Sökning: onr:"swepub:oai:gup.ub.gu.se/303474" > Neurofilament light...
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000 | 03967naa a2200721 4500 | |
001 | oai:gup.ub.gu.se/303474 | |
003 | SwePub | |
008 | 240528s2021 | |||||||||||000 ||eng| | |
024 | 7 | a https://gup.ub.gu.se/publication/3034742 URI |
024 | 7 | a https://doi.org/10.1038/s41467-021-22114-22 DOI |
040 | a (SwePub)gu | |
041 | a eng | |
042 | 9 SwePub | |
072 | 7 | a ref2 swepub-contenttype |
072 | 7 | a art2 swepub-publicationtype |
100 | 1 | a Weinhofer, Isabelle4 aut |
245 | 1 0 | a Neurofilament light chain as a potential biomarker for monitoring neurodegeneration in X-linked adrenoleukodystrophy. |
264 | c 2021-03-22 | |
264 | 1 | b Springer Science and Business Media LLC,c 2021 |
520 | a X-linked adrenoleukodystrophy (X-ALD), the most frequent monogenetic disorder of brain white matter, is highly variable, ranging from slowly progressive adrenomyeloneuropathy (AMN) to life-threatening inflammatory brain demyelination (CALD). In this study involving 94 X-ALD patients and 55 controls, we tested whether plasma/serum neurofilament light chain protein (NfL) constitutes an early distinguishing biomarker. In AMN, we found moderately elevated NfL with increased levels reflecting higher grading of myelopathy-related disability. Intriguingly, NfL was a significant predictor to discriminate non-converting AMN from cohorts later developing CALD. In CALD, markedly amplified NfL levels reflected brain lesion severity. In rare cases, atypically low NfL revealed a previously unrecognized smoldering CALD disease course with slowly progressive myelin destruction. Upon halt of brain demyelination by hematopoietic stem cell transplantation, NfL gradually normalized. Together, our study reveals that blood NfL reflects inflammatory activity and progression in CALD patients, thus constituting a potential surrogate biomarker that may facilitate clinical decisions and therapeutic development. | |
650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Medicinska och farmaceutiska grundvetenskaperx Neurovetenskaper0 (SwePub)301052 hsv//swe |
650 | 7 | a MEDICAL AND HEALTH SCIENCESx Basic Medicinex Neurosciences0 (SwePub)301052 hsv//eng |
653 | a Adolescent | |
653 | a Adrenoleukodystrophy | |
653 | a diagnosis | |
653 | a metabolism | |
653 | a Adult | |
653 | a Biomarkers | |
653 | a blood | |
653 | a metabolism | |
653 | a Child | |
653 | a Cohort Studies | |
653 | a Disease Progression | |
653 | a Humans | |
653 | a Intermediate Filaments | |
653 | a metabolism | |
653 | a Middle Aged | |
653 | a Nerve Degeneration | |
653 | a diagnosis | |
653 | a metabolism | |
653 | a Neurofilament Proteins | |
653 | a blood | |
653 | a metabolism | |
700 | 1 | a Rommer, Paulus4 aut |
700 | 1 | a Zierfuss, Bettina4 aut |
700 | 1 | a Altmann, Patrick4 aut |
700 | 1 | a Foiani, Martha4 aut |
700 | 1 | a Heslegrave, Amanda4 aut |
700 | 1 | a Zetterberg, Henrik,d 1973u Gothenburg University,Göteborgs universitet,Institutionen för neurovetenskap och fysiologi, sektionen för psykiatri och neurokemi,Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry4 aut0 (Swepub:gu)xzethe |
700 | 1 | a Gleiss, Andreas4 aut |
700 | 1 | a Musolino, Patricia L4 aut |
700 | 1 | a Gong, Yi4 aut |
700 | 1 | a Forss-Petter, Sonja4 aut |
700 | 1 | a Berger, Thomas4 aut |
700 | 1 | a Eichler, Florian4 aut |
700 | 1 | a Aubourg, Patrick4 aut |
700 | 1 | a Köhler, Wolfgang4 aut |
700 | 1 | a Berger, Johannes4 aut |
710 | 2 | a Göteborgs universitetb Institutionen för neurovetenskap och fysiologi, sektionen för psykiatri och neurokemi4 org |
773 | 0 | t Nature communicationsd : Springer Science and Business Media LLCg 12:1q 12:1x 2041-1723 |
856 | 4 | u https://www.nature.com/articles/s41467-021-22114-2.pdf |
856 | 4 8 | u https://gup.ub.gu.se/publication/303474 |
856 | 4 8 | u https://doi.org/10.1038/s41467-021-22114-2 |
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