November 15, 1998Journal of Clinical InvestigationOpen Access

Novel arterial pathology in mice and humans hemizygous for elastin.

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Why the study?

Does ELN hemizygosity cause compensatory structural changes in arterial development in mice and humans?

Population

Mice hemizygous for the elastin gene and humans with ELN hemizygosity.

Comparison

ELN hemizygosity vs Normal elastin alleles (implied wild-type)

Design

Preclinical

Authors

DLD.Y. LiSichuan Agricultural University GFGilles FauryInserm DTDan TaylorSheffield Teaching Hospitals NHS Foundation Trust

Discussion

Member takes

Implication

May account for stenosis risk in ELN disorders; leaves open targeted validation in humans.

Structured PICO

Does ELN hemizygosity cause compensatory structural changes in arterial development in mice and humans?

Population

Mice hemizygous for the elastin gene (ELN +/-) and humans with ELN hemizygosity (supravalvular aortic stenosis).

Intervention

ELN hemizygosity (loss-of-function mutations in one elastin allele)

Comparator

Normal elastin alleles (implied wild-type)

Outcome

Arterial compliance and structural changes (number of elastic lamellae and smooth muscle)surrogate

ELN hemizygosity induces a compensatory increase in elastic lamellae and smooth muscle during arterial development, explaining the profound arterial thickening and risk of obstructive vascular disease in affected humans.

Cite This Study

Li et al. (1998) studied this question.

synapsesocial.com/papers/6a8a66ca9be9d02581bdac0f https://doi.org/10.1172/jci4487

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Novel arterial pathology in mice and humans hemizygous for elastin.

Discussion

Member takes

Implication

Structured PICO

Cite This Study

Novel arterial pathology in mice and humans hemizygous for elastin.

Discussion

Member takes

Implication

Structured PICO

Cite This Study

Also Consider

Also Consider