Article

iPSC-derived astrocytes to model phenotype-specific differential neuroinflammatory and metabolic responses in X-linked adrenoleukodystrophy

2022-09-10

Abstract excerpt

<h4>SUMMARY</h4> X-linked adrenoleukodystrophy (X-ALD) is an inherited progressive metabolic disorder caused by pathogenic variants in the ABCD1 gene, which leads to accumulation of very long chain fatty acids in body fluids and tissues including brain and spinal cord. In the absence of a clear genotype-phenotype correlation the molecular mechanisms of the severe cerebral adrenoleukodystrophy (cALD) and the mild...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 9c4c1469-5d96-5664-952f-3c2be6298ade
DOI: 10.1101/2022.09.09.507263

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

iPSC-derived astrocytes to model phenotype-specific differential neuroinflammatory and metabolic responses in X-linked adrenoleukodystrophyDOI 10.1101/2022.09.09.507263Read

Generating human AMN and cALD iPSC-derived astrocytes with potential for modeling X-linked adrenoleukodystrophy phenotypesSemantic proximity Read

Functional genomic analysis unravels a metabolic-inflammatory interplay in adrenoleukodystrophySemantic proximity Read

Silencing of Abcd1 and Abcd2 genes sensitizes astrocytes for inflammation: implication for X-adrenoleukodystrophySemantic proximity Read

Induced pluripotent stem cell models from X-linked adrenoleukodystrophy patients.Semantic proximity Read

Adrenoleukodystrophy: Current understanding of disease mechanisms, diagnosis, and therapeutic advances-a recent review.Semantic proximity Read

ABCD1 and X-linked adrenoleukodystrophy: A disease with a markedly variable phenotype showing conserved neurobiology in animal models.Semantic proximity Read

Management of adrenoleukodystrophy: From pre-clinical studies to the development of new therapies.Semantic proximity Read

Management of adrenoleukodystrophy: From pre-clinical studies to the development of new therapiesSemantic proximity Read

X-linked adrenoleukodystrophy: clinical, metabolic, genetic and pathophysiological aspects.Semantic proximity Read

Evolution of adrenoleukodystrophy model systems.Semantic proximity Read

Defective neuritogenesis in <i>Abcd1/2</i> deficient rat neurons due to intrinsic and astrocyte-dependent mechanismsSemantic proximity Read

X-linked adrenoleukodystrophy (X-ALD): clinical presentation and guidelines for diagnosis, follow-up and management.Semantic proximity Read