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Lamin A/C Antibody
Mouse monoclonal antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, IF |
|---|---|
| Primary Accession | P02545 |
| Reactivity | Bovine, Human, Mouse, Rat |
| Host | Mouse |
| Clonality | monoclonal |
| Isotype | IgG1 |
| Clone Names | 4C4 |
| Calculated MW | 64, 74 KDa |
| Gene ID | 4000 |
|---|---|
| Gene Name | LMNA |
| Other Names | Prelamin-A/C, Lamin-A/C, 70 kDa lamin, Renal carcinoma antigen NY-REN-32, LMNA, LMN1 |
| Target/Specificity | Recombinant full length human lamin C expressed in and purified from E. Coli. |
| Dilution | WB~~ 1:5000 IF~~ 1:1000 |
| Format | Affinity purified from tissue culture supernatant. |
| Antibody Specificity | Specific for the ~64 and 74k lamin A and C proteins. |
| Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
| Precautions | Lamin A/C Antibody is for research use only and not for use in diagnostic or therapeutic procedures. |
| Shipping | Blue Ice |
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Provided below are standard protocols that you may find useful for product applications.
Background
Lamins A and C are nuclear structural proteins that are part of the intermediate filament family and coded for by the same gene (LMNA). Lamins A and C are nearly identical except for their carboxy termini (McKeon et al., 1986). Mutations in the gene encoding lamins A/C have been shown to cause a variety of diseases including autosomal dominant Emery-Dreifuss muscular dystrophy (Bonne et al., 1995), autosomal dominant limb-girdle muscular dystrophy (Muchir et al., 2000) and Charcot-Marie-Tooth disorder type 2 (De Sandre-Giavonnoli et al., 2002).
References
Bonne G, Di Barletta MR, Varnous S, Bécane HM, Hammouda EH, Merlini L, Muntoni F, Greenberg CR, Gary F, Urtizberea JA, Duboc D, Fardeau M, Toniolo D, Schwartz K. (1999) Mutations in the gene encoding lamin A/C cause autosomal dominant Emery-Dreifuss muscular dystrophy. Nat Genet. 21(3):285-8
De Sandre-Giovannoli A, Chaouch M, Kozlov S, Vallat JM, Tazir M, Kassouri N, Szepetowski P, Hammadouche T, Vandenberghe A, Stewart CL, Grid D, Lévy N. (2002) Homozygous defects in LMNA, encoding lamin A/C nuclear-envelope proteins, cause autosomal recessive axonal neuropathy in human (Charcot-Marie-Tooth disorder type 2) and mouse. Am J Hum Genet. 70(3):726-36.
McKeon FD, Kirschner MW, Caput (1986) Homologies in both primary and secondary structure between nuclear envelope and intermediate filament proteins. Nature 319(6053):463-8.
Muchir A, Bonne G, van der Kooi AJ, van Meegen M, Baas F, Bolhuis PA, de Visser M, Schwartz K. (2000) Identification of mutations in the gene encoding lamins A/C in autosomal dominant limb girdle muscular dystrophy with atrioventricular conduction disturbances (LGMD1B). Hum Mol Genet. (9):1453-9.
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