Mouse Anti-Lamin A Monoclonal Antibody (CBP2333)

Product Overview

Description

Lamins A and C are structural components of the lamina, a scaffold of proteins localized to the nuclear inner membrane, and contribute to nuclear membrane dynamics and chromatin structure. Mutations in the LMNA gene have been linked to multiple laminopathy disorders, including Hutchinson-Gilford progeria syndrome (HGPS), an autosomal dominant disorder characterized by the appearance of accelerated and dramatic aging. Patients with HGPS have a mutation in the LMNA gene that results in aberrant splicing and production of the toxic, truncated Lamin A protein progerin that functions in a dominant negative pattern.

Immunogen

GST fused to C-terminal fragment of Lamin A (amino acid residues 422-664)

Species Reactivity

Human; Mouse

Clonality

Monoclonal

Host Species

Mouse

Isotype

IgG3, κ

Clone Number

CBP2333

Applications

Application Notes

WB (0.1-1.0 µg per mL)

Relevant Diseases

Brain Tumors; Alzheimer's Disease; Parkinson's Disease; Neurocutaneous Syndromes

Research Areas

Neuroinflammation

Conjugation

Unconjugated

Product Properties

Form

Liquid

Formulation

PBS, pH 7.2, containing 0.09% sodium azide.

Concentration

0.5 mg/mL

Purification

Affinity chromatography

Purity

>95%, as determined by Coomassie stained SDS-PAGE.

Shipping

The product is shipped at 4°C. Upon receipt, store it immediately at the recommended temperature.

Storage

Store at -20°C. Upon initial thawing, apportion into working aliquots and store at -20°C. Avoid repeated freeze-thaw cycles to prevent denaturing the antibody.

Handling Advice

Store at -20°C. Upon initial thawing, apportion into working aliquots and store at -20°C. Avoid repeated freeze-thaw cycles to prevent denaturing the antibody.

Research Use Only

For research use only, not for diagnostic or therapeutic use.

Target

LMNA

Official Name

LMNA

Full Name

Lamin A/C

Alternative Names

LMNA; CDCD1; CDDC; CMD1A; CMT2B1; EMD2; FPL; FPLD; FPLD2; HGPS; IDC; LDP1; LFP; LGMD1B; LMN1; LMNC; LMNL1; PRO1; lamin A/C; MADA

Gene ID

4000 (Human); 16905 (Mouse)

Uniprot ID

P02545 (Human); P48678 (Mouse)

References

1. Machiels B, et al. 1996. J. Biol. Chem. 271:9249.
2. Barton R and Worman HJ. 1999. J. Biol. Chem. 274:30008.
3. Gruenbaum Y, et al. 2000. J. Struct. Biol. 129:313.
4. Stierlé V, et al. 2003. Biochemistry 42:4819.

Publications