ADAMTS-4 promotes neurodegeneration in a mouse model of amyotrophic lateral sclerosis

Molecular Neurodegeneration
Sighild LemarchantJari Koistinaho

Abstract

A disintegrin and metalloproteinase with thrombospondin motifs (ADAMTS) proteoglycanases are specialized in the degradation of chondroitin sulfate proteoglycans and participate in mechanisms mediating neuroplasticity. Despite the beneficial effect of ADAMTS-4 on neurorepair after spinal cord injury, the functions of ADAMTS proteoglycanases in other CNS disease states have not been studied. Therefore, we investigated the expression, effects and associated mechanisms of ADAMTS-4 during amyotrophic lateral sclerosis (ALS) in the SOD1(G93A) mouse model. ADAMTS-4 expression and activity were reduced in the spinal cord of SOD1(G93A) mice at disease end-stage when compared to WT littermates. To counteract the loss of ADAMTS-4, SOD1(G93A) and WT mice were treated with saline or a recombinant ADAMTS-4 before symptom onset. Administration of ADAMTS-4 worsened the prognosis of SOD1(G93A) mice by accelerating clinical signs of neuromuscular dysfunctions. The worsened prognosis of ADAMTS-4-treated SOD1(G93A) mice was accompanied by increased degradation of perineuronal nets enwrapping motoneurons and increased motoneuron degeneration in the lumbar spinal cord. Motoneurons of ADAMTS-4-treated SOD1(G93A) mice were more vulnerable to degenerat...Continue Reading

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Citations

Feb 10, 2016·Molecular Neurodegeneration·Sighild LemarchantJari Koistinaho
Jun 16, 2016·Glia·Sighild LemarchantJari Koistinaho
Jan 14, 2017·Journal of Neuroscience Research·Sighild LemarchantJari Koistinaho
Mar 11, 2020·Biomolecules·Yamina MohamediAlvaro J Obaya
Jun 15, 2019·Cellular and Molecular Life Sciences : CMLS·Santiago RiveraKévin Baranger
May 2, 2020·International Journal of Molecular Sciences·Roberta De GioiaStefania Corti
Aug 21, 2021·Cellular & Molecular Immunology·Joshua D CrapserKim N Green

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Methods Mentioned

BETA
nuclear translocation
genotyping
ELISA
PCR

Software Mentioned

GraphPad Prism
Pro Premier
Soft Imaging
GraphPad
Zen 2009 Image Analysis
Statview
Image
Pro Plus
GraphPad Quickcalcs

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