Frem3, a member of the 12 CSPG repeats-containing extracellular matrix protein family, is a basement membrane protein with tissue distribution patterns distinct from those of Fras1, Frem2, and QBRICK/Frem1

Matrix Biology

Volumn 26, Issue 6, 2007, Pages 456-462

  Kiyozumi, Daiji ^a,b   Sugimoto, Nagisa ^b   Nakano, Itsuko ^a,b   Sekiguchi, Kiyotoshi ^a,b  

^a OSAKA UNIVERSITY   (Japan)

^b JAPAN SCIENCE AND TECHNOLOGY AGENCY   (Japan)

Author keywords

Fraser syndrome

Indexed keywords

PROTEIN DERIVATIVE; PROTEIN FRAS1; PROTEIN FREM1; PROTEIN FREM2; PROTEIN FREM3; UNCLASSIFIED DRUG;

ANIMAL CELL; ARTICLE; BASEMENT MEMBRANE; CONTROLLED STUDY; EMBRYO; EPIDERMIS CELL; EXTRACELLULAR MATRIX; HAIR FOLLICLE; KIDNEY EPITHELIUM; MOUSE; NEWBORN; NONHUMAN; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN DEGRADATION; PROTEIN DOMAIN; PROTEIN EXPRESSION; PROTEIN FAMILY; PROTEIN LOCALIZATION; RETINA GANGLION CELL; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; SALIVARY GLAND; TISSUE DISTRIBUTION;

ANIMALS; ANIMALS, NEWBORN; BASEMENT MEMBRANE; EMBRYONIC STRUCTURES; EXTRACELLULAR MATRIX PROTEINS; FEMALE; GENE EXPRESSION PROFILING; HAIR FOLLICLE; IMMUNOHISTOCHEMISTRY; MICE; MICE, INBRED STRAINS; MICE, KNOCKOUT; MICE, MUTANT STRAINS; RETINA; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; SALIVARY GLANDS; TIME FACTORS;

MUS;

EID: 34447545502     PISSN: 0945053X     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.matbio.2007.03.001     Document Type: Article

References (13)

1. Bladt F., Tafuri A., Gelkop S., Langille L., and Pawson T. Epidermolysis bullosa and embryonic lethality in mice lacking the multi-PDZ domain protein GRIP1. Proc. Natl. Acad. Sci. U. S. A. 99 (2002) 6816-6821
2. Jadeja S., Smyth I., Pitera J.E., Taylor M.S., van Haelst M., Bentley E., McGregor L., Hopkins J., Chalepakis G., Philip N., Perez Aytes A., Watt F.M., Darling S.M., Jackson I., Woolf A.S., and Scambler P.J. Identification of a new gene mutated in Fraser syndrome and mouse myelencephalic blebs. Nat. Genet. 37 (2005) 520-525
3. Kiyozumi D., Osada A., Sugimoto N., Weber C.N., Ono Y., Imai T., Okada A., and Sekiguchi K. Identification of a novel cell-adhesive protein spatiotemporally expressed in the basement membrane of mouse developing hair follicle. Exp. Cell Res. 306 (2005) 9-23
4. Kiyozumi D., Sugimoto N., and Sekiguchi K. Breakdown of the reciprocal stabilization of QBRICK/Frem1, Fras1, and Frem2 at the basement membrane provokes Fraser syndrome-like defects. Proc. Natl. Acad. Sci. U. S. A. 99 (2006) 6816-6821
5. McGregor L., Makela V., Darling S.M., Vrontou S., Chalepakis G., Roberts C., Smart N., Rutland P., Prescott N., Hopkins J., Bentley E., Shaw A., Roberts E., Mueller R., Jadeja S., Philip N., Nelson J., Francannet C., Perez-Aytes A., Megarbane A., Kerr B., Wainwright B., Woolf A.S., Winter R.M., and Scambler P.J. Fraser syndrome and mouse blebbed phenotype caused by mutations in FRAS1/Fras1 encoding a putative extracellular matrix protein. Nat. Genet. 34 (2003) 203-208
6. Staub E., Hinzmann B., and Rosenthal A. A novel repeat in the melanoma-associated chondroitin sulfate proteoglycan defines a new protein family. FEBS Lett. 527 (2002) 114-118
7. Slavotinek A.M., and Tifft C.J. Fraser syndrome and cryptophthalmos: review of the diagnostic criteria and evidence for phenotypic modules in complex malformation syndromes. J. Med. Genet. 39 (2002) 623-633
8. Smyth I., and Scambler P. The genetics of Fraser syndrome and the blebs mouse mutants. Hum. Mol. Genet. 14 (2005) R269-R274
9. Smyth I., Du X., Taylor M.S., Justice M.J., Beutler B., and Jackson I. The extracellular matrix gene Frem1 is essential for the normal adhesion of the embryonic epidermis. Proc. Natl. Acad. Sci. U. S. A. 101 (2004) 13560-13565
10. Takamiya K., Kostourou V., Adams S., Jadeja S., Chalepakis G., Scambler P.J., Huganir R.L., and Adams R.H. A direct functional link between the multi-PDZ domain protein GRIP1 and the Fraser syndrome protein Fras1. Nat. Genet. 36 (2004) 172-177
11. Tillet E., Ruggiero F., Nishiyama A., and Stallcup W.B. The membrane-spanning proteoglycan NG2 binds to collagens V and VI through the central nonglobular domain of its core protein. J. Biol. Chem. 272 (1997) 10769-10776
12. Timmer J.R., Mak T.W., Manova K., Anderson K.V., and Niswander L. Tissue morphogenesis and vascular stability require the Frem2 protein, product of the mouse myelencephalic blebs gene. Proc. Natl. Acad. Sci. U. S. A. 102 (2005) 11746-11750
13. Vrontou S., Petrou P., Meyer B.I., Galanopoulos V.K., Imai K., Yanagi M., Chowdhury K., Scambler P.J., and Chalepakis G. Fras1 deficiency results in cryptophthalmos, renal agenesis and blebbed phenotype in mice. Nat. Genet. 34 (2003) 209-214