ANTI-COLLAGEN TYPE VII CLONE LH24 (C15-1885-088)

General description

Prelamin-A/C (UniProt: P48678) is encoded by the Lmna (also known as Lmn1) gene (Gene ID: 16905) in murine species. Lamins are components of the nuclear lamina that provide a framework for the nuclear envelope and may also interact with chromatin. Lamin A and C are present in equal amounts in the lamina of mammals. They play an important role in nuclear assembly, chromatin organization, nuclear membrane, and telomere dynamics. Unlike mature lamin A, prelamin A accumulates as discrete and localized foci at the nuclear periphery. Prelamin-A/C is synthesized with two pro-peptides (aa 648-662 and 663-665) that are subsequently cleaved off to produce Lamin A/C (aa 1-647). The prelamin-A/C maturation pathway includes farnesylation of CAAX motif, ZMPSTE24 mediated cleavage of the last three amino acids, methylation of the C-terminal cysteine and endoproteolytic removal of the last 15 C-terminal amino acids. ZMPSTE24 is shown to be essential for the conversion of farnesyl-prelamin A to mature lamin . In the absence of ZMPSTE24 farnesyl-prelamin A accumulates in the nucleus and results in toxicity causing a variety of disease phenotypes. Failure to convert prelamin A to lamin A has been linked to cardiac myopathy. Prelamin A/C can accelerate smooth muscle cell senescence. It can act to disrupt mitosis and induce DNA damage in vascular smooth muscle cells, leading to mitotic failure, genomic instability, and premature senescence. Mutations in Lmna gene is known to cause skeletal and cardiac myopathies, sarcopenia, osteopenia, decreased bone formation, and decreased muscle growth. (Ref.: Heizer, PJ., et al. (2020). J. Lipid Res. 61; 413-421; Li, W., et al. (2011). PLoS One. 6(4); e19313; Davies, BSJ., et al. (2010). Human Mol. Gen. 19(13); 2682-2694).

Specificity

Clone 3C8 is a rat monoclonal antibody that detects Prelamin-A. It targets an epitope within 18 amino acids from the C-terminal region.

Immunogen

A linear peptide corresponding to 18 amino acids from the C-terminal region of mouse Prelamin-A.

Application

Quality Control Testing

Evaluated by Western Blotting in lysate from Mouse smooth muscle cell with ZMPSTE24 knockout.

Western Blotting Analysis (WB): A 1:500 dilution of this antibody detected Prelamin-A in lysate from Mouse smooth muscle cells with ZMPSTE24 knockout, but not in lysate from wild-type Mouse smooth muscle cells.

Tested Applications

Immunocytochemistry Analysis: A representative lot detected Prelamin-A in Immunocytochemistry application (Davies, B.S.J., et al. (2010). Hum Mol Genet. 19(13); 2682-94).

Immunofluorescence Analysis: A representative lot detected Prelamin-A in Immunofluorescence application (Davies, B.S.J., et al. (2010). Hum Mol Genet. 19(13); 2682-94; Heizer, P.J., et al. (2020). J Lipid Res. 61(3); 413-421).

Western Blotting Analysis: A representative lot detected Prelamin-A in Western Blotting application (Heizer, P.J., et al. (2020). J Lipid Res. 61(3); 413-421).

Immunohistochemistry Applications: A representative lot detected Prelamin-A in Immunohistochemistry applications (Davies, B.S.J., et al. (2010). Hum Mol Genet. 19(13); 2682-94; Heizer, P.J., et al. (2020). J Lipid Res. 61(3); 413-421).

Note: Actual optimal working dilutions must be determined by end user as specimens, and experimental conditions may vary with the end user

Anti-Prelamin-A, clone 3C8, Cat. No. MABT1458, is a rat monoclonal antibody that detects Prelaminin-A and is tested for use in Immunocytochemistry, Immunofluorescence, Immunohistochemistry, and Western Blotting.

Physical form

Purified rat monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Storage and Stability

Recommended storage: +2°C to +8°C.

Other Notes

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

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.