GRN : granulin precursor
Description
The GRN (granulin precursor) is a protein-coding gene located on chromosome 17.
The GRN gene provides instructions for making a protein called progranulin. This protein is primarily found in the membrane of cellular structures called lysosomes, which are specialized compartments that digest and recycle materials. Within lysosomes, progranulin can be cut (cleaved) into smaller proteins, known as granulins, which are thought to function similar to progranulin. Progranulin is found in tissues throughout the body, but it is most active in cells that are dividing rapidly, such as skin cells (fibroblasts), immune system cells, and certain brain cells. This protein helps regulate the growth, division, and survival of these cells. It also plays important roles in early embryonic development, wound healing, and the body's immune response to injury (inflammation). Progranulin is active in several types of brain cells. However, little is known about this protein's role in the brain. It appears to be critical for the survival of nerve cells (neurons).
GRN encodes a secreted protein that acts as a key regulator of lysosomal function and as a growth factor involved in inflammation, wound healing, and cell proliferation. It regulates protein trafficking to lysosomes and the activity of lysosomal enzymes. GRN facilitates lysosomal acidification, leading to degradation of mature CTSD by CTSB. It functions as a wound-related growth factor that acts directly on dermal fibroblasts and endothelial cells to promote division, migration, and the formation of capillary-like tubule structures. GRN promotes epithelial cell proliferation by blocking TNF-mediated neutrophil activation, preventing the release of oxidants and proteases. It modulates inflammation in neurons by preserving neuron survival, axonal outgrowth, and neuronal integrity.
GRN is also known as CLN11, GEP, GP88, PCDGF, PEPI, PGRN.
Associated Diseases
- Semantic dementia
- Behavioral variant of frontotemporal dementia
- Frontotemporal lobar degeneration with TDP43 inclusions
- Ceroid lipofuscinosis, neuronal, 11
- Progressive non-fluent aphasia
- GRN-related frontotemporal lobar degeneration
- CLN11 disease
