ELK Biotechnology CEP290 rabbit pAb

제품명

CEP290 rabbit pAb

제품 정보

세포 내 위치 (Cellular Localization)

Cytoplasm, cytoskeleton, microtubule organizing center, centrosome.
Also found in centriolar satellite, nucleus, cell projection (cilium), cilium basal body, cytoplasmic vesicle.
Displaced from centriolar satellites in response to cellular stress (e.g., UV radiation, heat shock).
Localized at the transition zone between the basal body and the ciliary axoneme.
Found in the connecting cilium of photoreceptor cells and base of cilium in kidney collecting duct cells.

배경 (Background)

Centrosomal protein 290 (CEP290) is a human protein containing 13 putative coiled-coil domains, a region homologous to SMC chromosome segregation ATPases, six KID motifs, three tropomyosin homology domains, and an ATP/GTP binding site motif A.
The protein localizes to the centrosome and cilia and contains multiple post-translational modification sites (N-glycosylation, tyrosine sulfation, phosphorylation, N-myristoylation, and amidation).
Mutations in CEP290 are associated with Joubert syndrome and nephronophthisis.
Antibodies against this protein have been linked to several forms of cancer.
(RefSeq, Jul 2008)