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Testing Data

Mouse anti-Human Progerin Monoclonal Antibody | anti-LMNA antibody

Mouse Anti-Progerin [13A4D4]

Gene Names
LMNA; FPL; IDC; LFP; CDDC; EMD2; FPLD; HGPS; LDP1; LMN1; LMNC; PRO1; CDCD1; CMD1A; FPLD2; LMNL1; CMT2B1; LGMD1B
Reactivity
Human
Applications
Western Blot, Immunofluorescence, Immunoprecipitation
Purity
Protein G
Synonyms
Progerin; Monoclonal Antibody; Mouse Anti-Progerin [13A4D4]; anti-LMNA antibody
Ordering
For Research Use Only!
Host
Mouse
Reactivity
Human
Clonality
Monoclonal
Isotype
IgG1 kappa
Clone Number
13A4D4
Specificity
Progerin. No cross reactivity with lamin A or Lamin C.
Purity/Purification
Protein G
Form/Format
Purified antibody (from supernatant) containing PBS + 0.1% sodium azide
Concentration
1mg/ml (varies by lot)
Sequence Length
614
Applicable Applications for anti-LMNA antibody
Western Blot (WB), Immunofluorescence (IF), Immunoprecipitation (IP)
Application Notes
Optimal antibody dilution should be determined by titration, however as a guideline try; WB: 1/500. Detects a band of approximately 70 kDa
Target Antigen
Progerin
Immunogen
Peptide from human Progerin
Myeloma/fusion Partners
X63-Ag8.653
Preparation and Storage
Store at +4°C for one month, or in small aliquots at -20°C for longer periods. Storage in frost free freezers is not recommended. Avoid repeated freezing and thawing as this may denature the antibody.

Testing Data

Testing Data
References
Liu GH et al. Recapitulation of premature ageing with iPSCs from Hutchinson-Gilford progeria syndrome. Nature 472:221-5 (2011). WB, ICC/IF ; Human PUBMED

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Official Full Name
progerin
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; lamin A/C-like 1; prelamin-A/C; renal carcinoma antigen NY-REN-32
UniProt Protein Name
Prelamin-A/C
Protein Family
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; cytosol; nucleus

Molecular Function: protein binding; structural molecule activity

Biological Process: mitotic nuclear envelope reassembly; muscle development; unfolded protein response; apoptosis; 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; establishment and/or maintenance of microtubule cytoskeleton polarity; cell structure disassembly during apoptosis

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

Research Articles on LMNA

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Product Notes

The LMNA lmna (Catalog #AAA375020) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Mouse Anti-Progerin [13A4D4] reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Progerin can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunofluorescence (IF), Immunoprecipitation (IP). Optimal antibody dilution should be determined by titration, however as a guideline try; WB: 1/500. Detects a band of approximately 70 kDa. Researchers should empirically determine the suitability of the LMNA lmna for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Progerin, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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