Product Name:

LMNA-pS458


Product Number:

ab-pn965

Price:

Regular price
$98.00
Regular price
Sale price
$98.00

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Target Full Name: Prelamin-A/C

Target Alias: 70 kDa lamin; CMD1A; HGPS; LAMA; LGMD1B; LMN1; PRO1

Product Type Specific: LMNA phosphosite-specific antibody

Antibody Code: PN965

Antibody Target Type: Phosphosite-specific

Antibody Phosphosite: S458

Protein UniProt: P02545

Protein SigNET: LMNA

Antibody Type: Polyclonal

Antibody Host Species: Rabbit

Antibody Immunogen Source: Synthetic phosphopeptide patterned after human LMNA

Antibody Immunogen Sequence: LRNK(pS)NED(βA)C

Antibody Immunogen Description: Corresponds to amino acid residues L454 to D461. The effect of Lamin A,C S458 phosphorylation is unclear. It is a prominent site of in vivo phosphorylation based on ≥45 mass spectrometry reports documented in PhosphoSitePlus.

Production Method: The immunizing peptide was produced by solid phase synthesis on a multipep peptide synthesizer and purified by reverse-phase hplc chromatography. Purity was assessed by analytical hplc and the amino acid sequence confirmed by mass spectrometry analysis. This peptide was coupled to KLH prior to immunization into rabbits. New Zealand White rabbits were subcutaneously injected with KLH-coupled immunizing peptide every 4 weeks for 4 months. The sera from these animals was applied onto an agarose column to which the immunogen peptide was thio-linked. Antibody was eluted from the column with 0.1 M glycine, pH 2.5. Subsequently, the antibody solution was neutralized to pH 7.0 with saturated Tris.

Antibody Modification: Unconjugated. Contact KInexus if you are interest in having the antibody biotinylated or coupled with fluorescent dyes.

Antibody Concentration: 0.3 mg/ml

Storage Buffer: Phosphate buffered saline pH 7.4, 0.05% Thimerasol

Storage Conditions: For long term storage, keep frozen at -40°C or lower. Stock solution can be kept at +4°C for more than 3 months. Avoid repeated freeze-thaw cycles.

Product Use: Western blotting | Antibody microarray

Antibody Dilution Recommended: 2 µg/ml for immunoblotting

Antibody Species Reactivity: This antibody detects the target phosphoprotein in the following species due to conservation of amino acid sequence: Human | Chimpanzee | Rhesus macaque | Dog | Rat | Mouse | Platypus | Frog.

Scientific Background: LMNA (Prelamin-A/C) are cytoskeletal components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which provides 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. LMNA belongs to the intermediate filament family. LMNA functions in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. During mitosis, the nuclear lamina is reversibly disassembled following phosphorylation. It is required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation. It is required for osteoblastogenesis and bone formation. It also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone. It is required for cardiac homeostasis. Prelamin-A/C can accelerate smooth muscle cell senescence. It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence. It is also involved in DNA repair and is recruited by DNA repair proteins XRCC4 and IFFO1 to the DNA double-strand breaks (DSBs) to prevent chromosome translocation by immobilizing broken DNA ends. In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial VSMCs from aged individuals and in atherosclerotic lesions, where it often colocalizes with senescent and degenerate VSMCs. Prelamin-A/C expression increases with age and disease. In normal aging, the accumulation of prelamin-A/C is caused in part by the down-regulation of ZMPSTE24/FACE1 in response to oxidative stress. Mutations in the LMNA gene cause a range of diseases known as laminopathies, including cardiomyopathy, muscular dystrophies (Emery-Dreifuss muscular dystrophy and Limb-girdle muscular dystrophy), Familial partial lipodystrophy, and premature aging syndromes (progeria (Hutchinson-Gilford progeria syndrome (HGPS)). This description may include information annotated by UniProt and/or Google AI.