1z2a

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Template:STRUCTURE 1z2a

Contents 1 GDP-Bound Rab23 GTPase crystallized in P2(1)2(1)2(1) space group 2 Disease 3 Function 4 About this Structure 5 Reference

GDP-Bound Rab23 GTPase crystallized in P2(1)2(1)2(1) space group

Template:ABSTRACT PUBMED 16034420

Disease

[RAB23_MOUSE] Note=Defects in Rab23 are the cause of the open brain phenotype. Mice suffer from exencephaly and severe malformations of the spinal cord and the dorsal root ganglia, leading to complete embryonic lethality. In addition, mice display poorly developed eyes and polydactyly.^[1]

Function

[RAB23_MOUSE] The small GTPases Rab are key regulators of intracellular membrane trafficking, from the formation of transport vesicles to their fusion with membranes. Rabs cycle between an inactive GDP-bound form and an active GTP-bound form that is able to recruit to membranes different set of downstream effectors directly responsible for vesicle formation, movement, tethering and fusion (By similarity). Plays a role in autophagic vacuole assembly, and mediates defense against pathogens, such as S.aureus, by promoting their capture by autophagosomes that then merge with lysosomes (By similarity). Together with SUFU, prevents nuclear import of GLI1, and thereby inhibits GLI1 transcription factor activity. Regulates GLI1 in differentiating chondrocytes. Likewise, regulates GLI3 proteolytic processing and modulates GLI2 and GLI3 transcription factor activity.^{[2][3][4][5][6]}

About this Structure

1z2a is a 1 chain structure with sequence from Mus musculus. Full crystallographic information is available from OCA.

Reference

• Eathiraj S, Pan X, Ritacco C, Lambright DG. Structural basis of family-wide Rab GTPase recognition by rabenosyn-5. Nature. 2005 Jul 21;436(7049):415-9. PMID:16034420 doi:http://dx.doi.org/10.1038/nature03798

1. ↑ Gunther T, Struwe M, Aguzzi A, Schughart K. Open brain, a new mouse mutant with severe neural tube defects, shows altered gene expression patterns in the developing spinal cord. Development. 1994 Nov;120(11):3119-30. PMID:7720556
2. ↑ Gunther T, Struwe M, Aguzzi A, Schughart K. Open brain, a new mouse mutant with severe neural tube defects, shows altered gene expression patterns in the developing spinal cord. Development. 1994 Nov;120(11):3119-30. PMID:7720556
3. ↑ Eggenschwiler JT, Anderson KV. Dorsal and lateral fates in the mouse neural tube require the cell-autonomous activity of the open brain gene. Dev Biol. 2000 Nov 15;227(2):648-60. PMID:11071781 doi:10.1006/dbio.2000.9918
4. ↑ Eggenschwiler JT, Espinoza E, Anderson KV. Rab23 is an essential negative regulator of the mouse Sonic hedgehog signalling pathway. Nature. 2001 Jul 12;412(6843):194-8. PMID:11449277 doi:10.1038/35084089
5. ↑ Eggenschwiler JT, Bulgakov OV, Qin J, Li T, Anderson KV. Mouse Rab23 regulates hedgehog signaling from smoothened to Gli proteins. Dev Biol. 2006 Feb 1;290(1):1-12. Epub 2005 Dec 20. PMID:16364285 doi:10.1016/j.ydbio.2005.09.022
6. ↑ Yang L, Clinton JM, Blackburn ML, Zhang Q, Zou J, Zielinska-Kwiatkowska A, Tang BL, Chansky HA. Rab23 regulates differentiation of ATDC5 chondroprogenitor cells. J Biol Chem. 2008 Apr 18;283(16):10649-57. doi: 10.1074/jbc.M706795200. Epub 2008, Jan 23. PMID:18218620 doi:10.1074/jbc.M706795200