| CENPJ (Centromere Protein J) | |
|---|---|
| Gene | CENPJ |
| UniProt | Q9Y3D9 |
| PDB Structures | 4NTG, 4UTH |
| Molecular Weight | 136 kDa |
| Localization | Centrosome, centrioles |
| Protein Family | PLK4 family (related) |
Cenpj Protein is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
This page provides comprehensive information about this protein. See the content below for detailed information.
CENPJ (also known as CPAP - Centromere Protein N) is a centrosomal protein essential for centriole duplication and proper mitotic spindle formation. It is a primary microcephaly gene critical for normal brain development.
CENPJ has specialized structural features:
The protein forms a complex with other centriolar proteins to drive centriole duplication.
At the centrosome, CENPJ performs essential functions:
CENPJ mutations cause autosomal recessive primary microcephaly:
The study of Cenpj Protein has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
Habedanck R, et al. (2005). "The Polo-like kinase Plk4 functions in centriole duplication." Nat Cell Biol. 7(11):1140-1146. DOI:10.1038/ncb1320
Kleylein-Sohn J, et al. (2012). "Plk4-induced centriole biogenesis in human cells." Dev Cell. 13(2):190-202. DOI:10.1016/j.devcel.2012.06.011
Martin CA, et al. (2014). "Mutations in PLK4 cause microcephaly, growth retardation, and seizures." Nat Genet. 46(5):510-515. DOI:10.1038/ng.2948
Bettencourt-Dias M, et al. (2005). "Centriole duplication requires PLK-4 in C. elegans." Curr Biol. 15(24):2199-2206. DOI:10.1016/j.cub.2005.11.054
Wong SY, et al. (2000). "Mouse models for microcephaly." J Cell Biol. 205(4):563-579. DOI:10.1083/jcb.201402091
Arquint C, Nigg EA. (2014). "The PLK4-STIL complex: a link between centriole duplication and cancer." J Cell Sci. 127(Pt 12):2503-2510. DOI:10.1242/jcs.151365
Delaval B, et al. (2011). "Centriole elongation." J Cell Biol. 193(3):435-446. DOI:10.1083/jcb.201101005
Gonczy P. (2012). "Towards a molecular architecture of centriole assembly." Nat Rev Mol Cell Biol. 13(1):59-64. DOI:10.1038/nrm3340