Anti-SPG11 Antibody [RP23040242]

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

SPG11 also known as spatacsin is a protein with a mass of approximately 249 kDa. This protein is expressed mainly in neural tissues including the brain and spinal cord. Mechanically SPG11 interacts with various other proteins contributing to the maintenance and function of the lysosomal compartment within cells. It plays an important role in membrane trafficking processes particularly in the vesicles involved in endocytosis and autophagy.

Biological function summary

In the context of neuronal health SPG11 is significant for the function of neurons. It forms part of a larger protein complex known as the AP-5 adaptin complex which assists in lysosomal positioning and function. By ensuring proper lysosomal function SPG11 supports neuronal maintenance and health. Issues with its function disrupt lysosomal degradation pathways and can have broad implications for neuron viability.

Pathways

SPG11 is essential in endolysosomal and autophagic pathways serving as a connection point for effective lysosomal transport. These pathways include interactions with proteins such as AP5Z1 and ZFYVE26 which collaborate to modulate lysosomal positioning and maintenance. SPG11's proper operation within these pathways allows for efficient cellular digestion of macromolecules and removal of damaged organelles critical for cellular health.

Associated diseases and disorders

Mutations in SPG11 lead to rare neurological conditions like spastic paraplegia type 11 and some forms of juvenile amyotrophic lateral sclerosis (ALS). SPG11's mutation or dysfunction can result in improper lysosomal degradation contributing to the nervous system's degeneration. Related proteins like spastizin (ZFYVE26) and AP5Z1 when disrupted also exhibit similar symptoms of neural decay highlighting SPG11's integral role in neuronal disease mechanisms.