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Mouse Anti-GRN Recombinant Antibody (CBLG1-1807) (CBMAB-G5078-LY)


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Summary

Host Animal
Mouse
Specificity
Mouse, Human
Clone
CBLG1-1807
Antibody Isotype
IgG2a
Application
WB

Basic Information

Specificity
Mouse, Human
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Concentration
0.5 mg/mL
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Granulin Precursor
Introduction
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]
Entrez Gene ID
Human2896
Mouse14824
UniProt ID
HumanP28799
MouseQ9D2V3
Function
Secreted protein that acts as a key regulator of lysosomal function and as a growth factor involved in inflammation, wound healing and cell proliferation (PubMed:28541286, PubMed:28073925, PubMed:18378771, PubMed:28453791, PubMed:12526812).

Regulates protein trafficking to lysosomes and, also the activity of lysosomal enzymes (PubMed:28453791, PubMed:28541286).

Facilitates also the acidification of lysosomes, causing degradation of mature CTSD by CTSB (PubMed:28073925).

In addition, functions as 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 (By similarity).

Also promotes epithelial cell proliferation by blocking TNF-mediated neutrophil activation preventing release of oxidants and proteases (PubMed:12526812).

Moreover, modulates inflammation in neurons by preserving neurons survival, axonal outgrowth and neuronal integrity (PubMed:18378771).

Granulin-4:
Promotes proliferation of the epithelial cell line A431 in culture.

Granulin-3:
Inhibits epithelial cell proliferation and induces epithelial cells to secrete IL-8.

Granulin-7:
Stabilizes CTSD through interaction with CTSD leading to maintain its aspartic-type peptidase activity.
Biological Process
Astrocyte activation involved in immune response Source: UniProtKB
Lysosomal lumen acidification Source: UniProtKB
Lysosomal transport Source: UniProtKB
Lysosome organization Source: UniProtKB
Microglial cell activation involved in immune response Source: UniProtKB
Negative regulation of microglial cell activation Source: UniProtKB
Negative regulation of neuron apoptotic process Source: UniProtKB
Negative regulation of neutrophil activation Source: UniProtKB
Negative regulation of respiratory burst involved in inflammatory response Source: UniProtKB
Positive regulation of angiogenesis Source: UniProtKB
Positive regulation of aspartic-type peptidase activity Source: UniProtKB
Positive regulation of axon regeneration Source: UniProtKB
Positive regulation of cell migration Source: ARUK-UCL
Positive regulation of defense response to bacterium Source: UniProtKB
Positive regulation of endothelial cell migration Source: UniProtKB
Positive regulation of epithelial cell proliferation Source: UniProtKB
Positive regulation of inflammatory response to wounding Source: UniProtKB
Positive regulation of lysosome organization Source: UniProtKB
Positive regulation of neuron apoptotic process Source: UniProtKB
Positive regulation of protein folding Source: UniProtKB
Protein stabilization Source: UniProtKB
Regulation of inflammatory response Source: GO_Central
Signal transduction Source: ProtInc
Cellular Location
Secreted; Lysosome. Endocytosed by SORT1 and delivred to lysosomes (PubMed:21092856, PubMed:28073925). Targeted to lysosome by PSAP via M6PR and LRP1, in both biosynthetic and endocytic pathways (PubMed:26370502, PubMed:28073925). Co-localized with GBA in the intracellular trafficking compartments until to lysosome (By similarity).
Involvement in disease
Ubiquitin-positive frontotemporal dementia (UP-FTD):
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.
Ceroid lipofuscinosis, neuronal, 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.
PTM
Cleaved by ELANE; proteolysis is blocked by SLPI and is concentration- and time-dependent and induces CXCL8/IL-8 production; granulin-3 and granulin-4 are resistant to ELANE (PubMed:12526812, PubMed:28743268). Cleaved by CTSL in lysosome thus regulating the maturation and turnover of progranulin within the lysosome (PubMed:28743268).
More Infomation

Boland, S., Swarup, S., Ambaw, Y. A., Malia, P. C., Richards, R. C., Fischer, A. W., ... & Farese Jr, R. V. (2022). Deficiency of the frontotemporal dementia gene GRN results in gangliosidosis. Nature Communications, 13(1), 5924.

Zhou, X., Kukar, T., & Rademakers, R. (2021). Lysosomal dysfunction and other pathomechanisms in FTLD: evidence from progranulin genetics and biology. Frontotemporal Dementias: Emerging Milestones of the 21st Century, 219-242.

Petkau, T. L., Life, B., Lu, G., Yang, J., Fornes, O., Wasserman, W., ... & Leavitt, B. R. (2021). Human progranulin-expressing mice as a novel tool for the development of progranulin-modulating therapeutics. Neurobiology of Disease, 153, 105314.

Boland, S., Swarup, S., Ambaw, Y. A., Richards, R. C., Fischer, A. W., Singh, S., ... & Farese Jr, R. V. (2021). Progranulin deficiency results in reduced bis (monoacylglycero) phosphate (BMP) levels and gangliosidosis. bioRxiv, 2021-09.

Klupp, F., Kahlert, C., Franz, C., Halama, N., Schleussner, N., Wirsik, N. M., ... & Ulrich, A. B. (2021). Granulin: an invasive and survival-determining marker in colorectal cancer patients. International Journal of Molecular Sciences, 22(12), 6436.

Bhopatkar, A. A., Uversky, V. N., & Rangachari, V. (2020). Granulins modulate liquid–liquid phase separation and aggregation of the prion-like C-terminal domain of the neurodegeneration-associated protein TDP-43. Journal of Biological Chemistry, 295(8), 2506-2519.

Bateman, A., Cheung, S. T., & Bennett, H. P. (2018). A brief overview of progranulin in health and disease. Progranulin: Methods and Protocols, 3-15.

Paushter, D. H., Du, H., Feng, T., & Hu, F. (2018). The lysosomal function of progranulin, a guardian against neurodegeneration. Acta neuropathologica, 136, 1-17.

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For research use only. Not intended for any clinical use.

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