Anti-Lamin A/C antibody

[STJ93885] Download PDF Print Data Sheet

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Product name Anti-Lamin A/C antibody
Short Description Rabbit polyclonal to Lamin A/C.
Description Lamin A/C is a protein encoded by the LMNA gene which is approximately 74,1 kDa. Lamin A/C is localised to the nucleus and nuclear envelope. It is involved in the apoptosis and survival caspase cascade, mitotic cell cycle, unfolded protein response and arrhythmogenic right ventricular cardiomyopathy. It plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics and is required for normal development of the peripheral nervous system and skeletal muscle. It is also required for osteoblasto genesis and bone formation. Lamin A/C is expressed in the arteries. Mutations in the LMNA gene can result in Emery-Dreifuss muscular dystrophy 2. The antibody STJ93885 was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. This polyclonal antibody detects endogenous levels of Lamin A/C protein only when phosphorylated at S392.
Applications ELISA, IF, IHC, WB
Dilution range WB 1:500-1:2000
IHC 1:100-1:300
IF 1:200-1:1000
ELISA 1:20000
Specificity Lamin A/C Polyclonal Antibody detects endogenous levels of Lamin A/C protein.
Protein Name Prelamin-A/C
70 kDa lamin
Renal carcinoma antigen NY-REN-32
Immunogen Synthesized peptide derived from human Lamin A/C around the non-phosphorylation site of S392.
Immunogen Region 330-410 aa
Storage Instruction Store at -20°C, and avoid repeat freeze-thaw cycles.
Note For Research Use Only (RUO).
Validated Application IF IHC WB
Host Rabbit
Clonality Polyclonal
Reactivity Human, Mouse, Rat
Conjugation Unconjugated
Concentration 1 mg/ml
Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Isotype IgG
Formulation Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Gene ID 4000
Gene Symbol LMNA
Molecular Weight 75/65 kDa
Database Links HGNC:6636
Alternative Names Prelamin-A/C antibody
Lamin-A/C antibody
70 kDa lamin antibody
Renal carcinoma antigen NY-REN-32 antibody
LMN1 antibody
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. 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.; 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.
Post-translational Modifications Increased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations. 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, ZMPSTE24/FACE1 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. Proteolytic cleavage requires prior farnesylation and methylation, and absence of these blocks cleavage. Sumoylation is necessary for the localization to the nuclear envelope. Farnesylation of prelamin-A/C facilitates nuclear envelope targeting.
Cellular Localization Nucleus. Nucleus envelope. Nucleus lamina. Nucleus, nucleoplasm. Farnesylation of prelamin-A/C facilitates nuclear envelope targeting and subsequent cleaveage 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
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.
Swiss-Prot Key P02545_HUMAN
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