Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Single particle cryo-EM dataset of sarkosyl-insoluble fraction from the frontal cortex of an individual with progressive supranuclear palsy

ABSTRACT:

SUBMITTER: Yang Shi

PROVIDER: EMPIAR-10765 | biostudies-other |

REPOSITORIES: biostudies-other

ACCESS DATA

Json Xml

Publications

Structure-based classification of tauopathies.

Shi Yang Y Zhang Wenjuan W Yang Yang Y Murzin Alexey G AG Falcon Benjamin B Kotecha Abhay A van Beers Mike M Tarutani Airi A Kametani Fuyuki F Garringer Holly J HJ Vidal Ruben R Hallinan Grace I GI Lashley Tammaryn T Saito Yuko Y Murayama Shigeo S Yoshida Mari M Tanaka Hidetomo H Kakita Akiyoshi A Ikeuchi Takeshi T Robinson Andrew C AC Mann David M A DMA Kovacs Gabor G GG Revesz Tamas T Ghetti Bernardino B Hasegawa Masato M Goedert Michel M Scheres Sjors H W SHW

Nature 20210929 7880

The ordered assembly of tau protein into filaments characterizes several neurodegenerative diseases, which are called tauopathies. It was previously reported that, by cryo-electron microscopy, the structures of tau filaments from Alzheimer's disease1,2, Pick's disease3, chronic traumatic encephalopathy4 and corticobasal degeneration5 are distinct. Here we show that the structures of tau filaments from progressive supranuclear palsy (PSP) define a new ...[more]

PMID: 34588692

Similar Datasets

Single particle cryo-EM dataset of sarkosyl-insoluble fraction from the frontal cortex of an individual with Type I globular glial tauopathy

Project description: Not available

| EMPIAR-10766 | biostudies-other

Single particle cryo-EM dataset of sarkosyl-insoluble fraction from the frontal cortex of an individual with mutation +16 in intron 10 of MAPT

Project description: Not available

| EMPIAR-10769 | biostudies-other

Single particle cryo-EM dataset of sarkosyl-insoluble fraction from the frontal cortex of an individual with Alzheimer’s disease of amyloid-β 42 filaments

Project description: Not available

| EMPIAR-10916 | biostudies-other

Single particle cryo-EM dataset of sarkosyl-insoluble fraction from the frontal cortex of an individual with pathological aging of amyloid-β 42 filaments

Project description: Not available

| EMPIAR-10917 | biostudies-other

Single particle cryo-EM dataset of sarkosyl-insoluble fraction from the nucleus accumbens of an individual with argyrophilic grain disease

Project description: Not available

| EMPIAR-10768 | biostudies-other

Single particle cryo-EM dataset of sarkosyl-insoluble fraction from the cingulate cortex of an individual with Parkinson's disease dementia -synuclein filaments

Project description: Not available

| EMPIAR-11140 | biostudies-other

Single particle cryo-EM dataset of sarkosyl-insoluble fraction from the temporal cortex of an individual with limbic-predominant neuronal inclusion body 4R tauopathy

Project description: Not available

| EMPIAR-10767 | biostudies-other

Association of Progressive Supranuclear Palsy Rating Scale with Progressive Supranuclear Palsy Quality of Life Scale.

Project description:IntroductionThere is growing interest in using patient-reported outcomes as end points in clinical trials, such as the progressive supranuclear palsy quality of life (PSP-QoL) scale. However, this tool has not been widely validated and its correlation with validated motor scales has not been explored. To evaluate the potential utility of using PSP-QoL as an outcome, it is important to examine its relationship with a standard scale used to evaluate neurologic parameters, such as the PSP Rating Scale.MethodsPSP-QoL and PSP Rating Scale scores were gathered from 60 clinically diagnosed PSP patients, including patients with Richardson syndrome PSP (PSP-RS, n = 43) and those with non-RS PSP variants (n = 17). Linear regression analysis adjusted for age, sex, and disease duration was used to evaluate the cross-sectional relationship between the total and subscale scores of the 2 instruments.ResultsAmong 60 PSP patients, there was a significant correlation between total PSP-QoL and PSP Rating Scale scores. The physical and mentation subscales of each instrument also demonstrated significant correlations. Comparisons among PSP subtypes indicated that worsening PSP-QoL Total and Physical subscale scores correlated with worsening PSP Rating Scale gait subscale scores more strongly for the non-RS PSP variants than for PSP-RS.DiscussionThere is a significant association between the total scores and many of the subscale scores of the PSP-QoL and the PSP Rating Scale. Additionally, the relationship between these measures may differ for PSP-RS and non-RS variants. These findings suggest that the PSP-QoL may be useful in clinical trials as a patient-reported outcome measure. Large prospective multicenter studies utilizing the PSP-QoL are necessary to examine its relationship to disease evolution and changes in the PSP Rating Scale.

| S-EPMC8119324 | biostudies-literature

Genetics of Progressive Supranuclear Palsy.

Project description:Progressive supranuclear palsy (PSP) is a neurodegenerative syndrome that is clinically characterized by progressive postural instability, supranuclear gaze palsy, parkinsonism and cognitive decline. Pathologically, diagnosis of PSP is based on characteristic features, such as neurofibrillary tangles, neutrophil threads, tau-positive astrocytes and their processes in basal ganglia and brainstem, and the accumulation of 4 repeat tau protein. PSP is generally recognized as a sporadic disorder; however, understanding of genetic background of PSP has been expanding rapidly. Here we review relevant publications to outline the genetics of PSP. Although only small number of familial PSP cases have been reported, the recognition of familial PSP has been increasing. In some familial cases of clinically probable PSP, PSP pathologies were confirmed based on NINDS neuropathological diagnostic criteria. Several mutations in MAPT, the gene that causes a form of familial frontotemporal lobar degeneration with tauopathy, have been identified in both sporadic and familial PSP cases. The H1 haplotype of MAPT is a risk haplotype for PSP, and within H1, a sub-haplotype (H1c) is associated with PSP. A recent genome-wide association study on autopsyproven PSP revealed additional PSP risk alleles in STX6 and EIF2AK3. Several heredodegenerative parkinsonian disorders are referred to as PSP-look-alikes because their clinical phenotype, but not their pathology, mimics PSP. Due to the fast development of genomics and bioinformatics, more genetic factors related to PSP are expected to be discovered. Undoubtedly, these studies will provide a better understanding of the pathogenesis of PSP and clues for developing therapeutic strategies.

| S-EPMC4572662 | biostudies-literature

Diplopia in Progressive Supranuclear Palsy.

Project description: Not available

| S-EPMC7011663 | biostudies-literature