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Article

Haploinsufficiency of Col5a1 causes intrinsic lung and respiratory changes in a mouse model of classical Ehlers-Danlos syndrome.

Physiological reports - 1 Apr 2022

Fett Jordan, Dimori Milena, Carroll John L, Morello Roy

Abstract excerpt

The Ehlers-Danlos syndromes (EDS) are inherited connective tissue diseases with primary manifestations that affect the skin and the musculoskeletal system. However, the effects of EDS on the respiratory system are not well understood and are described in the literature as sporadic case reports. We performed histological, histomorphometric, and the first in-depth characterization of respiratory system function in...

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Identifiers

PMID: 35439366
DOI: 10.14814/phy2.15275
PMCID: PMC9017971

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