Progranulin Aa248-259/Granulin-3 (GRN) Antibody – Goat Polyclonal

Granulins are a family of secreted, glycosylated peptides that are cleaved from a single precursor protein with 7.5 repeats of a highly conserved 12-cysteine granulin/epithelin motif. The 88 kDa precursor protein, progranulin, is also called proepithelin and PC cell-derived growth factor. Cleavage of the signal peptide produces mature granulin which can be further cleaved into a variety of active, 6 kDa peptides. These smaller cleavage products are named granulin A, granulin B, granulin C, etc. Epithelins 1 and 2 are synonymous with granulins A and B, respectively. Both the peptides and intact granulin protein regulate cell growth. However, different members of the granulin protein family may act as inhibitors, stimulators, or have dual actions on cell growth. Granulin family members are important in normal development, wound healing, and tumorigenesis. [provided by RefSeq, Jul 2008]

GRN: Granulins have possible cytokine-like activity. They may play a role in inflammation, wound repair, and tissue remodeling. Defects in GRN are the cause of ubiquitin-positive frontotemporal dementia (UP-FTD); also known as tau- negative frontotemporal dementia linked to chromosome 17. Frontotemporal dementia (FTD) is the second most common cause of dementia in people under the age of 65 years. It is an autosomal dominant neurodegenerative disease. Defects in GRN are the cause of neuronal ceroid lipofuscinosis type 11 (CLN11). A form of neuronal ceroid lipofuscinosis characterized by rapidly progressive visual loss due to retinal dystrophy, seizures, cerebellar ataxia, and cerebellar atrophy. Cognitive decline may also occur. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material. Belongs to the granulin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 17q21.32

Cellular Component: cytosol; extracellular space; intracellular membrane-bound organelle

Molecular Function: cytokine activity; growth factor activity; protein binding

Biological Process: male mating behavior; response to estradiol stimulus; signal transduction; synaptic vesicle endocytosis

Disease: Ceroid Lipofuscinosis, Neuronal, 11; Frontotemporal Lobar Degeneration With Tdp43 Inclusions, Grn-related