메뉴 건너뛰기




Volumn 17, Issue 20, 2008, Pages 3180-3190

Failure of bone morphogenetic protein receptor trafficking in pulmonary arterial hypertension: Potential for rescue

Author keywords

[No Author keywords available]

Indexed keywords

4 PHENYLBUTYRIC ACID; BONE MORPHOGENETIC PROTEIN; BONE MORPHOGENETIC PROTEIN RECEPTOR 1; BONE MORPHOGENETIC PROTEIN RECEPTOR 2; CHAPERONE; CYSTEINE; GLYCEROL; MUTANT PROTEIN; MYC PROTEIN; SMAD1 PROTEIN; SMAD5 PROTEIN; THAPSIGARGIN;

EID: 53349173222     PISSN: 09646906     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/ddn214     Document Type: Article
Times cited : (81)

References (36)
  • 1
    • 0032578279 scopus 로고    scopus 로고
    • Primary pulmonary hypertension
    • Gaine, S.P. and Rubin, L.J. (1998) Primary pulmonary hypertension. Lancet, 352, 719-725.
    • (1998) Lancet , vol.352 , pp. 719-725
    • Gaine, S.P.1    Rubin, L.J.2
  • 2
    • 0033769779 scopus 로고    scopus 로고
    • Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension. A morphometric and immunohistochemical study
    • Yi, E.S., Kim, H., Ahn, H., Strother, J., Morris, T., Masliah, E., Hansen, L.A., Park, K. and Friedman, P.J. (2000) Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension. A morphometric and immunohistochemical study. Am. J. Respir. Crit. Care Med., 162, 1577-1586.
    • (2000) Am. J. Respir. Crit. Care Med , vol.162 , pp. 1577-1586
    • Yi, E.S.1    Kim, H.2    Ahn, H.3    Strother, J.4    Morris, T.5    Masliah, E.6    Hansen, L.A.7    Park, K.8    Friedman, P.J.9
  • 3
    • 0037046175 scopus 로고    scopus 로고
    • Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor
    • Atkinson, C., Stewart, S., Upton, P.D., Machado, R., Thomson, J.R., Trembath, R.C. and Morrell, N.W. (2002) Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor. Circulation, 105, 1672-1678.
    • (2002) Circulation , vol.105 , pp. 1672-1678
    • Atkinson, C.1    Stewart, S.2    Upton, P.D.3    Machado, R.4    Thomson, J.R.5    Trembath, R.C.6    Morrell, N.W.7
  • 4
    • 0033817459 scopus 로고    scopus 로고
    • Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium
    • Lane, K.B., Machado, R.D., Pauciulo, M.W., Thomson, J.R., Phillips, J.A., III, Loyd, J.E., Nichols, W.C. and Trembath, R.C. (2000) Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat. Genet., 26, 81-84.
    • (2000) Nat. Genet , vol.26 , pp. 81-84
    • Lane, K.B.1    Machado, R.D.2    Pauciulo, M.W.3    Thomson, J.R.4    Phillips III, J.A.5    Loyd, J.E.6    Nichols, W.C.7    Trembath, R.C.8
  • 8
    • 0036735752 scopus 로고    scopus 로고
    • Functional heterogeneity of bone morphogenetic protein receptor-II mutants found in patients with primary pulmonary hypertension
    • Nishihara, A., Watabe, T., Imamura, T. and Miyazono, K. (2002) Functional heterogeneity of bone morphogenetic protein receptor-II mutants found in patients with primary pulmonary hypertension. Mol. Biol. Cell, 13, 3055-3063.
    • (2002) Mol. Biol. Cell , vol.13 , pp. 3055-3063
    • Nishihara, A.1    Watabe, T.2    Imamura, T.3    Miyazono, K.4
  • 9
    • 44349101103 scopus 로고    scopus 로고
    • Stoichiometric imbalance in the receptor complex contributes to dysfunctional BMPR-II mediated signalling in pulmonary hypertension
    • Nasim, T.M., Ghouri, A., Patel, B., James, V., Rudarakanchana, N., Morrell, N.W. and Trembath, R.C. (2008) Stoichiometric imbalance in the receptor complex contributes to dysfunctional BMPR-II mediated signalling in pulmonary hypertension. Hum. Mol. Genet., 17, 1683-1694.
    • (2008) Hum. Mol. Genet , vol.17 , pp. 1683-1694
    • Nasim, T.M.1    Ghouri, A.2    Patel, B.3    James, V.4    Rudarakanchana, N.5    Morrell, N.W.6    Trembath, R.C.7
  • 11
    • 38549145590 scopus 로고    scopus 로고
    • Functional characterization of bone morphogenetic protein binding sites and Smad1/5 activation in human vascular cells
    • Upton, P.D., Long, L., Trembath, R.C. and Morrell, N.W. (2008) Functional characterization of bone morphogenetic protein binding sites and Smad1/5 activation in human vascular cells. Mol. Pharmacol., 73, 539-552.
    • (2008) Mol. Pharmacol , vol.73 , pp. 539-552
    • Upton, P.D.1    Long, L.2    Trembath, R.C.3    Morrell, N.W.4
  • 12
    • 27644494876 scopus 로고    scopus 로고
    • Smad transcription factors
    • Massague, J., Seoane, J. and Wotton, D. (2005) Smad transcription factors. Genes Dev., 19, 2783-2810.
    • (2005) Genes Dev , vol.19 , pp. 2783-2810
    • Massague, J.1    Seoane, J.2    Wotton, D.3
  • 14
    • 3442880434 scopus 로고    scopus 로고
    • Functional rescue of the constitutively internalized V2 vasopressin receptor mutant R137H by the pharmacological chaperone action of SR49059
    • Bernier, V., Lagace, M., Lonergan, M., Arthus, M.F., Bichet, D.G. and Bouvier, M. (2004) Functional rescue of the constitutively internalized V2 vasopressin receptor mutant R137H by the pharmacological chaperone action of SR49059. Mol. Endocrinol., 18, 2074-2084.
    • (2004) Mol. Endocrinol , vol.18 , pp. 2074-2084
    • Bernier, V.1    Lagace, M.2    Lonergan, M.3    Arthus, M.F.4    Bichet, D.G.5    Bouvier, M.6
  • 15
    • 33846237329 scopus 로고    scopus 로고
    • Functional rescue of vasopressin V2 receptor mutants in MDCK cells by pharmacochaperones: Relevance to therapy of nephrogenic diabetes insipidus
    • Robben, J.H., Sze, M., Knoers, N.V. and Deen, P.M. (2007) Functional rescue of vasopressin V2 receptor mutants in MDCK cells by pharmacochaperones: Relevance to therapy of nephrogenic diabetes insipidus. Am. J. Physiol. Renal Physiol., 292, F253-F260.
    • (2007) Am. J. Physiol. Renal Physiol , vol.292
    • Robben, J.H.1    Sze, M.2    Knoers, N.V.3    Deen, P.M.4
  • 16
    • 30044441872 scopus 로고    scopus 로고
    • Rescue of vasopressin V2 receptor mutants by chemical chaperones: Specificity and mechanism
    • Robben, J.H., Sze, M., Knoers, N.V. and Deen, P.M. (2006) Rescue of vasopressin V2 receptor mutants by chemical chaperones: Specificity and mechanism. Mol. Biol. Cell, 17, 379-386.
    • (2006) Mol. Biol. Cell , vol.17 , pp. 379-386
    • Robben, J.H.1    Sze, M.2    Knoers, N.V.3    Deen, P.M.4
  • 17
    • 33645235405 scopus 로고    scopus 로고
    • Maintaining low Ca2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells
    • Norez, C., Antigny, F., Becq, F. and Vandebrouck, C. (2006) Maintaining low Ca2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells. Traffic, 7 562-573.
    • (2006) Traffic , vol.7 , pp. 562-573
    • Norez, C.1    Antigny, F.2    Becq, F.3    Vandebrouck, C.4
  • 18
    • 0030809817 scopus 로고    scopus 로고
    • In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR
    • Rubenstein, R.C., Egan, M.E. and Zeitlin, P.L. (1997) In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. J. Clin. Invest., 100, 2457-2465.
    • (1997) J. Clin. Invest , vol.100 , pp. 2457-2465
    • Rubenstein, R.C.1    Egan, M.E.2    Zeitlin, P.L.3
  • 20
    • 0031889082 scopus 로고    scopus 로고
    • A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: Partial restoration of nasal epithelial CFTR function
    • Rubenstein, R.C. and Zeitlin, P.L. (1998) A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: Partial restoration of nasal epithelial CFTR function. Am. J. Respir. Crit. Care Med., 157, 484-490.
    • (1998) Am. J. Respir. Crit. Care Med , vol.157 , pp. 484-490
    • Rubenstein, R.C.1    Zeitlin, P.L.2
  • 21
    • 0036665609 scopus 로고    scopus 로고
    • Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate
    • Zeitlin, P.L., Diener-West, M., Rubenstein, R.C., Boyle, M.P., Lee, C.K. and Brass-Ernst, L. (2002) Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate. Mol. Ther., 6, 119-126.
    • (2002) Mol. Ther , vol.6 , pp. 119-126
    • Zeitlin, P.L.1    Diener-West, M.2    Rubenstein, R.C.3    Boyle, M.P.4    Lee, C.K.5    Brass-Ernst, L.6
  • 22
    • 33748337526 scopus 로고    scopus 로고
    • Diagnosis and management of pulmonary arterial hypertension: Implications for respiratory care
    • Levine, D.J. (2006) Diagnosis and management of pulmonary arterial hypertension: Implications for respiratory care. Respir. Care, 51, 368-381.
    • (2006) Respir. Care , vol.51 , pp. 368-381
    • Levine, D.J.1
  • 23
    • 0034021776 scopus 로고    scopus 로고
    • Bone morphogenetic protein receptor complexes on the surface of live cells: A new oligomerization mode for serine/threonine kinase receptors
    • Gilboa, L., Nohe, A., Geissendorfer, T., Sebald, W., Henis, Y.I. and Knaus, P. (2000) Bone morphogenetic protein receptor complexes on the surface of live cells: A new oligomerization mode for serine/threonine kinase receptors. Mol. Biol. Cell, 11, 1023-1035.
    • (2000) Mol. Biol. Cell , vol.11 , pp. 1023-1035
    • Gilboa, L.1    Nohe, A.2    Geissendorfer, T.3    Sebald, W.4    Henis, Y.I.5    Knaus, P.6
  • 25
    • 0036843129 scopus 로고    scopus 로고
    • Traffic jams II: An update of diseases of intracellular transport
    • Aridor, M. and Hannan, L.A. (2002) Traffic jams II: An update of diseases of intracellular transport. Traffic, 3, 781-790.
    • (2002) Traffic , vol.3 , pp. 781-790
    • Aridor, M.1    Hannan, L.A.2
  • 27
    • 33845958175 scopus 로고    scopus 로고
    • The unfolded protein response
    • Schroder, M. (2006) The unfolded protein response. Mol. Biotechnol. 34, 279-290.
    • (2006) Mol. Biotechnol , vol.34 , pp. 279-290
    • Schroder, M.1
  • 29
    • 7644238103 scopus 로고    scopus 로고
    • Thapsigargin or curcumin does not promote maturation of processing mutants of the ABC transporters, CFTR, and P-glycoprotein
    • Loo, T.W., Bartlett, M.C. and Clarke, D.M. (2004) Thapsigargin or curcumin does not promote maturation of processing mutants of the ABC transporters, CFTR, and P-glycoprotein. Biochem. Biophys. Res. Commun., 325, 580-585.
    • (2004) Biochem. Biophys. Res. Commun , vol.325 , pp. 580-585
    • Loo, T.W.1    Bartlett, M.C.2    Clarke, D.M.3
  • 31
    • 7644238103 scopus 로고    scopus 로고
    • Thapsigargin or curcumin does not promote maturation of processing mutants of the ABC transporters, CFTR, and P-glycoprotein
    • Loo, T.W., Bartlett, M.C. and Clarke, D.M. (2004) Thapsigargin or curcumin does not promote maturation of processing mutants of the ABC transporters, CFTR, and P-glycoprotein. Biochem. Biophys. Res. Commun., 325, 580-585.
    • (2004) Biochem. Biophys. Res. Commun , vol.325 , pp. 580-585
    • Loo, T.W.1    Bartlett, M.C.2    Clarke, D.M.3
  • 32
    • 0034099743 scopus 로고    scopus 로고
    • Sodium 4-phenylbutyrate downregulates Hsc70: Implications for intracellular trafficking of DeltaF508-CFTR
    • Rubenstein, R.C. and Zeitlin, P.L. (2000) Sodium 4-phenylbutyrate downregulates Hsc70: Implications for intracellular trafficking of DeltaF508-CFTR. Am. J. Physiol. Cell Physiol., 278, C259-C267.
    • (2000) Am. J. Physiol. Cell Physiol , vol.278
    • Rubenstein, R.C.1    Zeitlin, P.L.2
  • 33
    • 0034805392 scopus 로고    scopus 로고
    • Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation
    • Rubenstein, R.C. and Lyons, B.M. (2001) Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation. Am. J. Physiol. Lung Cell. Mol. Physiol., 281, L43-L51.
    • (2001) Am. J. Physiol. Lung Cell. Mol. Physiol , vol.281
    • Rubenstein, R.C.1    Lyons, B.M.2
  • 35
    • 0347065355 scopus 로고    scopus 로고
    • Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator
    • Zhang, X.M., Wang, X.T., Yue, H., Leung, S.W., Thibodeau, P.H., Thomas, P.J. and Guggino, S.E. (2003) Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator. J. Biol. Chem., 278, 51232-51242.
    • (2003) J. Biol. Chem , vol.278 , pp. 51232-51242
    • Zhang, X.M.1    Wang, X.T.2    Yue, H.3    Leung, S.W.4    Thibodeau, P.H.5    Thomas, P.J.6    Guggino, S.E.7
  • 36
    • 33646008876 scopus 로고    scopus 로고
    • Chemical chaperones: Mechanisms of action and potential use
    • Papp, E. and Csermely, P. (2006) Chemical chaperones: Mechanisms of action and potential use. Handb. Exp. Pharmacol., 405-416.
    • (2006) Handb. Exp. Pharmacol , pp. 405-416
    • Papp, E.1    Csermely, P.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.