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Literature DB >> 18344992

ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration.

Zhihui Zhong¹, Rashid Deane, Zarina Ali, Margaret Parisi, Yuriy Shapovalov, M Kerry O'Banion, Konstantin Stojanovic, Abhay Sagare, Severine Boillee, Don W Cleveland, Berislav V Zlokovic.

Abstract

We report here that amyotrophic lateral sclerosis-linked superoxide dismutase 1 (SOD1) mutants with different biochemical characteristics disrupted the blood-spinal cord barrier in mice by reducing the levels of the tight junction proteins ZO-1, occludin and claudin-5 between endothelial cells. This resulted in microhemorrhages with release of neurotoxic hemoglobin-derived products, reductions in microcirculation and hypoperfusion. SOD1 mutant-mediated endothelial damage accumulated before motor neuron degeneration and the neurovascular inflammatory response occurred, indicating that it was a central contributor to disease initiation.

Entities: Disease Gene Species

Mesh：

Substances：

Year: 2008 PMID： 18344992 PMCID： PMC2895310 DOI： 10.1038/nn2073

Source DB: PubMed Journal: Nat Neurosci ISSN： 1097-6256 Impact factor: 24.884

15 in total

Review 1. Neurovascular mechanisms of Alzheimer's neurodegeneration.

Authors: Berislav V Zlokovic
Journal: Trends Neurosci Date: 2005-04 Impact factor: 13.837

2. Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.

Authors: P Pasinelli; D R Borchelt; M K Houseweart; D W Cleveland; R H Brown
Journal: Proc Natl Acad Sci U S A Date: 1998-12-22 Impact factor: 11.205

3. Axonal damage markers in cerebrospinal fluid are increased in ALS.

Authors: J Brettschneider; A Petzold; S D Süssmuth; A C Ludolph; H Tumani
Journal: Neurology Date: 2006-03-28 Impact factor: 9.910

Review 4. Microglia as potential contributors to motor neuron injury in amyotrophic lateral sclerosis.

Authors: Siranush A Sargsyan; Peter N Monk; Pamela J Shaw
Journal: Glia Date: 2005-09 Impact factor: 7.452

5. Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration.

Authors: B Oosthuyse; L Moons; E Storkebaum; H Beck; D Nuyens; K Brusselmans; J Van Dorpe; P Hellings; M Gorselink; S Heymans; G Theilmeier; M Dewerchin; V Laudenbach; P Vermylen; H Raat; T Acker; V Vleminckx; L Van Den Bosch; N Cashman; H Fujisawa; M R Drost; R Sciot; F Bruyninckx; D J Hicklin; C Ince; P Gressens; F Lupu; K H Plate; W Robberecht; J M Herbert; D Collen; P Carmeliet
Journal: Nat Genet Date: 2001-06 Impact factor: 38.330

6. Toxic effect of hemoglobin on spinal cord neurons in culture.

Authors: R F Regan; Y Guo
Journal: J Neurotrauma Date: 1998-08 Impact factor: 5.269

7. VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death.

Authors: Diether Lambrechts; Erik Storkebaum; Masafumi Morimoto; Jurgen Del-Favero; Frederik Desmet; Stefan L Marklund; Sabine Wyns; Vincent Thijs; Jörgen Andersson; Ingrid van Marion; Ammar Al-Chalabi; Stephanie Bornes; Rhiannon Musson; Valerie Hansen; Lars Beckman; Rolf Adolfsson; Hardev Singh Pall; Hervé Prats; Severine Vermeire; Paul Rutgeerts; Shigehiro Katayama; Takuya Awata; Nigel Leigh; Loïc Lang-Lazdunski; Mieke Dewerchin; Christopher Shaw; Lieve Moons; Robert Vlietinck; Karen E Morrison; Wim Robberecht; Christine Van Broeckhoven; Désiré Collen; Peter M Andersen; Peter Carmeliet
Journal: Nat Genet Date: 2003-08 Impact factor: 38.330

8. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation.

Authors: M E Gurney; H Pu; A Y Chiu; M C Dal Canto; C Y Polchow; D D Alexander; J Caliendo; A Hentati; Y W Kwon; H X Deng
Journal: Science Date: 1994-06-17 Impact factor: 47.728

9. Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF.

Authors: San Pun; Alexandre Ferrão Santos; Smita Saxena; Lan Xu; Pico Caroni
Journal: Nat Neurosci Date: 2006-02-12 Impact factor: 24.884

10. Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS.

Authors: E D Hall; J A Oostveen; M E Gurney
Journal: Glia Date: 1998-07 Impact factor: 7.452

172 in total

Review 1. Blood-Brain Barrier Driven Pharmacoresistance in Amyotrophic Lateral Sclerosis and Challenges for Effective Drug Therapies.

Authors: Loqman A Mohamed; Shashirekha Markandaiah; Silvia Bonanno; Piera Pasinelli; Davide Trotti
Journal: AAPS J Date: 2017-08-04 Impact factor: 4.009

ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration.

Review 1. Neurovascular mechanisms of Alzheimer's neurodegeneration.

2. Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.

3. Axonal damage markers in cerebrospinal fluid are increased in ALS.

Review 4. Microglia as potential contributors to motor neuron injury in amyotrophic lateral sclerosis.

5. Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration.

6. Toxic effect of hemoglobin on spinal cord neurons in culture.

7. VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death.

8. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation.

9. Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF.

10. Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS.

Review 1. Blood-Brain Barrier Driven Pharmacoresistance in Amyotrophic Lateral Sclerosis and Challenges for Effective Drug Therapies.

2. Advances in the application of MRI to amyotrophic lateral sclerosis.

Review 3. Neurovascular pathways to neurodegeneration in Alzheimer's disease and other disorders.

Review 4. Unique biology of gliomas: challenges and opportunities.

5. Neurodegeneration and the neurovascular unit.

Review 6. Targeting the neurovascular unit for treatment of neurological disorders.

7. Immunological aspects in amyotrophic lateral sclerosis.

8. VEGF-mediated disruption of endothelial CLN-5 promotes blood-brain barrier breakdown.

9. Self-assembling vascular endothelial growth factor nanoparticles improve function in spinocerebellar ataxia type 1.

Review 10. Brain metabolism in health, aging, and neurodegeneration.