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DescriptionAdditional informationTechnical informationDownloadReferencesRelated productsInformation requestDescriptionLamin 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 informationSize200 µLApplicationClinical diagnostics| Life SciencesProduct typeReagents for researchReagent typeAntibodyCategoryLaminopathiesSubcategoryPolyclonal anti Farnesylated Prelamin A antibodyAntibody typePrimary antibodyImmunogenFarrnesylated prelamin A C-terminus peptideTechnical informationSize200 µLPurityPurified byaffinity ChromatographyFormLiquid. Supplied in 100mM sodium citrate, 50mM Tris and 0.05% v/v glycerol. Neutral pH.Shipping/storage conditionsShipped 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.UsageFor Laboratory Research Use OnlySourceRabbitSpecificityAnti 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 carboxymethylatedprelamin 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 AReact withCarboxymethylated prelamin Aharbouring the farnesyl residueRecommended dilutionsRecommended starting dilutions can vary lot-to-lot. Consult the suggested starting dilutions on the top of the page for lot specific values.DownloadDatasheet ReferencesNuclear Nox4 interaction with prelamin A is associated with nuclear redox control of stem cell agingPUBMED ID: 30362963Samp1 Mislocalization in Emery-Dreifuss Muscular DystrophyPUBMED ID: 30326651The relevance of prelamin A and RAD51 as molecular biomarkers in cervical cancerPUBMED ID: 29212225Statins and Histone Deacetylase Inhibitors Affect Lamin A/C - Histone Deacetylase 2 Interaction in Human CellsPUBMED ID: 30766871Related productsRecombinant Human ProgerinRecombinant human Progerin is a 614 amino acids protein with a molecular weight ofabout 68 kDa and is produced in E.coli.REP0051Recombinant Lamin ARecombinant human lamin A is a 645 amino acids protein with a molecular weight of 70 kDa and is produced in E.coli. Recombinant mature protein: human lamin A sequence, from 1 to 645 of Prelamin A aminoacidic sequenceREP0040Recombinant Lamin ARecombinant human lamin A is a 645 amino acids protein with a molecular weight of 70 kDa and is produced in E.coli. Recombinant mature protein: human lamin A sequence, from 1 to 645 of Prelamin A aminoacidic sequenceREP0041Recombinant Human Prelamin ARecombinant Prelamin A is a protein produced in E. coli. The Prelamin A is a 74kDa precursor of the nuclear lamin A protein, with N-terminal His tag and DDDDK tag.REP0038Recombinant Human Prelamin ARecombinant Prelamin A is a protein produced in E. coli. The Prelamin A is a 74kDa precursor of the nuclear lamin A protein, with N-terminal His tag and DDDDK tag.REP0039Recombinant Prelamin A R527HRecombinant human Prelamin A carrying the R527H mutation is a 645 amino acids protein with a molecular weight of 70 kDa and is produced in E.coli.REP0048Recombinant Rat ProgerinRecombinant human Progerin is a 614 amino acids protein with a molecular weight ofabout 68 kDa and is produced in E.coli.REP0047Rabbit Anti Human Lamin A Biotin conjugatedA-Type lamins are nuclear proteins required for the structural and functional integrity of the nucleus. In mammals, type A proteins are represented by lamins A and C, which are different transcripts arising from the same gene LMNA. This polyclonal antibody is able to detect full-lenght human prelamin A and Lamin A.ANT0079Rabbit Anti Human Lamin AA-Type lamins are nuclear proteins required for the structural and functional integrity of the nucleus. In mammals, type A proteins are represented by lamins A and C, which are different transcripts arising from the same gene LMNA. This polyclonal antibody is able to detect full-lenght prelamin A and human Lamin A.ANT0072Rabbit Anti Human Prelamin AA-Type lamins are nuclear proteins required for the structural and functional integrity of the nucleus. In mammals, type A proteins are represented by lamins A and C, which are different transcripts arising from the same gene LMNA. This polyclonal antibody is able to detect full-lenght human prelamin A.ANT0045Rabbit Anti Human Prelamin ABiotin conjugatedA-Type lamins are nuclear proteins required for the structural and functional integrity of the nucleus. In mammals, type A proteins are represented by lamins A and C, which are different transcripts arising from the same gene LMNA. This polyclonal antibody is able to detect full-lenght human prelamin A.The antibody Biotin conjugation garantees an improvement on the specificsignal and the best signal on semidirect immunologicalmethodsANT0078Information request Name * Last name * Email * Message I give my consent to direct marketing activities, including newsletters, in accordance with the Privacy Policy.I confirm that I have read the Privacy Policy, and I agree to the processing of my personal data..form-1{ font-size:14px; display: inline-block; }.form-1 textarea{ height:80px; }.textwidget input{ width: 155px; }input[type="checkbox"]{ width: 15px !important; }×
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