Ablation of Mitochondrial RCC1-L Induces Nigral Dopaminergic Neurodegeneration and Parkinsonian-like Motor Symptoms.

Mitochondrial dysfunction has been linked to both idiopathic and familial forms of Parkinson's disease (PD). We have previously identified RCC1-like (RCC1L) as a protein of the inner mitochondrial membrane important to mitochondrial fusion. Herein, to test whether deficits in RCC1L mitochondrial function might be involved in PD pathology, we have selectively ablated the gene in the dopaminergic (DA) neurons of mice.

Endocrine Nurses Society Position Statement on Transgender and Gender Diverse Care.

The Endocrine Nurses Society (ENS) is committed to clinical excellence in the art and science of endocrine nursing throughout the world. ENS recognizes that transgender and gender diverse (TGD) individuals face challenges and inequities that place them in the realm of health disparities. Further, TGD individuals often face substantial barriers to care and have difficulty finding healthcare providers who are knowledgeable about the unique health needs of this patient population.

Proteinase bone morphogenetic protein 1, but not tolloid-like 1, plays a dominant role in maintaining periodontal homeostasis.

Background: Periodontitis is caused by multiple factors involving a bacterial challenge and a susceptible host, although there is no report on gene mutation directly linked to this common disease. Mutations in the proteinase bone morphogenetic protein 1 (BMP1) were identified in patients with osteogenesis imperfecta, who display some dentin defects and alveolar bone loss. We previously reported essential roles of BMP1 and tolloid-like 1 (TLL1), two closely related extracellular proteinases with overlapping functions, in mouse periodontium growth by simultaneous knockout (KO) of both genes, although the separate roles of BMP1 and TLL1 have remained unclear.

Donor HLA-DR Drives the Development of De Novo Autoimmunity Following Lung and Heart Transplantation.

Unlabelled: Individuals harbor preexisting HLA-DR/DQ-restricted responses to collagen type V (ColV) mediated by Th17 cells under Treg control, both specific to peptides that bind to inherited HLA class II antigens. Yet after transplant, the donor-DR type somehow influences graft outcome. We hypothesized that, long after a lung or heart allograft, the particular HLA-DR type of the mismatched transplant donor transforms the specificity of the "anti-self" response.

Precise Spatiotemporal Control of Nodal Na Channel Clustering by Bone Morphogenetic Protein-1/Tolloid-like Proteinases.

During development of the peripheral nervous system (PNS), Schwann-cell-secreted gliomedin induces the clustering of Na channels at the edges of each myelin segment to form nodes of Ranvier. Here we show that bone morphogenetic protein-1 (BMP1)/Tolloid (TLD)-like proteinases confine Na channel clustering to these sites by negatively regulating the activity of gliomedin. Eliminating the Bmp1/TLD cleavage site in gliomedin or treating myelinating cultures with a Bmp1/TLD inhibitor results in the formation of numerous ectopic Na channel clusters along axons that are devoid of myelin segments.