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Products Polyclonal Anti-VCP
| Catalogue number: | PA2137 |
| Price: | $200.00 |
| Reactivities: | Human, Mouse, Rat |
| Applications: | Immunocytochemistry, Immunohistochemistry, Immunohistochemistry - frozen, Western Blot |
| Size: | 100ug/vial |
| Gene: | VCP |
| Swiss prot: | P55072 |
| Form: | Lyophilized |
| Format: | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. |
| Storage temp: | At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time.Avoid repeated freezing and thawing. |
| Scientific background: | Valosin-containing protein also called CDC48 is an enzyme that in humans is encoded by the VCP gene. It is a member of the AAA+ (ATPase associated with various activities) protein family. The VCP gene maps to chromosome 9p13.3. It is necessary for the fragmentation of Golgi stacks during mitosis and for their reassembly after mitosis. It is involved in the formation of the transitional endoplasmic reticulum. This gene plays a role in vesicle transport and fusion, 26S proteasome function, and assembly of peroxisomes. It also involved in DNA damage response: recruited to double-strand breaks (DSBs) sites in a RNF8- and RNF168-dependent manner and promotes the recruitment of TP53BP1 at DNA damage sites. |
| References: | 1. Custer, S. K., Neumann, M., Lu, H., Wright, A. C., Taylor, J. P. Transgenic mice expressing mutant forms VCP recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone. Hum. Molec. Genet. 19: 1741-1755, 2010. 2. Haubenberger, D., Bittner, R. E., Rauch-Shorny, S., Zimprich, F., Mannhalter, C., Wagner, L., Mineva, I., Vass, K., Auff, E., Zimprich, A. Inclusion body myopathy and Paget disease is linked to a novel mutation in the VCP gene. Neurology 65: 1304-1305, 2005. 3. Mueller, B., Klemm, E. J., Spooner, E., Claessen, J. H., Ploegh, H. L. SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteins. Proc. Nat. Acad. Sci. 105: 12325-12330, 2008. 4. Sacconi, S., Camano, P., de Greef, J. C., Lemmers, R. J. L. F., Salviati, L., Boileau, P., Lopez de Munain Arregui, A., van der Maarel, S. M., Desnuelle, C. Patients with a phenotype consistent with facioscapulohumeral muscular dystrophy display genetic and epigenetic heterogeneity. J. Med. Genet. 49: 41-46, 2012. |
| Additional info: | A synthetic peptide corresponding to a sequence at the C-terminal of human VCP, identical to the related rat and mouse sequences. |
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