NgBR

NgBR, or Nogo-B receptor, is a human membrane-associated protein encoded by the NUS1 gene. It was named for its ability to bind Nogo-B, a member of the reticulon family involved in neurite outgrowth. NgBR is primarily localized to the Golgi apparatus and endoplasmic reticulum membranes.

Functionally, NgBR is linked to the dolichol-phosphate mannose (DPM) biosynthesis pathway, playing a role in the production of dolichol-phosphate-mannose, a key donor for protein N-glycosylation. It is thought to act as a regulatory partner that supports proper glycosylation, potentially by stabilizing or modulating other DPM subunits. In addition to its glycosylation-related functions, NgBR participates in Nogo-B signaling; disruption of NgBR can influence endothelial cell behavior and angiogenesis in experimental systems.

NgBR has also been studied for its role in vascular biology due to its interaction with Nogo-B, and alterations in this pathway can affect angiogenic processes and vascular remodeling. The dual involvement in glycosylation and Nogo-B–mediated signaling reflects a multifaceted function for NgBR in cellular homeostasis and development.

Clinical significance is associated with variants in NUS1, which have been linked to neurodevelopmental disorders and a spectrum of congenital disorders of glycosylation, including cognitive impairment and seizures. The precise clinical presentation can vary, highlighting the essential role of NgBR in fundamental glycosylation pathways as well as in vascular signaling.

In summary, NgBR is a Golgi-associated protein encoded by NUS1 that interfaces with glycosylation machinery and Nogo-B signaling, with emerging relevance to human developmental and neurological disorders.