Mouse Anti-LMNA Recombinant Antibody (VS3-CJ299) (CAT#: VS3-CJ299)

• Immunogen
• Recombinant protein

• Host Species
• Mouse

• Type
• Mouse IgG1, κ

• Specificity
• Human, Mouse and Rat LMNA

• Species Reactivity
• Human, Mouse, Rat

• Applications
• WB

• Conjugate
• Unconjugated

• Purification
• Protein A/G affinity purified

• Purity
• >95% as determined by SDS-PAGE

• Buffer
• PBS

• Preservative
• 0.09% sodium azide

• Storage
• Store at 4°C for short term. Aliquot and store at -20°C for long term. Avoid repeated freeze/thaw cycles.

• Shipping
• Ice packs

• Application Notes
• This antibody has been tested for use in Western Blot (1:4000).

• Alternative Names
• FPL; IDC; LFP; CDDC; EMD2; FPLD; HGPS; LDP1; LMN1; LMNC; MADA; PRO1; CDCD1; CMD1A; FPLD2; LMNL1; CMT2B1; LGMD1B

• Gene ID
• 4000

• UniProt ID
• P02545

• Sequence Similarities
• Belongs to the intermediate filament family.

• Cellular Localization
• Intermediate filament, Nucleus

• Post Translation Modifications
• 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 (PubMed:31548606).
  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.

• Protein Refseq
• NP_001244303.1; NP_001269553.1; NP_001269554.1

• 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 (PubMed:31548606).
  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 (PubMed:10080180, PubMed:22431096, PubMed:10814726, PubMed:11799477, PubMed:18551513).
  Required for osteoblastogenesis and bone formation (PubMed:12075506, PubMed:15317753, PubMed:18611980).
  Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone (PubMed:10587585).
  Required for cardiac homeostasis (PubMed:10580070, PubMed:12927431, PubMed:18611980, PubMed:23666920).
  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.

Recommended Dilution Buffer

• MRO-0253YJ
• Reconstitution Buffer PBS

Select a product category from the dropdown menu below to view related products.