Anti-Phospho-LMNA-S22 antibody

Clonality:	Polyclonal
Conjugation:	Unconjugated
Isotype:	IgG
Formulation:	PBS with 0.01% Thimerosal, 50% Glycerol, pH7.3.
Purification:	Affinity purification
Dilution Range:	WB 1:500-1:2000 IP 1:50-1:100
Storage Instruction:	Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles.

Gene Symbol:	LMNA
Gene ID:	4000
Uniprot ID:	LMNA_HUMAN
Immunogen:	A synthetic phosphorylated peptide around S22 of human Lamin A (NP_005563.1).
Immunogen Sequence:	PLSPT

Tissue Specificity	In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial vascular smooth muscle cells (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.
Post Translational Modifications	Proteolytic cleavage of the C-terminal of 18 residues of prelamin-A/C results in the production of lamin-A/C. The prelamin-A/C maturation pathway includes farnesylation of CAAX motif by protein farnesyltransferase (FNTA and FNTB), removal of the last three amino acids (-AAX) by RCE1/FACE2 and/or ZMPSTE24, methylation of the C-terminal cysteine by ICMT and endoproteolytic removal of the last 15 C-terminal amino acids by ZMPSTE24. Proteolytic cleavage requires prior farnesylation and methylation, and absence of these blocks cleavage. Farnesylation of prelamin-A/C facilitates nuclear envelope targeting. Increased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations. Phosphorylation status of S-22 determines its localization between double-strand break (DSB) sites and the nuclear matrix. Sumoylation is necessary for the localization to the nuclear envelope.
Function	Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to 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. Recruited by DNA repair proteins XRCC4 and IFFO1 to the DNA double-strand breaks (DSBs) to prevent chromosome translocation by immobilizing broken DNA ends. Plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation. Required for osteoblastogenesis and bone formation. Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone. 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.
Protein Name	Prelamin-A/C Cleaved Into - Lamin-A/C 70 Kda Lamin Renal Carcinoma Antigen Ny-Ren-32
Database Links	Reactome: R-HSA-1221632 P02545-2 Reactome: R-HSA-2980766 Reactome: R-HSA-2995383 Reactome: R-HSA-352238 P02545-1 Reactome: R-HSA-381038 Reactome: R-HSA-4419969 Reactome: R-HSA-6802952 Reactome: R-HSA-8862803 P02545-1
Cellular Localisation	Nucleus Nucleus Envelope Nucleus Lamina Nucleoplasm Nucleus Matrix Farnesylation Of Prelamin-A/C Facilitates Nuclear Envelope Targeting And Subsequent Cleavage By Zmpste24/Face1 To Remove The Farnesyl Group Produces Mature Lamin-A/C Which Can Then Be Inserted Into The Nuclear Lamina Emd Is Required For Proper Localization Of Non-Farnesylated Prelamin-A/C Isoform C: Nucleus Speckle
Alternative Antibody Names	Anti-Prelamin-A/C Cleaved Into - Lamin-A/C antibody Anti-70 Kda Lamin antibody Anti-Renal Carcinoma Antigen Ny-Ren-32 antibody Anti-LMNA antibody Anti-LMN1 antibody

Information sourced from Uniprot.org

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