New Paradigms

8.17 A zebrafish model of X-linked adrenoleukodystrophy recapitulates key disease features and demonstrates a developmental requirement for abcd1 in oligodendrocyte patterning and myelination

LR Strachan , TJ Stevenson , B Freshner , MD Keefe , D Miranda Bowles , JL Bonkowsky

To read the full abstract: Hum Mol Genet. 2017; 26(18): 3600-3614X-linked adrenoleukodystrophy (ALD) is caused by genetic mutations in the ABCD1 gene coding for a peroxisomal transmembrane protein responsible for the metabolism of very long chain fatty acids (VLCFA). It manifests with a broad phenotype mainly involving the peripheral and central nervous system and the adrenals. However, the exact pa...

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ey0015.8-18 | New Paradigms | ESPEYB15

8.18 PKA signaling drives reticularis differentiation and sexually dimorphic adrenal cortex renewal

T Dumontet , I Sahut-Barnola , A Septier , N Montanier , I Plotton , F Roucher-Boulez , V Ducros , AM Lefrançois-Martinez , JC Pointud , M Zubair , KI Morohashi , DT Breault , P Val , A Martinez

To read the full abstract: JCI Insight. 2018;3(2). pii: 98394The (human) adrenal cortex undergoes massive changes in structure and function from fetal to postnatal life, with the first consisting of a small outer definitive zone and a larger inner fetal zone, and the latter finally consisting of three distinct layers, namely the zona glomerulosa (ZG), fasciculata (ZF) and reticularis (ZR). Ho...

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ESPE Yearbook of Paediatric Endocrinology

Yearbook of Paediatric Endocrinology 2018

8 Adrenals

8.17 A zebrafish model of X-linked adrenoleukodystrophy recapitulates key disease features and demonstrates a developmental requirement for abcd1 in oligodendrocyte patterning and myelination

8.18 PKA signaling drives reticularis differentiation and sexually dimorphic adrenal cortex renewal

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