Home/Clinical Diagnostics, Laminopathies, Life Sciences, Reagents for research/Rabbit Anti Human Cleaved Farnesylated Prelamin A

Rabbit Anti Human Cleaved Farnesylated Prelamin A

The antibody is able to detect cleaved farnesylated prelamin A, while it does not recognize either full-length prelamin A or mature lamin A. This antibody can be used for the discrimination of prelamin A forms and to check the efficacy of potential therapeutic compounds acting on prelamin A processing.

SKU: ANT0046 Categories: , , ,
Product type: Reagents for research
Reagent type: Antibody
Size: 200 µL

Description

Lamin A is translated as a precursor protein harbouring a C-terminus CaaX sequence, which is typical of farnesylated proteins. In prelamin A, the CaaX sequence is made up of cysteine, serine, isoleucine and methionine (CSIM). Methionine directs farnesylation at the cysteine residue by the enzyme protein farnesyltransferase. Following cysteine farnesylation, the SIM sequence is removed by the endoprotease ZMPSTE24. This proteolytic reaction is thought to be also carried out by the endoprotease RCE1, which could replace ZMPSTE24 . The cysteine residue is then methylated by the methyltransferase Icmt and a final proteolytic cleavage, performed by ZMPSTE24, removes a further 15 C-terminal residues to produce mature lamin A. Thus, at least four prelamin A species are transiently formed during prelamin A maturation, including unprocessed prelamin A, which is not farnesylated (preLA-CSIM), farnesylated full-length prelamin A (preLA- farnesyl-CSIM), farnesylated prelamin A lacking the SIM sequence (preLA-farnesyl-C), and carboxymethylated-farnesylated prelamin A (preLA-farnesyl-C-CH3). Mutations in lamin A produce a range of diseases that have collectively been referred to as `laminopathies’ . Two progeroid (premature aging) syndromes, Hutchinson-Gilford progeria (HGPS) and restrictive dermopathy (RD), are laminopathies that arise through defects in maturation of the lamin A precursor, prelamin A. Mandibuloacral dysplasia (MAD), which can be considered a milder form of RD, also results in accumulation of the lamin A precursor. By contrast, the majority of laminopathies are due to point mutations in the A/C lamins; these exhibit a multitude of phenotypes depending on the site of the mutation .

Additional information

Size 200 µL
Application Clinical diagnostics| Life Sciences
Product type Reagents for research
Reagent type Antibody
Category Laminopathies
Subcategory Polyclonal anti Farnesylated Prelamin A antibody
Antibody type Primary antibody
Immunogen Farrnesylated prelamin A C-terminus peptide

Technical information

Size 200 µL
Purity Purified by affinity Chromatography
Form Liquid. Supplied in 100mM sodium citrate, 50mM Tris and 0.05% v/v glycerol. Neutral pH.
Shipping/storage conditions Shipped at 2-8°C. When stored at +4°C the antibody is stable for 18 months. For extended storage up to 24 months, the solution may be frozen at -20°C in working aliquots. Avoid repested freezing and thawing cycles.
Usage For Laboratory Research Use Only
Source Rabbit
Specificity Anti cleaved farnesylated prelamin A polyclonal antibody detects carboxymethylated prelamin A harbouring the farnesyl residue in cell lines and in patient-derived specimens. Anti cleaved farnesylated prelamin A polyclonal antibody detects carboxymethylated prelamin A harbouring the farnesyl residue. This antibody also recognizes progerin, the truncated form of prelamin A found in Hutchinson-Gilford Progeria Syndrome cells, and does not cross react with mature lamin A
React with Carboxymethylated prelamin Aharbouring the farnesyl residue
Recommended dilutions Recommended starting dilutions can vary lot-to-lot. Consult the suggested starting dilutions on the top of the page for lot specific values.

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References

Nuclear Nox4 interaction with prelamin A is associated with nuclear redox control of stem cell aging PUBMED ID: 30362963
Samp1 Mislocalization in Emery-Dreifuss Muscular Dystrophy PUBMED ID: 30326651
The relevance of prelamin A and RAD51 as molecular biomarkers in cervical cancer PUBMED ID: 29212225
Statins and Histone Deacetylase Inhibitors Affect Lamin A/C - Histone Deacetylase 2 Interaction in Human Cells PUBMED ID: 30766871

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