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Characteristics of progressive supranuclear palsy presenting with corticobasal syndrome: a cortical variant.

Ling H, Ling H, de Silva R, Massey LA, Courtney R, Hondhamuni G, Bajaj N, Lowe J, Holton JL, Lees A, Revesz T - Neuropathol. Appl. Neurobiol. (2014)

Bottom Line: We identified that there was a shift of tau burden towards the cortical regions away from the basal ganglia; supporting the notion that PSP-CBS is a 'cortical' PSP variant.PSP-CBS also had less severe neuronal loss in the dorsolateral and ventrolateral subregions of the substantia nigra and more severe microglial response in the corticospinal tract than in PSP-RS; however, neuronal loss in subthalamic nucleus was equally severe in both groups.A better understanding of the factors that influence the selective pathological vulnerability in different PSP variants will provide further insights into the neurodegenerative process underlying tauopathies.

View Article: PubMed Central - PubMed

Affiliation: Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, University College London, London, UK; Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, University College London, London, UK; Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK.

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Western blot analysis of sarkosyl-insoluble tau from frontal cortex homogenates of CBD-CBS, PSP-CBS and PSP-RS. The characteristic doublet of predominant 4R-tau pathology was observed in both CBD and PSP cases (arrowheads). CBD-CBS cases have a lower molecular weight doublet consisting of proteolytic fragments at approximately 37 kDa (**); whereas PSP-RS and PSP-CBS cases have a single band at approximately 33 kDa (*). Numbers on the left indicate positions of molecular weight markers (kDa).
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fig06: Western blot analysis of sarkosyl-insoluble tau from frontal cortex homogenates of CBD-CBS, PSP-CBS and PSP-RS. The characteristic doublet of predominant 4R-tau pathology was observed in both CBD and PSP cases (arrowheads). CBD-CBS cases have a lower molecular weight doublet consisting of proteolytic fragments at approximately 37 kDa (**); whereas PSP-RS and PSP-CBS cases have a single band at approximately 33 kDa (*). Numbers on the left indicate positions of molecular weight markers (kDa).

Mentions: Western blots of the sarkosyl-insoluble tau fractions from the frontal cortical homogenates showed the characteristic doublet at 64 and 68 kDa indicating predominant 4R tau in PSP-CBS, PSP-RS and CBD-CBS cases (Figure 6). Our PSP-CBS and PSP-RS cases showed a single band at approximately 33 kDa, whereas the CBD-CBS cases had a doublet at approximately 37 kDa (Figure 6), which are consistent with previous findings on the molecular differences in the low molecular weight proteolytic fragments between CBD and PSP 14,40. There was no significant association between H1/H1 or H1/H2 genotype with either of the PSP subgroups (χ2 test; P = 0.21, Table 1).


Characteristics of progressive supranuclear palsy presenting with corticobasal syndrome: a cortical variant.

Ling H, Ling H, de Silva R, Massey LA, Courtney R, Hondhamuni G, Bajaj N, Lowe J, Holton JL, Lees A, Revesz T - Neuropathol. Appl. Neurobiol. (2014)

Western blot analysis of sarkosyl-insoluble tau from frontal cortex homogenates of CBD-CBS, PSP-CBS and PSP-RS. The characteristic doublet of predominant 4R-tau pathology was observed in both CBD and PSP cases (arrowheads). CBD-CBS cases have a lower molecular weight doublet consisting of proteolytic fragments at approximately 37 kDa (**); whereas PSP-RS and PSP-CBS cases have a single band at approximately 33 kDa (*). Numbers on the left indicate positions of molecular weight markers (kDa).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4260147&req=5

fig06: Western blot analysis of sarkosyl-insoluble tau from frontal cortex homogenates of CBD-CBS, PSP-CBS and PSP-RS. The characteristic doublet of predominant 4R-tau pathology was observed in both CBD and PSP cases (arrowheads). CBD-CBS cases have a lower molecular weight doublet consisting of proteolytic fragments at approximately 37 kDa (**); whereas PSP-RS and PSP-CBS cases have a single band at approximately 33 kDa (*). Numbers on the left indicate positions of molecular weight markers (kDa).
Mentions: Western blots of the sarkosyl-insoluble tau fractions from the frontal cortical homogenates showed the characteristic doublet at 64 and 68 kDa indicating predominant 4R tau in PSP-CBS, PSP-RS and CBD-CBS cases (Figure 6). Our PSP-CBS and PSP-RS cases showed a single band at approximately 33 kDa, whereas the CBD-CBS cases had a doublet at approximately 37 kDa (Figure 6), which are consistent with previous findings on the molecular differences in the low molecular weight proteolytic fragments between CBD and PSP 14,40. There was no significant association between H1/H1 or H1/H2 genotype with either of the PSP subgroups (χ2 test; P = 0.21, Table 1).

Bottom Line: We identified that there was a shift of tau burden towards the cortical regions away from the basal ganglia; supporting the notion that PSP-CBS is a 'cortical' PSP variant.PSP-CBS also had less severe neuronal loss in the dorsolateral and ventrolateral subregions of the substantia nigra and more severe microglial response in the corticospinal tract than in PSP-RS; however, neuronal loss in subthalamic nucleus was equally severe in both groups.A better understanding of the factors that influence the selective pathological vulnerability in different PSP variants will provide further insights into the neurodegenerative process underlying tauopathies.

View Article: PubMed Central - PubMed

Affiliation: Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, University College London, London, UK; Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, University College London, London, UK; Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK.

Show MeSH
Related in: MedlinePlus