ELP1/IKBKAP Antibody #5071
- WB
- IP
Supporting Data
| REACTIVITY | H Mk |
| SENSITIVITY | Endogenous |
| MW (kDa) | 150 |
| SOURCE | Rabbit |
Application Key:
- WB-Western Blotting
- IP-Immunoprecipitation
Species Cross-Reactivity Key:
- H-Human
- Mk-Monkey
Product Information
Product Usage Information
| Application | Dilution |
|---|---|
| Western Blotting | 1:1000 |
| Immunoprecipitation | 1:50 |
Storage
Protocol
Specificity / Sensitivity
Species Reactivity:
Source / Purification
Background
The ELP1/IKBKAP subunit of Elongator was initially thought to function as a scaffolding protein within the NFκB signaling pathway (7). It contains several WD40 domains and is critical for the formation of the Elongator complex (8). Investigators have determined that mutations in ELP1 are the cause of Familial Dysautonomia (FD), an autosomal recessive neurodegenerative disorder (9). Research studies have demonstrated that defects in Elongator function upon ELP1 mutation affect transcription elongation of several genes involved in cell motility and neuronal development that may be the underlying cause of the neuropathology of FD patients (10,11).
- Otero, G. et al. (1999) Mol Cell 3, 109-18.
- Creppe, C. and Buschbeck, M. (2011) J Biomed Biotechnol 2011, 924898.
- Wittschieben, B.O. et al. (1999) Mol Cell 4, 123-8.
- Hawkes, N.A. et al. (2002) J Biol Chem 277, 3047-52.
- Kim, J.H. et al. (2002) Proc Natl Acad Sci USA 99, 1241-6.
- Creppe, C. et al. (2009) Cell 136, 551-64.
- Cohen, L. et al. (1998) Nature 395, 292-6.
- Frohloff, F. et al. (2003) J Biol Chem 278, 956-61.
- Anderson, S.L. et al. (2001) Am J Hum Genet 68, 753-8.
- Close, P. et al. (2006) Mol Cell 22, 521-31.
- Cohen-Kupiec, R. et al. (2011) PLoS One 6, e19147.
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