Evidence map›Paper›PMID 20211931›Full record

ReviewJournal of lipid research2010

Multi-system disorders of glycosphingolipid and ganglioside metabolism.

You-Hai Xu, Sonya Barnes, Ying Sun, Gregory A Grabowski

Open access · hybridAbstract readReview
In one paragraph

Review in Journal of lipid research, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 82 papers.

0numbers the graph read from it
0cells of the map it votes in
82citing papers in PubMed
5.7field-weighted citation impact, top 3% 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

82 citing papers in PubMed, 166 citations in OpenAlex.

  1. Trial
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  5. Article
  6. Lysosomal homeostasis at the crossroads of neurodegeneration.The Journal of clinical investigation · 2026
    Review
  7. Article
  8. Article
  9. Review
  10. Review
  11. Article
  12. Human genetic defects of sphingolipid synthesis.Journal of inherited metabolic disease · 2025
    Review
  13. Article
  14. Article
  15. Article
  16. iPSC-derived neural precursor cells engineeringMolecular therapy. Methods & clinical development · 2023
    Article
  17. Article
  18. Article
  19. Article
  20. Article

22 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

4 authors at 2 institutions in 1 country.

You-Hai XuDivision of Human Genetics, Cincinnati Children's Hospital Medical Center and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229-3039, USA.
Sonya Barnes
Ying Sun
Gregory A Grabowski
University of Cincinnati · USCincinnati Children's Hospital Medical Center · US

Funding

STUDIES OF GAUCHER DISEASE: A PROTOTYPE LIPIDOSISR01DK036729 · NIDDK · MOUNT SINAI SCHOOL OF MEDICINE OF CUNY · PI GRABOWSKI, GREGORY A. · 1986 to 2011
$4.5M
Therapy of Neuronopathic Gaucher DiseaseR21HD059823 · NICHD · CINCINNATI CHILDRENS HOSP MED CTR · PI GRABOWSKI, GREGORY A. · 2009 to 2010
$423k
Therapy of Gaucher Disease: In Vivo Enhancement of Residual Mutant ActivityR21NS064352 · NINDS · CINCINNATI CHILDRENS HOSP MED CTR · PI GRABOWSKI, GREGORY A. · 2009 to 2010
$413k
NICHD NIH HHS HD59823NICHD NIH HHS R21 HD059823NIDDK NIH HHS DK36729NIDDK NIH HHS R01 DK036729NINDS NIH HHS NS64352NINDS NIH HHS R21 NS064352
6 · The paper itself

Abstract

Glycosphingolipids (GSLs) and gangliosides are a group of bioactive glycolipids that include cerebrosides, globosides, and gangliosides. These lipids play major roles in signal transduction, cell adhesion, modulating growth factor/hormone receptor, antigen recognition, and protein trafficking. Specific genetic defects in lysosomal hydrolases disrupt normal GSL and ganglioside metabolism leading to their excess accumulation in cellular compartments, particularly in the lysosome, i.e., lysosomal storage diseases (LSDs). The storage diseases of GSLs and gangliosides affect all organ systems, but the central nervous system (CNS) is primarily involved in many. Current treatments can attenuate the visceral disease, but the management of CNS involvement remains an unmet medical need. Early interventions that alter the CNS disease have shown promise in delaying neurologic involvement in several CNS LSDs. Consequently, effective treatment for such devastating inherited diseases requires an understanding of the early developmental and pathological mechanisms of GSL and ganglioside flux (synthesis and degradation) that underlie the CNS diseases. These are the focus of this review.

Indexed as

Lysosomal Storage DiseasesAnimalsCentral Nervous SystemGangliosidesGlycosphingolipidsHumansInflammationMolecular StructureNeurodegenerative DiseasesGangliosidesGlycosphingolipids

Identifiers

PMID20211931
PMCPMC2882741
OpenAlexW2100789202

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.