Publications by authors named "M N Van Thielen"

Background: Popliteal pterygium syndrome (PPS) is a rare congenital disorder characterized by orofacial, cutaneous, musculoskeletal, and genital anomalies. Surgical interventions are necessary to address the severe knee flexion contracture and equinovarus deformity, but there are no established treatment guidelines.

Methods: We present the case of a one-year-old patient with PPS and discuss the challenges in managing the knee deformity.

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  • Tendrils of climbing plants like Passiflora discophora feature a unique spring-like structure made of two helices with opposite twisting, acting as effective coil springs in the plant's attachment system.
  • The study found that while these tendrils exhibit significant changes in material properties and structure as they transition from a fresh to a dried state, they still function effectively, maintaining high energy efficiency and strength.
  • This spring mechanism provides a 'fail-safe' system for the plant, allowing it to securely attach to supports while conserving energy by not needing to sustain the senescent tendrils metabolically.
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  • Plant mitochondrial and chloroplast transcripts undergo specific cytidine-to-uridine (C-to-U) RNA editing, facilitated by specialized nuclear-encoded PPR proteins that are imported into these organelles.
  • Research in the model moss Physcomitrium patens tested whether similar RNA editing could occur in the cytosol by expressing specific PPR proteins, revealing that while constitutive expression yielded poor results, hormone-induced expression led to significant cytosolic RNA editing.
  • The findings suggest that PPR-mediated C-to-U RNA editing can occur in the cytosol but is evolutionarily limited in plants due to its restricted activity and the simpler transcriptomes present in mitochondria and chloroplasts.
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Introduction: Popliteal pterygium syndrome is a rare congenital disorder characterized by orofacial, cutaneous, musculoskeletal, and genital anomalies. The popliteal pterygium (PP) affects ambulation due to severe knee flexion contracture and equinovarus deformities. Surgical treatment aims to correct these deformities while preserving limb sensibility.

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