Mouse development is not obviously affected by the absence of dermatan sulfate epimerase 2 in spite of a modified brain dermatan sulfate composition.

• Dermatan sulfate epimerase 2 (DS-epi2), together with its homologue DS-epi1, transform glucuronic acid into iduronic acid in dermatan sulfate polysaccharide chains. Iduronic acid gives dermatan sulfate increased chain flexibility and promotes protein binding. DS-epi2 is ubiquitously expressed and is the predominant epimerase in brain. Here we report the generation and initial characterization of DS-epi2 null mice. DS-epi2 deficient mice showed no anatomical, histological or morphological abnormalities. The body weights and lengths of mutated and wild-type littermates were indistinguishable. They were fertile and had a normal lifespan. Chondroitin/dermatan sulfate (CS/DS) isolated from newborn mutated mouse brains had a 38% reduction in iduronic acid compared to wild type littermates and compositional analysis revealed a decrease of 4-O-sulfate and an increase of 6-O-sulfate containing structures. Despite the reduction in iduronic acid, adult DS-epi2-/- brain showed normal extracellular matrix features by immunohistological stainings. We conclude that DS-epi1 compensates in vivo for the loss of DS-epi2.. These results extend previous findings of functional redundancy of brain extracellular matrix components.

Ämnesord

NATURVETENSKAP  -- Biologi -- Biokemi och molekylärbiologi (hsv//swe)

NATURAL SCIENCES  -- Biological Sciences -- Biochemistry and Molecular Biology (hsv//eng)

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