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MABN1795

Sigma-Aldrich

Anti-Progranulin/GRN Antibody, clone 8H10

clone 8H10, from rat

别名:

Granulins, Progranulin/GRN

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About This Item

分類程式碼代碼:
12352203
eCl@ss:
32160702
NACRES:
NA.41

生物源

rat

品質等級

抗體表格

purified immunoglobulin

抗體產品種類

primary antibodies

無性繁殖

8H10, monoclonal

物種活性

mouse

技術

immunohistochemistry: suitable
western blot: suitable

同型

IgG2cκ

NCBI登錄號

UniProt登錄號

運輸包裝

wet ice

目標翻譯後修改

unmodified

基因資訊

mouse ... Grn (14824)

一般說明

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.

特異性

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

免疫原

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

應用

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).

品質

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.

標靶描述

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

外觀

Protein G Purified
Format: 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.

儲存和穩定性

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

其他說明

Concentration: Please refer to lot specific datasheet.

免責聲明

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.

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儲存類別代碼

12 - Non Combustible Liquids

水污染物質分類(WGK)

WGK 1

閃點(°F)

Not applicable

閃點(°C)

Not applicable


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Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis.
Gotzl, JK; Mori, K; Damme, M; Fellerer, K; Tahirovic, S; Kleinberger, G; Janssens et al.
Acta neuropathologica null

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