일반 설명
Prelamin-A/C (UniProt: P02545) is encoded by the LMNA (also known as LMN1) gene (Gene ID: 4000) in human. It is cleaved into Lamin-A/C (also known as 70 kDa Lamin, Renal carcinoma antigen NY-REN-32). Lamins are components of the nuclear lamina that 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. They play an important role in nuclear assembly, chromatin organization, nuclear membrane, and telomere dynamics. Human lamin A and lamin C are identical in their first 566 amino acids with lamin C having six unique carboxyl-terminal amino acids. Lamin A is initially synthesized as prelamin A that undergoes several modifications in the carboxyl terminal region that allow incorporation of prelamin A into the nuclear envelope and its subsequent processing into the mature lamin A. Prelamin A contains a CAAX motif at its carboxyl-terminus, which signals a series of posttranslational enzymatic modifications, including farnesylation of the cysteine, cleavage of the AAX, and methylation of the carboxyl group of the newly exposed farnesylcysteine. Cleavage of 15 residues (aa 647-662) by ZMPSTE24 generates the final protein product. Unlike mature lamin A, prelamin A accumulates as discrete and localized foci at the nuclear periphery. Prelamin can accelerate smooth muscle cell senescence. It can act to disrupt mitosis and induce DNA damage in vascular smooth muscle cells, leading to mitotic failure, genomic instability, and premature senescence. Mutations in LMNA gene are known to cause Emery-Dreifuss muscular dystrophy that is characterized by weakness and atrophy of muscle without involvement of the nervous system. Some mutations have also been linked to familial type of lipodystrophy characterized by the loss of subcutaneous adipose tissue in the lower parts of the body. (Ref.: Casasola, A., et al. (2016). Nucleus 7(1); 84-102; Bonne, G., et al (1999). Nat. Genet. 21(3); 285-288).
특이성
Clone 3E12 is a mouse monoclonal antibody that detects Lamin A. It targets an epitope within 6 amino acids from the C-terminal region.
면역원
KLH-conjugated linear peptide corresponding to 6 amino acids from the C-terminal region of human mature Lamin A.
애플리케이션
Quality Control Testing
Evaluated by Western Blotting in HAP1 cell lysate.
Western Blotting Analysis: A 1:1,000 dilution of this antibody detected mature Lamin A in lysates from HAP1 cells, but not in lysates from HAP1 cells with ZMPSTE24 knockouts or LMNA knockouts.
Tested Applications
Immunocytochemistry Analysis: A representative lot detected Mature Lamin A in wild-type HAP1 cells , but not in cells with ZMPSTE24 knockout. (Data courtesy of Dr. Stefan Schüchner, Dr. Egon Ogris, Max Perutz Labs, Medical University of Vienna, Austria).
Immunoprecipitation Analysis: A representative lot immunoprecipitated Mature Lamin A in lysates from HAP1 cells. (Data courtesy of Dr. Stefan Schüchner and Dr. Egon Ogris, Max Perutz Labs, Medical University of Vienna, Austria).
Western Blotting Analysis: A representative lot detected Mature Lamin A in lysates from wild-type HAP1 cells, but not in cells with ZMPSTE24 or LMNA knockouts. (Data courtesy of Dr. Stefan Schüchner and Dr. Egon Ogris, Max Perutz Labs, Medical University of Vienna, Austria).
Note: Actual optimal working dilutions must be determined by end user as specimens, and experimental conditions may vary with the end user
Anti-Mature Lamin A, clone 3E12, Cat. No. MABT1577, is a mouse monoclonal antibody that detects mature Lamin A and is tested for use in Immunocytochemistry, Immunoprecipitation, and Western Blotting.
물리적 형태
Purified mouse monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
저장 및 안정성
Recommended storage: +2°C to +8°C.
기타 정보
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
면책조항
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.