ANTI-PROGRANULIN/GRN CLONE 8H10 (RAT MONOCLONAL)

General description

Granulins constitute 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 602 aa murine precursor protein, preprogranulin (UniProt Q3U9N4), is also called proepithelin and PC cell-derived growth factor. Cleavage of the signal peptide (aa 1-17) produces progranulin (Acrogranin; aa 18-589), which can be further cleaved into 7 active peptides named granulin-1 through granulin-7. Both the peptides and progranulin protein regulate cell growth. Granulin family members are important in normal development, wound healing, and tumorigenesis. Heterozygous loss-of-function mutations in the GRN gene is a known cause for frontotemporal lobar degeneration (FTLD) with accumulation of TAR DNA-binding protein (TDP)-43, while a complete GRN deficiency due to a homozygous loss-of-function mutation causes the lysosomal storage disorder known as neuronal ceroid lipofuscinosis (NCL). In Grn-/- mice, a murine model for GRN-associated FTLD-TDP (FTLD-TDP/GRN), the lysosomal proteins cathepsin D (CTSD) is found to be elevated, while an upregulated granulin level is observed in Ctsd(−/−) mice, a model for NCL. Moreover, both types of knockout mice are found to display increased levels of transmembrane protein 106B (TMEM106B) as a common phenotype.

Specificity

Reacts with Preprogranulin and Progranulin (Acrogranin, Proepithelin), but not Granulin-1 through Granulin-7.

Immunogen

Epitope: Near C-terminus

Linear peptide corresponding to rat progranulin sequence near the C-terminus

Application

Research Category
Neuroscience

Research Sub Category
Developmental Signaling

This Anti-Progranulin/GRN Antibody, clone 8H10 is validated for use in Western Blotting, Immunohistochemistry for the detection of Progranulin/GRN.

Western Blotting Analysis: A representative lot detected elevated progranulin expression in brain homogenates from Ctsd-/- mice, and loss of progranulin expression in brain homogenates from Grn-/- mice (Gotzl, J.K., et al (2014). Acta Neuropathol.127(6):845-860).
Immunohistochemistry Analysis: A representative lot detected elevated progranulin expression in the cortex, hippocampus, and thalamus regions of Ctsd-/- mice when compared with wild-type mice using frozen brain sections (Gotzl, J.K., et al (2014). Acta Neuropathol.127(6):845-860).

Quality

Evaluated by Western Blotting in mouse uterus tissue lysate.

Western Blotting Analysis: 2.0 µg/mL of this antibody detected Progranulin/GRN in 10 µg of mouse uterus tissue lysate.

Target description

~65 kDa observed. Uncharacterized band(s) may appear in some lysates.

Physical form

Format: Purified

Protein G Purified

Purified rat monoclonal IgG2cκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Storage and Stability

Stable for 1 year at 2-8°C from date of receipt.

Other Notes

Concentration: Please refer to lot specific datasheet.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.