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GeneBio Systems

Anti-Lamin A+C/LMNA Antibody Picoband® (monoclonal, 5F3C12)

Anti-Lamin A+C/LMNA Antibody Picoband® (monoclonal, 5F3C12)

SKU:M00438-6

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Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human

Gene Name: LMNA

Specificity:

Background: Lamins are structural protein components of the nuclear lamina, a protein network underlying the inner nuclear membrane that determines nuclear shape and size. There are three types of lamins, A,B and C. The lamin A/C (LMNA) gene contains 12 exons. Alternative splicing within exon 10 gives rise to two different mRNAs that code for pre-lamin A and lamin C. Lamin A/C is mapped to 1q21.2-q21.3 and mutations in this gene cause a variety of human diseases including Emery-Dreifuss muscular dystrophy, dilated cardiomyopathy, and Hutchinson-Gilford progeria syndrome. Lamin A/C deficiency is thus associated with both defective nuclear mechanics and impaired mechanically activated gene transcription.

Immunogen: E.coli-derived human Lamin A/C recombinant protein (Position: Y481-Y646). Human Lamin A/C shares 90% and 92% amino acid (aa) sequence identity with mouse and rat Lamin A/C, respectively.

Clonality: Monoclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Lammerding, J.; Schulze, P. C.; Takahashi, T.; Kozlov, S.; Sullivan, T.; Kamm, R. D.; Stewart, C. L.; Lee, R. T. : Lamin A/C deficiency causes defective nuclear mechanics and mechanotransduction. J. Clin. Invest. 113: 370-378, 2004. 2. Lin, F.; Worman, H. J. : Structural organization of the human gene encoding nuclear lamin A and nuclear lamin C. J. Biol. Chem. 268: 16321-16326, 1993. 3. Wydner, K. L.; McNeil, J. A.; Lin, F.; Worman, H. J.; Lawrence, J. B. : Chromosomal assignment of human nuclear envelope protein genes LMNA, LMNB1, and LBR by fluorescence in situ hybridization. Genomics 32: 474-478, 1996.

Uniprot ID: P02545

Host: Mouse

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: IgG2b

Phospho_site:

Clone Number: Clone: 5F3C12

Observed Molecular Weight: 74 kDa

Calculated Molecular Weight:

Gene ID: 4000

Protein Name: Prelamin-A/C [Cleaved into: Lamin-A/C

Gene Full Name: lamin A/C

Synonyms: 70 kDa lamin; CDCD1; CDDC; CMD1A; CMT2B1; EMD2; FPL; FPLD; HGPS; IDC; lamin A; lamin A/C; LDP1; LFP; LGMD1B; LMN1; LMNA; LMNC; Prelamin A/C; PRO1; progerin

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