Strabismus (protein)

Strabismus (protein)

Strabismus was originally identified as a "Drosophila" protein involved in planar cell polarity [Tanya Wolff and Gerald M. Rubin (1998) "Strabismus, a novel gene that regulates tissue polarity and cell fate decisions in Drosophila" in "Development" Volume 125, pages 1149-1159. Entrez Pubmed|9463361] . Flies with mutated "stranismus" genes have altered development of omatidia in their eyes. Vertebrates have two Strabismus-related proteins, VANGL1 and VANGL2 (an alternate name for the "Drosophila" "Strabismus" protein is "Van Gogh").

The amino acid sequence and localization studies for Strabismus indicate that it is a membrane protein. Prickle is another protein in the planar cell polarity signaling pathway. Prickle is recruited to the cell surface membrane by strabismusR. Bastock, H. Strutt and D. Strutt (2003) "Strabismus is asymmetrically localised and binds to Prickle and Dishevelled during Drosophila planar polarity patterning" in "Development" Volume 130, pages 3007-3014. Entrez Pubmed|12756182] . In cells of the developing "Drosophila" wing, Prickle and Strabismus are concentrated at the cell surface membrane on the most proximal side of cellsM. Fanto and H. McNeill (2004) "Planar polarity from flies to vertebrates" in "Journal of Cell Science" Volume 117, pages 527-533. Entrez Pubmed|14730010] .

Vertebrate cell movement

VANGL2 is involved in the migration of groups of cells during vertebrate embryogenesis [Rachel S. Darken, Adriane M. Scola, Andrew S. Rakeman, Gishnu Das, Marek Mlodzik and Paul A. Wilson (2002) "The planar polarity gene strabismus regulates convergent extension movements in Xenopus" in "EMBO Journal" Volume 21, pages 976-985. Entrez Pubmed|11867525] .

Humans

In humans, mutations in VANGL1 have been associated with neural tube defects including spina bifida,cite journal
author = Kibar Z, Torban E, McDearmid JR, Reynolds A, Berghout J, Mathieu M, Kirillova I, De Marco P, Merello E, Hayes JM, Wallingford JB, Drapeau P, Capra V, Gros P
title = Mutations in VANGL1 associated with neural-tube defects.
journal = N. Engl. J. Med.
volume = 356
issue = 14
pages = 1432–7
year = 2007
pmid = 17409324
doi = 10.1056/NEJMoa060651
url = http://content.nejm.org/cgi/pmidlookup?view=short&pmid=17409324&promo=ONFLNS19
issn = ] and with some forms of cancer including hepatocellular carcinoma.cite journal
author = Yagyu R, Hamamoto R, Furukawa Y, Okabe H, Yamamura T, Nakamura Y
title = Isolation and characterization of a novel human gene, VANGL1, as a therapeutic target for hepatocellular carcinoma.
journal = Int. J. Oncol.
volume = 20
issue = 6
pages = 1173–8
year = 2002
pmid = 12011995
doi =
issn = ] cite journal
author = Katoh M
title = Strabismus (STB)/Vang-like (VANGL) gene family (Review).
journal = Int. J. Mol. Med.
volume = 10
issue = 1
pages = 11–5
year = 2002
pmid = 12060845
doi =
issn = ]

References


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