Peroxisome deficiency underlies failures in hepatic immune cell development and antigen presentation in a severe Zellweger disease model.

Peroxisome biogenesis disorders (PBDs) represent a group of metabolic conditions that cause severe developmental defects. Peroxisomes are essential metabolic organelles, present in virtually every eukaryotic cell and mediating key processes in immunometabolism. To date, the full spectrum of PBDs remains to be identified, and the impact PBDs have on immune function is unexplored. This study presents a characterization of the hepatic immune compartment of a neonatal PBD mouse model at single-cell resolution to establish the importance and function of peroxisomes in developmental hematopoiesis. We report that hematopoietic defects are a feature in a severe PBD murine model. Finally, we identify a role for peroxisomes in the regulation of the major histocompatibility class II expression and antigen presentation to CD4+ T cells in dendritic cells. This study adds to our understanding of the mechanisms of PBDs and expands our knowledge of the role of peroxisomes in immunometabolism.

The ethical challenges of providing fertility care to patients with chronic illness or terminal disease.

The field of fertility is rapidly evolving, bringing opportunities for improvement in our patients' quality of life as well as bringing new ethical dilemmas. As medical science continues to advance, significant numbers of the reproductive-aged population are living with chronic and/or terminal conditions but have reasonable odds of lengthy survival and wish to have children. Likewise, there are adolescents diagnosed with cancer who are increasingly expected to achieve an improved, if not normal, life expectancy after treatment. Oftentimes these children are told they must sacrifice their ability to later have genetically related offspring; however, technologies to preserve fertility are changing this prognosis. Patients with chronic infection are living longer, more normal lives and are increasingly seeking reproductive assistance. Moreover, there is an increasing number of patients' families desiring posthumous use of gametes, which also raises ethical and legal issues. This article discusses ethical principles of bioethics and then highlights specific ethical issues through four plausible cases that may be seen in a fertility practice providing medical care to patients with chronic illness or terminal disease. It concludes that prompt referral of patients to the reproductive endocrinologist, along with a multidisciplinary approach to care, provides increased chances of successful treatment of this group of patients.