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Superoxide dismutas...
Superoxide dismutase-1 and other proteins in inclusions from transgenic amyotrophic lateral sclerosis model mice
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- Bergemalm, Daniel, 1977- (författare)
- Umeå universitet,Klinisk kemi
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- Forsberg, Karin (författare)
- Umeå universitet,Patologi
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- Srivastava, Vaibhav (författare)
- Sveriges lantbruksuniversitet
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visa fler...
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- Graffmo, Karin S. (författare)
- Umeå universitet,Patologi
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- Andersen, Peter M. (författare)
- Umeå universitet,Neurologi
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- Brännström, Thomas (författare)
- Umeå universitet,Patologi
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- Wingsle, Gunnar (författare)
- Sveriges lantbruksuniversitet
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- Marklund, Stefan L. (författare)
- Umeå universitet,Klinisk kemi
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visa färre...
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(creator_code:org_t)
- Engelska.
- Relaterad länk:
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https://urn.kb.se/re...
Abstract
Ämnesord
Stäng
- Mutant superoxide dismutase-1 (SOD1) causes amyotrophic lateral sclerosis (ALS) through a cytotoxic mechanism of unknown nature. A hallmark in ALS patients and transgenic mouse models carrying human SOD1 (hSOD1) mutations are hSOD1-immunoreactive inclusions in spinal cord ventral horns. The hSOD1 inclusions may block essential cellular functions or cause toxicity through sequestering of other proteins. Inclusions from 4 different transgenic mouse models were examined after density gradient ultracentrifugation. The inclusions are complex structures with heterogeneous densities and are disrupted by detergents. The aggregated hSOD1 was mainly composed of subunits that lacked the native stabilizing intrasubunit disulfide bond. A proportion of subunits formed hSOD1 oligomers or was bound to other proteins through disulfide bonds. Dense inclusions could be isolated and the protein composition was analyzed using proteomic techniques. Mutant hSOD1 accounted for half of the protein. Ten other proteins were identified. Two were cytoplasmic chaperones, 4 were cytoskeletal proteins, and 4 were proteins that normally reside in the endoplasmic reticulum (ER). The presence of ER proteins in inclusions containing the primarily cytosolic hSOD1 further supports the notion that ER stress is involved in ALS.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Annan klinisk medicin (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Other Clinical Medicine (hsv//eng)
Nyckelord
- Amyotrophic lateral sclerosis
- Superoxide dismutase-1
- transgenic mice
- inclusion
- proteomics
- endoplasmic reticulum
- Clinical chemistry
- Klinisk kemi
- medicin
- Medicine
- neurologi
- Neurology
- biokemi
- Biochemistry
Publikations- och innehållstyp
- vet (ämneskategori)
- ovr (ämneskategori)