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We are analyzing https://link.springer.com/article/10.1007/s00401-012-1039-8.

Title:
Blood–spinal cord barrier breakdown and pericyte reductions in amyotrophic lateral sclerosis | Acta Neuropathologica
Description:
The blood–brain barrier and blood–spinal cord barrier (BSCB) limit the entry of plasma components and erythrocytes into the central nervous system (CNS). Pericytes play a key role in maintaining blood–CNS barriers. The BSCB is damaged in patients with amyotrophic lateral sclerosis (ALS). Moreover, transgenic ALS rodents and pericyte-deficient mice develop BSCB disruption with erythrocyte extravasation preceding motor neuron dysfunction. Here, we studied whether BSCB disruption with erythrocyte extravasation and pericyte loss are present in human ALS. We show that 11 of 11 cervical cords from ALS patients, but 0 of 5 non-neurodegenerative disorders controls, possess perivascular deposits of erythrocyte-derived hemoglobin and hemosiderin typically 10–50 Îźm in diameter suggestive of erythrocyte extravasation. Immunostaining for CD235a, a specific marker for erythrocytes, confirmed sporadic erythrocyte extravasation in ALS, but not controls. Quantitative analysis revealed a 3.1-fold increase in perivascular hemoglobin deposits in ALS compared to controls showing hemoglobin confined within the vascular lumen, which correlated with 2.5-fold increase in hemosiderin deposits (r = 0.82, p < 0.01). Spinal cord parenchymal accumulation of plasma-derived immunoglobulin G, fibrin and thrombin was demonstrated in ALS, but not controls. Immunostaining for platelet-derived growth factor receptor-β, a specific marker for CNS pericytes, indicated a 54 % (p < 0.01) reduction in pericyte number in ALS patients compared to controls. Pericyte reduction correlated negatively with the magnitude of BSCB damage as determined by hemoglobin abundance (r = âˆ’0.75, p < 0.01). Thus, the BSCB disruption with erythrocyte extravasation and pericyte reductions is present in ALS. Whether similar findings occur in motor cortex and affected brainstem motor nuclei remain to be seen.
Website Age:
28 years and 1 months (reg. 1997-05-29).

Matching Content Categories {📚}

  • Education
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Content Management System {📝}

What CMS is link.springer.com built with?

Custom-built

No common CMS systems were detected on Link.springer.com, and no known web development framework was identified.

Traffic Estimate {📈}

What is the average monthly size of link.springer.com audience?

🌠 Phenomenal Traffic: 5M - 10M visitors per month


Based on our best estimate, this website will receive around 5,000,019 visitors per month in the current month.
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How Does Link.springer.com Make Money? {💸}

We can't figure out the monetization strategy.

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Keywords {🔍}

article, google, scholar, pubmed, cord, cas, spinal, motor, lateral, amyotrophic, sclerosis, pericyte, subjects, hemoglobin, analysis, bscb, deposits, human, barrier, patients, cervical, hemosiderin, nndc, sections, neuron, sporadic, anterior, horn, neurol, cases, disruption, extravasation, fig, erythrocytes, pericytes, tissue, perivascular, zlokovic, erythrocyte, usa, mice, vascular, proteins, disease, neurons, capillaries, bloodspinal, controls, injury, study,

Topics {✒️}

blood–spinal cord barrier blood–spinal cord barriers blood–brain barrier integrity chromosome 9p21-linked als-ftd blood–brain barrier multiple plasma-derived proteins blood-gsf barrier article download pdf chromosome 9p-linked ftd goat anti-human podocalyxin bovine anti-goat cy3 z-stack images utilizing pdgf-b-deficient mice utilizing post-image thresholding iron-dependent oxidative injury lectin-positive capillary profiles lenalidomide regulate erythropoiesis controls cerebrovascular integrity maintaining blood–cns barriers amyotrophic lateral sclerosis endothelial cell-specific lectin pdgfrβ-positive surface area detect erythrocyte-derived products spinal cord capillaries open access spinal cord isolation impaired blood–brain pdgfrβ-positive pericyte coverage extravascular erythrocyte-derived hemoglobin perivascular hemosiderin deposits mm2 spinal cord pdgf beta receptor spinal cord tissues immediately snap frozen optimize pulse sequences spinal cord neurons serum-csf protein gradients pdgfrβ-positive pericyte number lumbar spinal cords motor neuron injury full access visualize neun-positive cells protein-bound iron including prussian blue staining human sod1 develop possess perivascular deposits endothelial tight junctions sporadic cd235a-positive erythrocytes rabbit anti-fibrinogen perivascular hemoglobin deposits

Questions {❓}

  • Andersen PM, Al-Chalabi A (2011) Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

Schema {🗺️}

WebPage:
      mainEntity:
         headline:Blood–spinal cord barrier breakdown and pericyte reductions in amyotrophic lateral sclerosis
         description:The blood–brain barrier and blood–spinal cord barrier (BSCB) limit the entry of plasma components and erythrocytes into the central nervous system (CNS). Pericytes play a key role in maintaining blood–CNS barriers. The BSCB is damaged in patients with amyotrophic lateral sclerosis (ALS). Moreover, transgenic ALS rodents and pericyte-deficient mice develop BSCB disruption with erythrocyte extravasation preceding motor neuron dysfunction. Here, we studied whether BSCB disruption with erythrocyte extravasation and pericyte loss are present in human ALS. We show that 11 of 11 cervical cords from ALS patients, but 0 of 5 non-neurodegenerative disorders controls, possess perivascular deposits of erythrocyte-derived hemoglobin and hemosiderin typically 10–50 Οm in diameter suggestive of erythrocyte extravasation. Immunostaining for CD235a, a specific marker for erythrocytes, confirmed sporadic erythrocyte extravasation in ALS, but not controls. Quantitative analysis revealed a 3.1-fold increase in perivascular hemoglobin deposits in ALS compared to controls showing hemoglobin confined within the vascular lumen, which correlated with 2.5-fold increase in hemosiderin deposits (r = 0.82, p < 0.01). Spinal cord parenchymal accumulation of plasma-derived immunoglobulin G, fibrin and thrombin was demonstrated in ALS, but not controls. Immunostaining for platelet-derived growth factor receptor-β, a specific marker for CNS pericytes, indicated a 54 % (p < 0.01) reduction in pericyte number in ALS patients compared to controls. Pericyte reduction correlated negatively with the magnitude of BSCB damage as determined by hemoglobin abundance (r = −0.75, p < 0.01). Thus, the BSCB disruption with erythrocyte extravasation and pericyte reductions is present in ALS. Whether similar findings occur in motor cortex and affected brainstem motor nuclei remain to be seen.
         datePublished:2012-09-01T00:00:00Z
         dateModified:2012-09-01T00:00:00Z
         pageStart:111
         pageEnd:120
         license:https://creativecommons.org/licenses/by/2.0
         sameAs:https://doi.org/10.1007/s00401-012-1039-8
         keywords:
            Amyotrophic lateral sclerosis
            Pericytes
            Vascular injury
            Blood–brain barrier
            Blood–spinal cord barrier
            Pathology
            Neurosciences
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            name:Acta Neuropathologica
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                     address:
                        name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
                        type:PostalAddress
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               name:Jesse D. Sengillo
               affiliation:
                     name:University of Southern California
                     address:
                        name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
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               name:John S. Sullivan
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                     name:University of Southern California
                     address:
                        name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
                        type:PostalAddress
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               name:Jenny S. Henkel
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                     address:
                        name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
                        type:PostalAddress
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               affiliation:
                     name:The Methodist Hospital Research Institute, The Methodist Hospital
                     address:
                        name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
                        type:PostalAddress
                     type:Organization
               type:Person
               name:Berislav V. Zlokovic
               affiliation:
                     name:University of Southern California
                     address:
                        name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
                        type:PostalAddress
                     type:Organization
               email:[email protected]
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ScholarlyArticle:
      headline:Blood–spinal cord barrier breakdown and pericyte reductions in amyotrophic lateral sclerosis
      description:The blood–brain barrier and blood–spinal cord barrier (BSCB) limit the entry of plasma components and erythrocytes into the central nervous system (CNS). Pericytes play a key role in maintaining blood–CNS barriers. The BSCB is damaged in patients with amyotrophic lateral sclerosis (ALS). Moreover, transgenic ALS rodents and pericyte-deficient mice develop BSCB disruption with erythrocyte extravasation preceding motor neuron dysfunction. Here, we studied whether BSCB disruption with erythrocyte extravasation and pericyte loss are present in human ALS. We show that 11 of 11 cervical cords from ALS patients, but 0 of 5 non-neurodegenerative disorders controls, possess perivascular deposits of erythrocyte-derived hemoglobin and hemosiderin typically 10–50 Οm in diameter suggestive of erythrocyte extravasation. Immunostaining for CD235a, a specific marker for erythrocytes, confirmed sporadic erythrocyte extravasation in ALS, but not controls. Quantitative analysis revealed a 3.1-fold increase in perivascular hemoglobin deposits in ALS compared to controls showing hemoglobin confined within the vascular lumen, which correlated with 2.5-fold increase in hemosiderin deposits (r = 0.82, p < 0.01). Spinal cord parenchymal accumulation of plasma-derived immunoglobulin G, fibrin and thrombin was demonstrated in ALS, but not controls. Immunostaining for platelet-derived growth factor receptor-β, a specific marker for CNS pericytes, indicated a 54 % (p < 0.01) reduction in pericyte number in ALS patients compared to controls. Pericyte reduction correlated negatively with the magnitude of BSCB damage as determined by hemoglobin abundance (r = −0.75, p < 0.01). Thus, the BSCB disruption with erythrocyte extravasation and pericyte reductions is present in ALS. Whether similar findings occur in motor cortex and affected brainstem motor nuclei remain to be seen.
      datePublished:2012-09-01T00:00:00Z
      dateModified:2012-09-01T00:00:00Z
      pageStart:111
      pageEnd:120
      license:https://creativecommons.org/licenses/by/2.0
      sameAs:https://doi.org/10.1007/s00401-012-1039-8
      keywords:
         Amyotrophic lateral sclerosis
         Pericytes
         Vascular injury
         Blood–brain barrier
         Blood–spinal cord barrier
         Pathology
         Neurosciences
      image:
         https://media.springernature.com/lw1200/springer-static/image/art%3A10.1007%2Fs00401-012-1039-8/MediaObjects/401_2012_1039_Fig1_HTML.gif
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         name:Acta Neuropathologica
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                     type:PostalAddress
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            type:Person
            name:Jesse D. Sengillo
            affiliation:
                  name:University of Southern California
                  address:
                     name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
                     type:PostalAddress
                  type:Organization
            type:Person
            name:John S. Sullivan
            affiliation:
                  name:University of Southern California
                  address:
                     name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
                     type:PostalAddress
                  type:Organization
            type:Person
            name:Jenny S. Henkel
            affiliation:
                  name:The Methodist Hospital Research Institute, The Methodist Hospital
                  address:
                     name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
                     type:PostalAddress
                  type:Organization
            type:Person
            name:Stanley H. Appel
            affiliation:
                  name:The Methodist Hospital Research Institute, The Methodist Hospital
                  address:
                     name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
                     type:PostalAddress
                  type:Organization
            type:Person
            name:Berislav V. Zlokovic
            affiliation:
                  name:University of Southern California
                  address:
                     name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
                     type:PostalAddress
                  type:Organization
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         name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
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      name:University of Southern California
      address:
         name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
         type:PostalAddress
      name:The Methodist Hospital Research Institute, The Methodist Hospital
      address:
         name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
         type:PostalAddress
      name:The Methodist Hospital Research Institute, The Methodist Hospital
      address:
         name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
         type:PostalAddress
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Person:
      name:Ethan A. Winkler
      affiliation:
            name:University of Southern California
            address:
               name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
               type:PostalAddress
            type:Organization
      name:Jesse D. Sengillo
      affiliation:
            name:University of Southern California
            address:
               name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
               type:PostalAddress
            type:Organization
      name:John S. Sullivan
      affiliation:
            name:University of Southern California
            address:
               name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
               type:PostalAddress
            type:Organization
      name:Jenny S. Henkel
      affiliation:
            name:The Methodist Hospital Research Institute, The Methodist Hospital
            address:
               name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
               type:PostalAddress
            type:Organization
      name:Stanley H. Appel
      affiliation:
            name:The Methodist Hospital Research Institute, The Methodist Hospital
            address:
               name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
               type:PostalAddress
            type:Organization
      name:Berislav V. Zlokovic
      affiliation:
            name:University of Southern California
            address:
               name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
               type:PostalAddress
            type:Organization
      email:[email protected]
PostalAddress:
      name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
      name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
      name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA
      name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
      name:Department of Neurology, Methodist Neurological Institute, The Methodist Hospital Research Institute, The Methodist Hospital, Houston, USA
      name:Center for Neurodegeneration and Regeneration, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, USA

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