Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients

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Fältnamn	Indikatorer	Metadata
000		04039naa a2200481 4500
001		oai:DiVA.org:umu-40739
003		SwePub
008		110308s2010 \| \|\|\|\|\|\|\|\|\|\|\|000 \|\|eng\|
024	7	a https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-407392 URI
024	7	a https://doi.org/10.1371/journal.pone.00115522 DOI
040		a (SwePub)umu
041		a engb eng
042		9 SwePub
072	7	a ref2 swepub-contenttype
072	7	a art2 swepub-publicationtype
100	1	a Forsberg, Karinu Umeå universitet,Patologi,Thomas Brännström4 aut0 (Swepub:umu)nonkin02
245	1 0	a Novel antibodies reveal inclusions containing non-native SOD1 in sporadic ALS patients
264		c 2010-07-14
264	1	b Public library of science,c 2010
338		a electronic2 rdacarrier
520		a Mutations in CuZn-superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS) and are found in 6% of ALS patients. Non-native and aggregation-prone forms of mutant SOD1s are thought to trigger the disease. Two sets of novel antibodies, raised in rabbits and chicken, against peptides spaced along the human SOD1 sequence, were by enzyme-linked immunosorbent assay and an immunocapture method shown to be specific for denatured SOD1. These were used to examine SOD1 in spinal cords of ALS patients lacking mutations in the enzyme. Small granular SOD1-immunoreactive inclusions were found in spinal motoneurons of all 37 sporadic and familial ALS patients studied, but only sparsely in 3 of 28 neurodegenerative and 2 of 19 non-neurological control patients. The granular inclusions were by confocal microscopy found to partly colocalize with markers for lysosomes but not with inclusions containing TAR DNA binding protein-43, ubiquitin or markers for endoplasmic reticulum, autophagosomes or mitochondria. Granular inclusions were also found in carriers of SOD1 mutations and in spinobulbar muscular atrophy (SBMA) patients and they were the major type of inclusion detected in ALS patients homozygous for the wild type-like D90A mutation. The findings suggest that SOD1 may be involved in ALS pathogenesis in patients lacking mutations in the enzyme.
650	7	a MEDICIN OCH HÄLSOVETENSKAPx Medicinsk bioteknologix Medicinsk bioteknologi0 (SwePub)304012 hsv//swe
650	7	a MEDICAL AND HEALTH SCIENCESx Medical Biotechnologyx Medical Biotechnology0 (SwePub)304012 hsv//eng
653		a amyotrophic-lateral-sclerosis; cu/zn superoxide-dismutase; motor-neuron degeneration; molecular pathology; gene mutation; linked SOD1; mutant SOD1; mice; disease; immunoreactivity
653		a MEDICINE
653		a MEDICIN
653		a medicin
653		a Medicine
700	1	a Jonsson, P Andreasu Umeå universitet,Klinisk kemi4 aut0 (Swepub:umu)aasjon95
700	1	a Andersen, Peter Mu Umeå universitet,Neurologi4 aut0 (Swepub:umu)pean0001
700	1	a Bergemalm, Danielu Umeå universitet,Klinisk kemi4 aut0 (Swepub:umu)dalbem99
700	1	a Graffmo, Karin Su Umeå universitet,Patologi4 aut0 (Swepub:umu)kanerl79
700	1	a Hultdin, Magnusu Umeå universitet,Patologi4 aut0 (Swepub:umu)magn0001
700	1	a Jacobsson, Johanu Umeå universitet,Neurologi4 aut0 (Swepub:umu)joja0063
700	1	a Rosquist, Rolandu Umeå universitet,Institutionen för molekylärbiologi (Medicinska fakulteten)4 aut0 (Swepub:umu)roro0002
700	1	a Marklund, Stefan Lu Umeå universitet,Klinisk kemi4 aut0 (Swepub:umu)stma0003
700	1	a Brännström, Thomasu Umeå universitet,Patologi4 aut0 (Swepub:umu)thbr0001
710	2	a Umeå universitetb Patologi4 org
773	0	t PLOS ONEd : Public library of scienceg 5:7, s. e11552-q 5:7<e11552-x 1932-6203
856	4	u https://umu.diva-portal.org/smash/get/diva2:402367/FULLTEXT01.pdfx primaryx Raw objecty fulltext:print
856	4	u https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0011552&type=printable
856	4 8	u https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-40739
856	4 8	u https://doi.org/10.1371/journal.pone.0011552

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Av författaren/redakt...: Forsberg, Karin; Jonsson, P Andre ...; Andersen, Peter ...; Bergemalm, Danie ...; Graffmo, Karin S; Hultdin, Magnus; visa fler...; Jacobsson, Johan; Rosquist, Roland; Marklund, Stefan ...; Brännström, Thom ...; visa färre...

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