SwePub
Sök i LIBRIS databas

  Extended search

WFRF:(Brännström Thomas)
 

Search: WFRF:(Brännström Thomas) > (2000-2004) > Minute quantities o...

Minute quantities of misfolded mutant superoxide dismutase-1 cause amyotrophic lateral sclerosis

Jonsson, Andreas P. (author)
Umeå universitet,Klinisk kemi
Ernhill, Karin (author)
Umeå universitet,Patologi
Andersen, Peter M (author)
Umeå universitet,Neurologi
show more...
Bergemalm, Daniel (author)
Umeå universitet,Klinisk kemi
Brännström, Thomas (author)
Umeå universitet,Patologi
Gredal, Ole (author)
Nilsson, Peter (author)
Marklund, Stefan L (author)
Umeå universitet,Klinisk kemi
show less...
 (creator_code:org_t)
2004-01
2004
English.
In: Brain. - : Oxford University Press. - 0006-8950 .- 1460-2156. ; 127:Pt 1, s. 73-88
  • Journal article (peer-reviewed)
Abstract Subject headings
Close  
  • Mutant forms of superoxide dismutase-1 (SOD1) cause amyotrophic lateral sclerosis (ALS) by an unknown noxious mechanism. Using an antibody against a novel epitope in the G127insTGGG mutation, mutant SOD1 was studied for the first time in spinal cord and brain of an ALS patient. The level was below 0.5% of the SOD1 level in controls. In corresponding transgenic mice the content of mutant SOD1 was also low, although it was enriched in spinal cord and brain compared with other tissues. In the mice the misfolded mutant SOD1 aggregated rapidly and 20% occurred in steady state as detergent-soluble protoaggregates. The misfolded SOD1 and the protoaggregates form, from birth until death, a potentially noxious burden that may induce the motor neuron injury. Detergent-resistant aggregates, as well as inclusions of mutant SOD1 in motor neurons and astrocytes, accumulated in spinal cord ventral horns of the patient and mice with terminal disease. The inclusions and aggregates may serve as terminal markers of long-term assault by misfolded SOD1 and protoaggregates.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Medicinska och farmaceutiska grundvetenskaper -- Neurovetenskaper (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Basic Medicine -- Neurosciences (hsv//eng)

Keyword

aggregation
transgenic mice
SOD
motor neuron disease

Publication and Content Type

ref (subject category)
art (subject category)

Find in a library

  • Brain (Search for host publication in LIBRIS)

To the university's database

Search outside SwePub

Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.

 
pil uppåt Close

Copy and save the link in order to return to this view