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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 IHC-p, ELISA, IF, 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 - LMNA - HGPS - MADA - LMN1 - CMD1A - LGMD1B - PRO1 - LMNL1 - lamin A/C
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).
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 (Human) / 16905 (Mouse) / 60374 (Rat)
Gene Symbol LMNA
Molecular Weight 75/65 kDa
Database Links Human UniProt/Swiss-Prot:P02545 Mouse UniPort/Swiss-Prot: P48678 Rat UniProt/Swiss-Port: G3V8L3 Human Entrez Gene: 4000 Mouse Entrez Gene: 16905 Rat Entrez Gene: Rn.44161
Alternative Names LMNA / lamin A /C / 70 kDa lamin antibody / AS27_05922 antibody / CB1_000161025 antibody / cdcd1 antibody / cddc antibody / CMD1A antibody / CMT2B1 antibody / D623_10031981 antibody / Dhe antibody / EGK_01409 antibody / EMD2 antibody / fk66d12 antibody / fpl antibody / FPLD antibody / FPLD2 antibody / H920_13701 antibody / HGPS antibody / hypothetical protein antibody / I79_009616 antibody / IDC antibody / LAM antibody / lama antibody / lamin antibody / lamin A antibody / Lamin-A antibody / lamin-A /C antibody / lamin A /C-like 1 antibody / lamin C antibody / lamin C2 antibody / lamin-L(III)-like antibody / LDP1 antibody / LFP antibody / LGMD1B antibody / lmn1 antibody / lmna-A antibody / LMNC antibody / LMNL1 antibody / N300_10932 antibody / N301_01214 antibody / N302_02193 antibody / N305_13421 antibody / N327_06812 antibody / PAL_GLEAN10005359 antibody / prelamin-A /C antibody / pro1 antibody / renal carcinoma antigen NY-REN-32 antibody / TREES_T100016132 antibody / Y956_00433 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. Note=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 degener

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