Rabbit Lamin A/C Polyclonal Antibody | anti-LMNA antibody

Lamin A/C Antibody

Cat. Num. AAA9613773

• Gene Names: LMNA; FPL; IDC; LFP; CDDC; EMD2; FPLD; HGPS; LDP1; LMN1; LMNC; PRO1; CDCD1; CMD1A; FPLD2; LMNL1; CMT2B1; LGMD1B
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunofluorescence, Immunocytochemistry, ELISA
• Purity: The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin
• Synonyms: Lamin A/C; Polyclonal Antibody; Lamin A/C Antibody; 70kDa lamin; Cardiomyopathy dilated 1A (autosomal dominant); CDCD1; CDDC; CMD1A; CMT2B1; EMD2; FPL; FPLD; FPLD2; HGPS; IDC; Lamin A; Lamin A/C like 1; Lamin; Lamin C; Lamin-A/C; LDP1; LFP; LGMD1B; Limb girdle muscular dystrophy 1B (autosomal dominant); LMN 1; LMN A; LMN C; LMN1; LMNA; LMNA_HUMAN; LMNC; LMNL1; Prelamin A/C; PRO1; Renal carcinoma antigen NY REN 32; Renal carcinoma antigen NY-REN-32; Renal carcinoma antigen NYREN32; anti-LMNA antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Lamin A/C Antibody detects endogenous levels of total Lamin A/C.; 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.
• Purity/Purification: The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin
• Form/Format: Liquid. Rabbit IgG in phosphate buffered saline, pH7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1mg/ml (varies by lot)

• Applicable Applications for anti-LMNA antibody: Western Blot (WB), Immunofluorescence (IF), Immunocytochemistry (ICC), Peptide ELISA (EIA)
• Application Notes: IF/ICC: 1:100-1:500; WB: 1:500-1:2000; Peptide ELISA: 1:20,000-1:40,000
• Immunogen: A synthesized peptide derived from human Lamin A/C.
• Conjugation: Unconjugated
• Fragment: Fab fragment
• 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.
• Subunit Structure: Homodimer of lamin A and lamin C. Interacts with lamin-associated polypeptides IA, IB and TMPO-alpha, RB1 and with emerin. Interacts with SREBF1, SREBF2, SUN2 and TMEM43. Interacts with TMEM201 (By similarity). Proteolytically processed isoform A interacts with NARF. Interacts with SUN1. Prelamin-A/C interacts with EMD. Interacts with MLIP. Interacts with DMPK; may regulate nuclear envelope stability. Interacts with SUV39H1; the interaction increases stability of SUV39H1. Interacts with SYNE2. Interacts with ITSN1 isoform 2.
• Similarity: Belongs to the intermediate filament family.
• Subcellular Location: Nucleus. Nucleus envelope. Nucleus lamina. Nucleus>Nucleoplasm.; Note: 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.Nucleus speckle.
• Preparation and Storage: Store at -20 degree C. Stable for 12 months from date of receipt.

Western Blot (WB)

(Western blot analysis of extracts from various samples, using Lamin A/C Antibody.Lane 1: MCF7 cells(UV treatment), blocked with antigen-specific peptides,Lane 2: MCF7 cells(UV treatment),Lane 3: Hela cells(LPS treatment),Lane 4: Hela cells(heat shock treatment).)

Immunofluorescence (IF)/Immunocytochemistry (ICC)

(Staining A549 cells by IF/ICC. The samples were fixed with PFA and permeabilized in 0.1% Triton X-100,then blocked in 10% serum for 45 minutes at 25 degree C. Samples were then incubated with primary Ab(AF6752) and mouse anti-beta tubulin Ab(T0023) for 1 hour at 37 degree C. An AlexaFluor594 conjugated goat anti-rabbit IgG(H+L) Ab(Red) and an AlexaFluor488 conjugated goat anti-mouse IgG(H+L) Ab(Green) were used as the secondary antibody.The nuclear counter stain is DAPI (blue).)

Related Product Information for anti-LMNA antibody

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. 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.

NCBI and Uniprot Product Information

• NCBI GI #: 383792150
• NCBI GeneID: 4000
• NCBI Accession #: NP_001244303.1
• NCBI GenBank Nucleotide #: NM_001257374.1
• UniProt Accession #: P02545; P02546; Q5TCJ2; Q5TCJ3; Q969I8; Q96JA2; B4DI32; D3DVB0; E7EUI9
• Molecular Weight: 74,65kDa
• NCBI Official Full Name: lamin isoform D
• NCBI Official Synonym Full Names: lamin A/C
• NCBI Official Symbol: LMNA
• NCBI Official Synonym Symbols: FPL; IDC; LFP; CDDC; EMD2; FPLD; HGPS; LDP1; LMN1; LMNC; PRO1; CDCD1; CMD1A; FPLD2; LMNL1; CMT2B1; LGMD1B
• NCBI Protein Information: lamin; 70 kDa lamin; prelamin-A/C; lamin A/C-like 1; renal carcinoma antigen NY-REN-32
• UniProt Protein Name: Prelamin-A/C
• Protein Family: Lamin
• UniProt Gene Name: LMNA
• UniProt Synonym Gene Names: LMN1
• UniProt Entry Name: LMNA_HUMAN

NCBI Description

The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. The lamin family of proteins make up the matrix and are highly conserved in evolution. During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. Vertebrate lamins consist of two types, A and B. Alternative splicing results in multiple transcript variants. Mutations in this gene lead to several diseases: Emery-Dreifuss muscular dystrophy, familial partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease, and Hutchinson-Gilford progeria syndrome. [provided by RefSeq, Apr 2012]

Uniprot Description

lamin A/C: nuclear lamins are intermediate filament proteins that constitute the lattice-like matrix at the inner face of the nuclear membrane that underlies the nuclear envelop. The lamins, highly conserved throughout evolution, are encoded by three genes in the human: LMNA, LMNB1, and LMNB2. The A-type lamins (lamin A/C) are developmentally regulated and are generally expressed in differentiated cells. The anchoring of chromatin to the nuclear lamina is involved in the control of gene expression and in DNA replication and repair. During mitosis, the nuclear lamina is reversibly disassembled as the lamin proteins are phosphorylated. Cleaved by caspase-6 during apoptosis into a 40-45 kDa and a28 kDa fragment.

Protein type: Cytoskeletal

Chromosomal Location of Human Ortholog: 1q22

Cellular Component: nucleoplasm; nuclear lamina; nuclear membrane; perinuclear region of cytoplasm; cytoplasm; lamin filament; intermediate filament; nuclear speck; nuclear envelope; nucleus; cytosol

Molecular Function: protein binding; structural molecule activity

Biological Process: muscle development; mitotic nuclear envelope reassembly; apoptosis; unfolded protein response; ventricular cardiac muscle cell development; mitotic nuclear envelope disassembly; regulation of cell migration; cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; sterol regulatory element binding protein nuclear translocation; mitotic cell cycle; cell structure disassembly during apoptosis; establishment and/or maintenance of microtubule cytoskeleton polarity

Disease: Heart-hand Syndrome, Slovenian Type; Emery-dreifuss Muscular Dystrophy 2, Autosomal Dominant; Restrictive Dermopathy, Lethal; Muscular Dystrophy, Congenital, Lmna-related; Mandibuloacral Dysplasia With Type A Lipodystrophy; Emery-dreifuss Muscular Dystrophy 3, Autosomal Recessive; Charcot-marie-tooth Disease, Axonal, Type 2b1; Lipodystrophy, Familial Partial, Type 2; Hutchinson-gilford Progeria Syndrome; Cardiomyopathy, Dilated, 1a; Muscular Dystrophy, Limb-girdle, Type 1b; Cardiomyopathy, Dilated, With Hypergonadotropic Hypogonadism