 · 2023: 1 · 2021: 2 · 2018: 2 · 2017: 1 · 2016: 2 · 2015: 1 · 2014: 1 · 2013: 2 · 2012: 2
FWCI
2.64
Type
journal article
Language
en
Open access
closed
Locations
  • Brain (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • Wellcome Trust
  • Muscular Dystrophy UK
Topics
  • Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords
spinal muscular atrophy, survival motor neuron protein, SMN gene, protein expression profiling, antibody arrays, RNA-binding proteins, transcription factors, p53, motor neuron degeneration, skeletal muscle, transcriptional pathways
MeSH terms
Adolescent, Adult, Cells, Cultured, Female, Humans, Immunoassay, Male, Models, Biological, Muscle Proteins, Muscular Atrophy, Spinal, Nerve Tissue Proteins, RNA, Messenger, Blotting, Western, Gene Expression, RNA-Binding Proteins, Cyclic AMP Response Element-Binding Protein, Muscle, Skeletal, Oligonucleotide Array Sequence Analysis, Proteomics, SMN Complex Proteins

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