Evidence map›Paper›PMID 29311330›Full record

ArticleProceedings of the National Academy of Sciences of the United States of America2018

GBA1 deficiency negatively affects physiological α-synuclein tetramers and related multimers.

Sangjune Kim, Seung Pil Yun, Saebom Lee, George Essien Umanah, Veera Venkata Ratnam Bandaru, Xiling Yin, Peter Rhee, Senthilkumar S Karuppagounder, Seung-Hwan Kwon, Hojae Lee and 7 more

Open access · bronzeAbstract read
In one paragraph

Article in Proceedings of the National Academy of Sciences of the United States of America, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 128 papers.

0numbers the graph read from it
0cells of the map it votes in
128citing papers in PubMed
14.9field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

128 citing papers in PubMed, 174 citations in OpenAlex.

  1. Trial
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  7. Parkinson disease is a fatty acidopathy.Nature reviews. Neurology · 2025
    Review
  8. Article
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  11. Review
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  14. Conformational Selection of α-Synuclein Tetramers at Biological Interfaces.Journal of chemical information and modeling · 2024
    Article
  15. Article
  16. Review
  17. Article
  18. International journal of molecular sciences · 2024
    Review
  19. Article
  20. Article

68 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

17 authors at 1 institution in 1 country.

Sangjune KimNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Seung Pil YunNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Saebom LeeNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
George Essien UmanahNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Veera Venkata Ratnam BandaruNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Xiling YinNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Peter RheeNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Senthilkumar S KaruppagounderNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Seung-Hwan KwonNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Hojae LeeNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Xiaobo MaoNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Donghoon KimNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Akhilesh PandeyMckusick-Nathans Institute of Genetic Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.ORCID 0000-0001-9943-6127
Gabsang LeeNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Valina L DawsonNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Ted M DawsonNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Han Seok KoNeuroregeneration and Stem Cell Programs, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD 21205; hko3@jhmi.edu.
Johns Hopkins University · US

Funding

Understanding Mechanisms of a-synuclein pathologyP50NS038377 · NINDS · JOHNS HOPKINS UNIVERSITY · PI KO, HANSEOK · 1998 to 2019
$38.8M
'T V ASSESSMENT - MEMORY DISORDERED PATIENTS'P50AG005146 · NIA · JOHNS HOPKINS UNIVERSITY · PI HUGANIR, RICHARD L · 1985 to 2019
$37.6M
Translational Research Enhancement CoreP30DK089502 · NIDDK · JOHNS HOPKINS UNIVERSITY · PI DONOWITZ, MARK · 2011 to 2020
$12.0M
Translational Neuroscience CoreU54HD079123 · NICHD · HUGO W. MOSER RES INST KENNEDY KRIEGER · PI VALLE, DAVID · 2014 to 2019
$7.9M
Resource CoreU24NS078338 · NINDS · MCLEAN HOSPITAL · PI ISACSON, OLE · 2012 to 2013
$1.8M
Interrogating functional and molecular properties of PAX7+ putative skeletal muscle stem/progenitor cells derived from human iPSCs of healthy donors and Duchenne muscular dystrophy patientsR01AR070751 · NIAMS · JOHNS HOPKINS UNIVERSITY · PI LEE, GABSANG · 2017 to 2021
$1.8M
Cell extrinsic factors' roles on direct conversion to human induced neural crestR01NS093213 · NINDS · JOHNS HOPKINS UNIVERSITY · PI LEE, GABSANG · 2015 to 2019
$1.8M
Glucocerebrosidase Biology and It's Role in Parkinson's DiseaseR01NS082205 · NINDS · JOHNS HOPKINS UNIVERSITY · PI KO, HANSEOK · 2013 to 2017
$1.8M
Study of White Matter Development in a Rodent Model of Perinatal Brain InjuryK08NS063956 · NINDS · HUGO W. MOSER RES INST KENNEDY KRIEGER · PI FATEMI, S. ALI · 2009 to 2013
$897k
Characterization and Validation of Mouse VPS35 Model of Parkinson's DiseaseR21NS098006 · NINDS · JOHNS HOPKINS UNIVERSITY · PI KO, HANSEOK · 2017 to 2018
$449k
NIAMS NIH HHS R01 AR070751NIA NIH HHS P50 AG005146NICHD NIH HHS U54 HD079123NIDDK NIH HHS P30 DK089502NINDS NIH HHS K08 NS063956NINDS NIH HHS P50 NS038377NINDS NIH HHS R01 NS082205NINDS NIH HHS R01 NS093213NINDS NIH HHS R21 NS098006NINDS NIH HHS U24 NS078338
6 · The paper itself

Abstract

Accumulating evidence suggests that α-synuclein (α-syn) occurs physiologically as a helically folded tetramer that resists aggregation. However, the mechanisms underlying the regulation of formation of α-syn tetramers are still mostly unknown. Cellular membrane lipids are thought to play an important role in the regulation of α-syn tetramer formation. Since glucocerebrosidase 1 (GBA1) deficiency contributes to the aggregation of α-syn and leads to changes in neuronal glycosphingolipids (GSLs) including gangliosides, we hypothesized that GBA1 deficiency may affect the formation of α-syn tetramers. Here, we show that accumulation of GSLs due to GBA1 deficiency decreases α-syn tetramers and related multimers and increases α-syn monomers in CRISPR-GBA1 knockout (KO) SH-SY5Y cells. Moreover, α-syn tetramers and related multimers are decreased in N370S

Indexed as

1-Deoxynojirimycinalpha-SynucleinCell Line, TumorDopaminergic NeuronsGlucosylceramidaseGlycosphingolipidsHumansParkinson DiseaseProtein Multimerization1-Deoxynojirimycinalpha-SynucleinGlucosylceramidaseGlycosphingolipidsmiglustatGBA1glucosylceramideParkinson’s diseasetetramersα-synuclein

Identifiers

PMID29311330
PMCPMC5789900
OpenAlexW2783378118

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.