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Gene Review

FRAS1  -  Fraser extracellular matrix complex subunit 1

Homo sapiens

Synonyms: Extracellular matrix protein FRAS1, FLJ14927, FLJ22031, KIAA1500
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Disease relevance of FRAS1


Psychiatry related information on FRAS1


High impact information on FRAS1


Chemical compound and disease context of FRAS1

  • OBJECTIVE: To evaluate disease-specific cardiovascular reactivity patterns in patients with fibromyalgia (FM) using a recently described method called fractal and recurrence analysis score (FRAS) [8].

Biological context of FRAS1


Anatomical context of FRAS1

  • The similarity of the phenotypic characteristics among the bleb mouse mutants, together with the fact that Fras1/Frem proteins are co-localized in embryonic epithelial basement membranes, suggest that they operate in a common pathway [9].
  • In sum, our data suggest that perturbations in the composition of the extracellular space underlying epithelia could account for the onset of the blebbed phenotype in mouse and Fraser syndrome manifestation in human [12].
  • Thus, our findings identify Fras1 as an important structural component of the sub-lamina densa of basement membranes required for lobar septation and the organization of blood capillaries in the peripheral lung [13].
  • Expression of Fras1 was detected in the mesothelial cells of the visceral pleura and in the conducting airway epithelia [13].
  • We demonstrate that loss of Fras1 causes alterations in the molecular composition of basement membranes, concomitant with local disruptions of epithelial-endothelial contacts and extravasation of erythrocytes into the embryonic respiratory lumen [13].

Associations of FRAS1 with chemical compounds

  • Based on these predictors, the "Fractal & Recurrence Analysis-based Score" (FRAS) was calculated: FRAS=76.2+0.04*HR-supine-DET -12.9*HR-tilt-R/L -0.31*HR-tilt-s.d. -19.27*PTT-tilt-R/L -9.42*PTT-tilt-WAVE [14].
  • However, the induction of the chronic AP-1 complex and the chronic Fras provides a mechanism capable of underlying long-lasting alterations in gene expression following chronic cocaine treatment [15].

Other interactions of FRAS1


Analytical, diagnostic and therapeutic context of FRAS1


  1. Fraser and Ablepharon macrostomia phenotypes: Concurrence in one family and association with mutated FRAS1. Cavalcanti, D.P., Matejas, V., Luquetti, D., Mello, M.F., Zenker, M. Am. J. Med. Genet. A (2007) [Pubmed]
  2. Fraser syndrome and mouse blebbed phenotype caused by mutations in FRAS1/Fras1 encoding a putative extracellular matrix protein. 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., Scambler, P.J. Nat. Genet. (2003) [Pubmed]
  3. Identification of a new gene mutated in Fraser syndrome and mouse myelencephalic blebs. 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., Scambler, P.J. Nat. Genet. (2005) [Pubmed]
  4. Pulmonary hyperplasia in the Fraser cryptophthalmos syndrome. Stevens, C.A., McClanahan, C., Steck, A., Shiel, F.O., Carey, J.C. Am. J. Med. Genet. (1994) [Pubmed]
  5. Fused eyelids, airway anomalies, ovarian cysts, and digital abnormalities in siblings: a new autosomal recessive syndrome or a variant of Fraser syndrome? Mena, W., Krassikoff, N., Philips, J.B. Am. J. Med. Genet. (1991) [Pubmed]
  6. Congenital orbito-palpebral cyst in a case of Fraser syndrome. Amrith, S., Lee, Y., Lee, J., Liew, G., Leo, S., Khoo, B. Orbit (Amsterdam, Netherlands) (2003) [Pubmed]
  7. A direct functional link between the multi-PDZ domain protein GRIP1 and the Fraser syndrome protein Fras1. Takamiya, K., Kostourou, V., Adams, S., Jadeja, S., Chalepakis, G., Scambler, P.J., Huganir, R.L., Adams, R.H. Nat. Genet. (2004) [Pubmed]
  8. Cardiovascular reactivity in fibromyalgia: evidence for pathogenic heterogeneity. Naschitz, J.E., Rozenbaum, M., Fields, M.C., Enis, S., Manor, H., Dreyfuss, D., Peck, S., Peck, E.R., Babich, J.P., Mintz, E.P., Sabo, E., Slobodin, G., Rosner, I. J. Rheumatol. (2005) [Pubmed]
  9. Spatiotemporal distribution of Fras1/Frem proteins during mouse embryonic development. Chiotaki, R., Petrou, P., Giakoumaki, E., Pavlakis, E., Sitaru, C., Chalepakis, G. Gene Expr. Patterns (2007) [Pubmed]
  10. Mutation analysis of the FRAS1 gene demonstrates new mutations in a propositus with Fraser syndrome. Slavotinek, A., Li, C., Sherr, E.H., Chudley, A.E. Am. J. Med. Genet. A (2006) [Pubmed]
  11. Prenatal diagnosis of Fraser syndrome at 18.5 weeks gestation, with autopsy findings at 19 weeks. Schauer, G.M., Dunn, L.K., Godmilow, L., Eagle, R.C., Knisely, A.S. Am. J. Med. Genet. (1990) [Pubmed]
  12. Fras1 deficiency results in cryptophthalmos, renal agenesis and blebbed phenotype in mice. Vrontou, S., Petrou, P., Meyer, B.I., Galanopoulos, V.K., Imai, K., Yanagi, M., Chowdhury, K., Scambler, P.J., Chalepakis, G. Nat. Genet. (2003) [Pubmed]
  13. Basement membrane distortions impair lung lobation and capillary organization in the mouse model for fraser syndrome. Petrou, P., Pavlakis, E., Dalezios, Y., Galanopoulos, V.K., Chalepakis, G. J. Biol. Chem. (2005) [Pubmed]
  14. Assessment of cardiovascular reactivity by fractal and recurrence quantification analysis of heart rate and pulse transit time. Naschitz, J.E., Rosner, I., Shaviv, N., Khorshidi, I., Sundick, S., Isseroff, H., Fields, M., Priselac, R.M., Yeshurun, D., Sabo, E., Itzhak, R. Journal of human hypertension. (2003) [Pubmed]
  15. Novel transcription factors are induced by chronic cocaine treatment. Hope, B.T. Ann. N. Y. Acad. Sci. (1996) [Pubmed]
  16. Two sibs with microcephaly, hygroma colli, renal dysplasia, and cutaneous syndactyly: a new lethal MCA syndrome? Janssen, H.C., Schaap, C., Vandevijver, N., Moerman, P., de Die-Smulders, C.E., Fryns, J.P. J. Med. Genet. (1999) [Pubmed]
  17. Anaesthetic considerations in a patient with Fraser syndrome. Jagtap, S.R., Malde, A.D., Pantvaidya, S.H. Anaesthesia. (1995) [Pubmed]
  18. Resuscitation failure due to Fraser syndrome in a newborn undiagnosed in the prenatal period. Okumus, N., Onal, E.E., Turkyilmaz, C., Biri, A., Gönül, I.I., Unal, S., Poyraz, A., Atalay, Y. Resuscitation. (2005) [Pubmed]
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