Publications by authors named "D J B Keene"

Mutations in the collagen-modifying enzyme lysyl hydroxylase 1 (LH1) cause Warmblood Fragile Foal Syndrome (WFFS) in horses. We investigated the impact of this mutation on collagen structure and function. Our results show that LH1 deficiency leads to reduced lysine hydroxylation, altered collagen fibril organization, and tissue abnormalities resembling human Ehlers-Danlos syndrome.

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Significant photoinduced voltages observed in permalloy structures consist of two contributions with different origins, which depend on illumination conditions, structure geometry and magnetic field in distinct ways. The first component is the plasmon drag effect voltage closely associated with plasmon propagation. The second contribution is magnetically dependent and can be related to photoinduced gradients in the sample temperature and spin polarization.

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  • Supramolecular ECM networks, crucial for skin structure and function, include EMILIN proteins that interact with elastin and influence cellular signaling.
  • Our study mapped the distribution of EMILIN-1, -2, and -3 in human skin, revealing their role in elastic fibers and their connections with basal keratinocytes.
  • We found that in conditions like Marfan syndrome and scleroderma, the presence and localization of EMILINs change, indicating they can be used as markers for monitoring dermal ECM rearrangements due to aging and disease.
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  • - The study investigates the effectiveness of tendon allograft reconstruction in maintaining pubic stability after iliac osteotomies in patients undergoing bladder exstrophy repair, a condition needing surgical intervention due to pelvic organ issues.
  • - Eleven patients, averaging 14 months old, underwent the procedure, followed by a period with external fixators, and their pelvic positions were monitored through scans and X-rays at various times, showing a successful urologic closure across the board.
  • - Results indicated that in 9 of the 11 patients, the distance between their pubic bones either stabilized or decreased over an average follow-up of 11 months, suggesting that the reconstruction method is promising for managing pubic symphysis issues post-surgery.
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Background: RNA-binding proteins (RBPs) modulate the synaptic proteome and are instrumental in maintaining synaptic homeostasis. Moreover, aberrant expression of an RBP in a disease state would have deleterious downstream effects on synaptic function. While many underlying mechanisms of synaptic dysfunction in Alzheimer's disease (AD) have been proposed, the contribution of RBPs has been relatively unexplored.

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