Publications by authors named "R Ordemann"

Chorea-acanthocytosis (ChAc) is a neurodegenerative disease caused by mutations in the gene. It is characterized by several neurological symptoms and the appearance of acanthocytes. Elevated tyrosine kinase Lyn activity has been recently identified as one of the key pathophysiological mechanisms in this disease, and therefore represents a promising drug target.

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Article Synopsis
  • Chorea-Acanthocytosis (ChAc) is a serious neurodegenerative disease linked to VPS13A mutations, characterized by neuroinflammation and disrupted autophagy.
  • Research using Vps13a mice models showed that active Lyn tyrosine kinase accumulation contributed to these issues, leading to the identification of potential therapeutic targets.
  • Nilotinib, a specific Lyn kinase inhibitor that crosses the blood-brain barrier, showed promise in improving both blood and neurological symptoms in these mice, suggesting it could be a potential treatment for ChAc patients.
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We conducted a prospective clinical trial to investigate the safety and efficacy of plerixafor (P) in allogeneic peripheral blood stem cells (PBSC) donors with poor mobilization response to standard-dose granulocyte colony-stimulating factor (G-CSF), defined by <2 × 10 CD34 + cells/kg recipient body-weight (CD34+/kg RBW) after 1st apheresis. A single dose of 240 µg/kg P was injected subcutaneously at 10 p.m.

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Despite the increasing role of molecular markers, differential counts and morphology of hematopoietic cells in the bone marrow (BM) remain essential diagnostic criteria in hematological diseases. However, the respective reference values for BM myelogram commonly used came from small series with limited numbers of healthy individuals. We evaluated the myelograms of 236 healthy individuals who underwent unrelated bone marrow donation.

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