CDCD1; CMD1A; CMT2B1; EMD2; FPL; FPLD; HGPS; IDC; LDP1; LFP; LGMD1B; LMN1; LMNC; PRO1; Cardiomyopathy Dilated 1A; Limb Girdle Muscular Dystrophy 1B; Progeria 1

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

Mouse

The antibody is a mouse monoclonal antibody raised against LMNA. It has been selected for its ability to recognize LMNA in immunohistochemical staining and western blotting.

Protein A + Protein G affinity chromatography

Liquid

500ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-LMNA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Control Antibody; Metabolic pathway

Western Blot (WB)

Loading Control of WB.

Western Blot: Sample: Lane1: Rat Uterus lysate; Lane2: 3T3-L1 cell lysate

69,249 Da

lamin A/C

lamin

Prelamin-A/C

LMN1??[Similar Products]

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]

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: cytosol; extracellular matrix; lamin filament; nuclear envelope; nuclear membrane; nuclear speck; nucleoplasm; nucleus

Molecular Function: protein binding; structural molecule activity

Biological Process: cellular response to hypoxia; establishment or maintenance of microtubule cytoskeleton polarity; IRE1-mediated unfolded protein response; mitotic nuclear envelope disassembly; mitotic nuclear envelope reassembly; negative regulation of cell proliferation; negative regulation of extrinsic apoptotic signaling pathway; negative regulation of mesenchymal cell proliferation; negative regulation of release of cytochrome c from mitochondria; positive regulation of cell aging; protein localization to nucleus; regulation of cell migration; regulation of protein localization to nucleus; sterol regulatory element binding protein import into nucleus; ventricular cardiac muscle cell development

Disease: Cardiomyopathy, Dilated, 1a; Cardiomyopathy, Dilated, With Hypergonadotropic Hypogonadism; Charcot-marie-tooth Disease, Axonal, Type 2b1; Emery-dreifuss Muscular Dystrophy 2, Autosomal Dominant; Emery-dreifuss Muscular Dystrophy 3, Autosomal Recessive; Heart-hand Syndrome, Slovenian Type; Hutchinson-gilford Progeria Syndrome; Lipodystrophy, Familial Partial, Type 2; Mandibuloacral Dysplasia With Type A Lipodystrophy; Muscular Dystrophy, Congenital, Lmna-related; Muscular Dystrophy, Limb-girdle, Type 1b; Restrictive Dermopathy, Lethal

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