Mouse Anti-LMNA Monoclonal Antibody (VS7-0425-WR301) (CAT#: VS7-0425-WR301)

• Immunogen
• Recombinant human LMNA fragment expressed in E. Coli.

• Host Species
• Mouse

• Type
• Mouse IgG

• Specificity
• Human LMNA

• Species Reactivity
• Human, Mouse, Rat

• Molecule Class
• Type V Intermediate Filament Protein

• Applications
• WB, IHC-P, IF, ELISA

• Conjugate
• Unconjugated

• Related Disease
• Emery-Dreifuss muscular dystrophy 2, autosomal dominant (EDMD2); Emery-Dreifuss muscular dystrophy 3, autosomal recessive (EDMD3); Cardiomyopathy, dilated, 1A (CMD1A); Lipodystrophy, familial partial, 2 (FPLD2); Charcot-Marie-Tooth disease, axonal, 2B1 (CMT2B1); Hutchinson-Gilford progeria syndrome (HGPS); Cardiomyopathy, dilated, with hypergonadotropic hypogonadism (CMDHH); Mandibuloacral dysplasia with type A lipodystrophy (MADA); Restrictive dermopathy 2 (RSDM2); Heart-hand syndrome Slovenian type (HHS-Slovenian); Muscular dystrophy congenital LMNA-related (MDCL)

• Clonality
• Monoclonal

• Purity
• >95% as determined by SDS-PAGE and SEC-HPLC analysis

• Format
• Liquid

• Concentration
• 1 mg/ml

• Preservative
• 0.03% sodium azide

• Storage
• Centrifuge briefly prior to opening vial. Aliquot and store at -20°C for one year. Avoid repeated freeze/thaw cycles.

• Shipping
• Ice packs

• Application Notes
• Western Blot: 1:500 - 1:2000
  Immunohistochemistry: 1:200 - 1:1000
  Enzyme-linked Immunosorbent Assay: 1:10000

• Alternative Names
• LMNA; LFP; Lamin A/C; Limb Girdle Muscular Dystrophy 1B (Autosomal Dominant); CMT2B1; Prelamin-A/C; Progeria 1 (Hutchinson-Gilford Type); LMN1; IDC; LGMD1B; Lamin; FPL; LMNC; FPLD; CDDC; CMD1A; CDCD1; LDP1; FPLD2; LMNL1; PRO1; 70 KDa Lamin; Lamin A/C-Like

• Gene ID
• 4000

• UniProt ID
• P02545

• Long Name
• Lamin A

• Cellular Localization
• Intermediate filament; Nucleus

• Post Translation Modifications
• Acetylation; Glycoprotein; Isopeptide bond; Lipoprotein; Methylation; Phosphoprotein; Prenylation; Ubl conjugation

• Protein Refseq
• NP_001244303.1

• Function
• Lamin-A/C; Lamins are intermediate filament proteins that assemble into a filamentous meshwork, and which constitute the major components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane;; Lamins provide a framework for the nuclear envelope, bridging the nuclear envelope and chromatin, thereby playing an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics;; Lamin A and C also regulate matrix stiffness by conferring nuclear mechanical properties .; The structural integrity of the lamina is strictly controlled by the cell cycle, as seen by the disintegration and formation of the nuclear envelope in prophase and telophase, respectively;; Lamin A and C are present in equal amounts in the lamina of mammals. Also invoved in DNA repair: recruited by DNA repair proteins XRCC4 and IFFO1 to the DNA double-strand breaks (DSBs) to prevent chromosome translocation by immobilizing broken DNA ends;; 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; Prelamin-A/C can accelerate smooth muscle cell senescence (PubMed:20458013).; It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence

Isotype Control

• MRO-A1591-YJ
• Mouse IgG Isotype control, Polyclonal

Secondary Antibody

• 2ND-040CQ
• Goat Anti-Mouse IgG (H&L) Antibody

• 2ND-042CQ
• Rabbit Anti-Mouse IgG (H&L) Antibody

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