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MABN1795

Sigma-Aldrich

Anti-Progranulin/GRN Antibody, clone 8H10

clone 8H10, from rat

Sinónimos:

Granulins, Progranulin/GRN

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About This Item

Código UNSPSC:
12352203
eCl@ss:
32160702
NACRES:
NA.41

origen biológico

rat

Nivel de calidad

forma del anticuerpo

purified immunoglobulin

tipo de anticuerpo

primary antibodies

clon

8H10, monoclonal

reactividad de especies

mouse

técnicas

immunohistochemistry: suitable
western blot: suitable

isotipo

IgG2cκ

Nº de acceso NCBI

Nº de acceso UniProt

Condiciones de envío

wet ice

modificación del objetivo postraduccional

unmodified

Información sobre el gen

mouse ... Grn (14824)

Descripción general

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.

Especificidad

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

Inmunógeno

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

Aplicación

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).

Calidad

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.

Descripción de destino

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

Forma física

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.

Almacenamiento y estabilidad

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

Otras notas

Concentration: Please refer to lot specific datasheet.

Cláusula de descargo de responsabilidad

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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Código de clase de almacenamiento

12 - Non Combustible Liquids

Clase de riesgo para el agua (WGK)

WGK 1

Punto de inflamabilidad (°F)

Not applicable

Punto de inflamabilidad (°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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