Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF-β expression and connective tissue features
Fibromuscular dysplasia (FMD) is a rare, nonatherosclerotic arterial disease for which the molecular basis is unknown. We comprehensively studied 47 subjects with FMD, including physical examination, spine magnetic resonance imaging, bone densitometry, and brain magnetic resonance angiography. Infla...
Saved in:
| Published in | The FASEB journal Vol. 28; no. 8; p. 3313 |
|---|---|
| Main Authors | , , , , , , , , , , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
United States
01.08.2014
|
| Subjects | |
| Online Access | Get more information |
| ISSN | 1530-6860 |
| DOI | 10.1096/fj.14-251207 |
Cover
Loading…
| Abstract | Fibromuscular dysplasia (FMD) is a rare, nonatherosclerotic arterial disease for which the molecular basis is unknown. We comprehensively studied 47 subjects with FMD, including physical examination, spine magnetic resonance imaging, bone densitometry, and brain magnetic resonance angiography. Inflammatory biomarkers in plasma and transforming growth factor β (TGF-β) cytokines in patient-derived dermal fibroblasts were measured by ELISA. Arterial pathology other than medial fibrodysplasia with multifocal stenosis included cerebral aneurysm, found in 12.8% of subjects. Extra-arterial pathology included low bone density (P<0.001); early onset degenerative spine disease (95.7%); increased incidence of Chiari I malformation (6.4%) and dural ectasia (42.6%); and physical examination findings of a mild connective tissue dysplasia (95.7%). Screening for mutations causing known genetically mediated arteriopathies was unrevealing. We found elevated plasma TGF-β1 (P=0.009), TGF-β2 (P=0.004) and additional inflammatory markers, and increased TGF-β1 (P=0.0009) and TGF-β2 (P=0.0001) secretion in dermal fibroblast cell lines from subjects with FMD compared to age- and gender-matched controls. Detailed phenotyping of patients with FMD allowed us to demonstrate that FMD is a systemic disease with alterations in common with the spectrum of genetic syndromes that involve altered TGF-β signaling and offers TGF-β as a marker of FMD. |
|---|---|
| AbstractList | Fibromuscular dysplasia (FMD) is a rare, nonatherosclerotic arterial disease for which the molecular basis is unknown. We comprehensively studied 47 subjects with FMD, including physical examination, spine magnetic resonance imaging, bone densitometry, and brain magnetic resonance angiography. Inflammatory biomarkers in plasma and transforming growth factor β (TGF-β) cytokines in patient-derived dermal fibroblasts were measured by ELISA. Arterial pathology other than medial fibrodysplasia with multifocal stenosis included cerebral aneurysm, found in 12.8% of subjects. Extra-arterial pathology included low bone density (P<0.001); early onset degenerative spine disease (95.7%); increased incidence of Chiari I malformation (6.4%) and dural ectasia (42.6%); and physical examination findings of a mild connective tissue dysplasia (95.7%). Screening for mutations causing known genetically mediated arteriopathies was unrevealing. We found elevated plasma TGF-β1 (P=0.009), TGF-β2 (P=0.004) and additional inflammatory markers, and increased TGF-β1 (P=0.0009) and TGF-β2 (P=0.0001) secretion in dermal fibroblast cell lines from subjects with FMD compared to age- and gender-matched controls. Detailed phenotyping of patients with FMD allowed us to demonstrate that FMD is a systemic disease with alterations in common with the spectrum of genetic syndromes that involve altered TGF-β signaling and offers TGF-β as a marker of FMD. |
| Author | Dietz, Harry C Raza, Rafi Francomano, Clair A Ganesh, Santhi K Milewicz, Dianna M Yang, Jiandong Kuo, Shinie Hunker, Kristina Morissette, Rachel Xu, Zhi Sloper, Leslie Van Eyk, Jennifer Tong, Lan Schoenhoff, Florian McDonnell, Nazli B Yang, Min-Lee Griswold, Benjamin F |
| Author_xml | – sequence: 1 givenname: Santhi K surname: Ganesh fullname: Ganesh, Santhi K email: nazli.mcdonnell@gmail.com, sganesh@umich.edu organization: Division of Cardiovascular Medicine, Department of Internal Medicine, and Department of Human Genetics, University of Michigan, Ann Arbor, Michigan, USA; nazli.mcdonnell@gmail.com sganesh@umich.edu – sequence: 2 givenname: Rachel surname: Morissette fullname: Morissette, Rachel email: nazli.mcdonnell@gmail.com, sganesh@umich.edu organization: Laboratory of Clinical Investigation, National Institute on Aging, Baltimore, Maryland, USA; nazli.mcdonnell@gmail.com sganesh@umich.edu – sequence: 3 givenname: Zhi surname: Xu fullname: Xu, Zhi organization: Laboratory of Clinical Investigation, National Institute on Aging, Baltimore, Maryland, USA – sequence: 4 givenname: Florian surname: Schoenhoff fullname: Schoenhoff, Florian organization: Johns Hopkins Bayview Proteomics Center, Department of Medicine, Johns Hopkins University, Baltimore, Maryland, USA – sequence: 5 givenname: Benjamin F surname: Griswold fullname: Griswold, Benjamin F organization: Laboratory of Clinical Investigation, National Institute on Aging, Baltimore, Maryland, USA – sequence: 6 givenname: Jiandong surname: Yang fullname: Yang, Jiandong organization: Laboratory of Clinical Investigation, National Institute on Aging, Baltimore, Maryland, USA – sequence: 7 givenname: Lan surname: Tong fullname: Tong, Lan organization: Division of Cardiovascular Medicine, Department of Internal Medicine, and Department of Human Genetics, University of Michigan, Ann Arbor, Michigan, USA – sequence: 8 givenname: Min-Lee surname: Yang fullname: Yang, Min-Lee organization: Division of Cardiovascular Medicine, Department of Internal Medicine, and Department of Human Genetics, University of Michigan, Ann Arbor, Michigan, USA – sequence: 9 givenname: Kristina surname: Hunker fullname: Hunker, Kristina organization: Division of Cardiovascular Medicine, Department of Internal Medicine, and Department of Human Genetics, University of Michigan, Ann Arbor, Michigan, USA – sequence: 10 givenname: Leslie surname: Sloper fullname: Sloper, Leslie organization: Laboratory of Clinical Investigation, National Institute on Aging, Baltimore, Maryland, USA – sequence: 11 givenname: Shinie surname: Kuo fullname: Kuo, Shinie organization: Division of Cardiovascular Medicine, Department of Internal Medicine, and Department of Human Genetics, University of Michigan, Ann Arbor, Michigan, USA – sequence: 12 givenname: Rafi surname: Raza fullname: Raza, Rafi organization: Laboratory of Clinical Investigation, National Institute on Aging, Baltimore, Maryland, USA – sequence: 13 givenname: Dianna M surname: Milewicz fullname: Milewicz, Dianna M organization: Division of Medical Genetics, Department of Internal Medicine, University of Texas Health Science Center, Houston, Texas, USA – sequence: 14 givenname: Clair A surname: Francomano fullname: Francomano, Clair A organization: Greater Baltimore Medical Center, Towson, Maryland, USA – sequence: 15 givenname: Harry C surname: Dietz fullname: Dietz, Harry C organization: McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA; and Howard Hughes Medical Institute, Baltimore, Maryland, USA – sequence: 16 givenname: Jennifer surname: Van Eyk fullname: Van Eyk, Jennifer organization: Johns Hopkins Bayview Proteomics Center, Department of Medicine, Johns Hopkins University, Baltimore, Maryland, USA – sequence: 17 givenname: Nazli B surname: McDonnell fullname: McDonnell, Nazli B email: nazli.mcdonnell@gmail.com, sganesh@umich.edu organization: Laboratory of Clinical Investigation, National Institute on Aging, Baltimore, Maryland, USA; nazli.mcdonnell@gmail.com sganesh@umich.edu |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/24732132$$D View this record in MEDLINE/PubMed |
| BookMark | eNo1kE1OwzAQhS0Eoj-wY43mAikeJ3HSJapoQarEpqwr2xm3rtIkyiRAD8JFOAhnohRYPT3p_UjfSJxXdUVC3KCcoJzqO7-bYBKpFJXMzsQQ01hGOtdyIEbMOyklStSXYqCSLFYYq6H4mJWhCs6UYKoCbKjdlvYn37S1DyUxcL_ZEHfgg23rfc-uL00LxYGb0nAwEBgM8IG7nyIUgckwwVvotmDKjloqYLWYR1-fQO9NS8yhrk5vrq4qcl14JegCc0_gyXT9MXIlLrwpma7_dCxe5g-r2WO0fF48ze6XkYtzmUY4lalFk9vM-yRDqwkzzArK7dSj9JQnOvaYxEhaF9apNHdpksTeWnRHBFKNxe3vbtPbPRXrpg170x7W_3zUNxfFaw8 |
| CitedBy_id | crossref_primary_10_1016_j_jaccas_2019_11_058 crossref_primary_10_1016_j_radcr_2023_04_030 crossref_primary_10_1093_cvr_cvaa353 crossref_primary_10_1002_ajmg_a_61054 crossref_primary_10_1161_HYPERTENSIONAHA_120_15349 crossref_primary_10_1177_1358863X15592192 crossref_primary_10_1093_cvr_cvab086 crossref_primary_10_3390_ijms19051526 crossref_primary_10_1161_CIRCGEN_121_003496 crossref_primary_10_1177_1358863X18821816 crossref_primary_10_17116_jnevro202212210182 crossref_primary_10_1053_j_semvascsurg_2021_02_009 crossref_primary_10_1016_S0001_4079_19_30386_3 crossref_primary_10_1093_ajh_hpad056 crossref_primary_10_3109_01913123_2016_1156797 crossref_primary_10_17116_jnevro2020120111116 crossref_primary_10_1161_JAHA_114_001259 crossref_primary_10_1016_j_amjcard_2022_09_032 crossref_primary_10_1053_j_tvir_2014_11_007 crossref_primary_10_1111_eci_13023 crossref_primary_10_5551_jat_ED040 crossref_primary_10_1038_s41440_018_0063_z crossref_primary_10_1038_s44161_024_00533_w crossref_primary_10_1042_CS20210990 crossref_primary_10_1038_nrm3896 crossref_primary_10_1016_j_cca_2023_117613 crossref_primary_10_24075_brsmu_2019_064 crossref_primary_10_1016_j_ccl_2016_12_006 crossref_primary_10_2147_VHRM_S362844 crossref_primary_10_1016_j_athoracsur_2014_06_107 crossref_primary_10_1016_j_tcm_2021_01_002 crossref_primary_10_1161_CIRCRESAHA_121_318300 crossref_primary_10_1056_NEJMcpc1707558 crossref_primary_10_1016_j_tcm_2021_01_006 crossref_primary_10_1007_s10157_018_1575_1 crossref_primary_10_1212_CPJ_0000000000000339 crossref_primary_10_4103_heartviews_heartviews_138_23 crossref_primary_10_1016_j_amjcard_2018_12_045 crossref_primary_10_3390_medicines7020009 crossref_primary_10_1097_HJH_0000000000002019 crossref_primary_10_1177_1358863X241254796 crossref_primary_10_1001_jamaneurol_2018_2848 crossref_primary_10_15420_usc_2023_03 crossref_primary_10_1016_j_jvscit_2024_101444 crossref_primary_10_1161_CIR_0000000000000564 crossref_primary_10_3389_fcvm_2024_1409278 crossref_primary_10_1016_j_avsg_2018_11_030 crossref_primary_10_1016_j_ijcard_2018_11_023 crossref_primary_10_1101_cshperspect_a021907 crossref_primary_10_7759_cureus_8755 crossref_primary_10_1161_ATVBAHA_119_313885 crossref_primary_10_1007_s11936_016_0460_z crossref_primary_10_11603_1811_2471_2020_v_i2_11293 crossref_primary_10_1007_s11936_017_0579_6 crossref_primary_10_1007_s11936_019_0780_x crossref_primary_10_1016_j_avsg_2022_12_092 crossref_primary_10_1007_s00261_016_0778_8 crossref_primary_10_1038_s10038_019_0626_0 crossref_primary_10_1161_CIRCGENETICS_117_001933 crossref_primary_10_1097_HJH_0000000000000625 crossref_primary_10_1016_j_jvsc_2014_12_004 crossref_primary_10_1177_1358863X16637913 crossref_primary_10_7861_clinmed_2023_0403 crossref_primary_10_1161_JAHA_120_017240 crossref_primary_10_5551_jat_32318 crossref_primary_10_1016_j_pcad_2018_03_001 crossref_primary_10_15746_sms_23_013 |
| ContentType | Journal Article |
| Copyright | FASEB. |
| Copyright_xml | – notice: FASEB. |
| DBID | CGR CUY CVF ECM EIF NPM |
| DOI | 10.1096/fj.14-251207 |
| DatabaseName | Medline MEDLINE MEDLINE (Ovid) MEDLINE MEDLINE PubMed |
| DatabaseTitle | MEDLINE Medline Complete MEDLINE with Full Text PubMed MEDLINE (Ovid) |
| DatabaseTitleList | MEDLINE |
| Database_xml | – sequence: 1 dbid: NPM name: PubMed url: https://proxy.k.utb.cz/login?url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 2 dbid: EIF name: MEDLINE url: https://proxy.k.utb.cz/login?url=https://www.webofscience.com/wos/medline/basic-search sourceTypes: Index Database |
| DeliveryMethod | no_fulltext_linktorsrc |
| Discipline | Biology |
| EISSN | 1530-6860 |
| ExternalDocumentID | 24732132 |
| Genre | Research Support, American Recovery and Reinvestment Act Research Support, N.I.H., Intramural Research Support, Non-U.S. Gov't Journal Article Research Support, N.I.H., Extramural |
| GrantInformation_xml | – fundername: Intramural NIH HHS – fundername: Howard Hughes Medical Institute – fundername: NHLBI NIH HHS grantid: P30HL101290 – fundername: NHLBI NIH HHS grantid: RC1 HL100021 – fundername: NHLBI NIH HHS grantid: R00 HL089413 – fundername: NCRR NIH HHS grantid: 1U54RR023561-01A1 – fundername: NHLBI NIH HHS grantid: P30 HL101290 – fundername: NHLBI NIH HHS grantid: 5RC1HL100021-02 – fundername: NHLBI NIH HHS grantid: R00HL089413 – fundername: NCATS NIH HHS grantid: UL1 TR000371 |
| GroupedDBID | --- -DZ -~X .55 0R~ 0VX 123 18M 1OB 1OC 29H 2WC 33P 34G 39C 3O- 4.4 53G 5GY 5RE 85S AAHHS AAHQN AAMNL AANLZ AAYCA ABCUV ABDNZ ABEFU ABJNI ABOCM ACCFJ ACCZN ACGFS ACIWK ACNCT ACPOU ACPRK ACXQS ACYGS ADKYN ADZMN AEEZP AEIGN AENEX AEQDE AEUYR AFFNX AFFPM AFRAH AFWVQ AGCDD AHBTC AI. AITYG AIURR AIWBW AIZAD AJBDE ALMA_UNASSIGNED_HOLDINGS ALUQN ALVPJ AMYDB BFHJK BIYOS C1A CGR CS3 CUY CVF DCZOG DU5 D~5 E3Z EBS ECM EIF EJD F5P F9R FRP H13 HGLYW HZ~ H~9 J5H L7B LATKE LEEKS MEWTI MVM NEJ NPM O9- OHT OVD Q-A RHF RHI RJQFR ROL SAMSI SJN SUPJJ TEORI TFA TR2 TWZ U18 VH1 VXZ W8F WH7 WHG WOQ WXSBR X7M XJT XOL XSW Y6R YBU YCJ YHG YKV YNH YSK Z0Y Z5M ZCA ZE2 ZGI ZXP ~KM |
| ID | FETCH-LOGICAL-c3805-1905b1a8b7ff471b6e1717de8b9f10fe8463f1431e66dbc258c5443fbb1c24702 |
| IngestDate | Wed Feb 19 01:55:31 EST 2025 |
| IsDoiOpenAccess | false |
| IsOpenAccess | true |
| IsPeerReviewed | true |
| IsScholarly | true |
| Issue | 8 |
| Keywords | human genetics TGF-β pathway aneurysm FMD biomarker |
| Language | English |
| License | FASEB. |
| LinkModel | OpenURL |
| MergedId | FETCHMERGED-LOGICAL-c3805-1905b1a8b7ff471b6e1717de8b9f10fe8463f1431e66dbc258c5443fbb1c24702 |
| OpenAccessLink | https://hdl.handle.net/2027.42/154380 |
| PMID | 24732132 |
| ParticipantIDs | pubmed_primary_24732132 |
| PublicationCentury | 2000 |
| PublicationDate | August 2014 |
| PublicationDateYYYYMMDD | 2014-08-01 |
| PublicationDate_xml | – month: 08 year: 2014 text: August 2014 |
| PublicationDecade | 2010 |
| PublicationPlace | United States |
| PublicationPlace_xml | – name: United States |
| PublicationTitle | The FASEB journal |
| PublicationTitleAlternate | FASEB J |
| PublicationYear | 2014 |
| References | 2312537 - J Bone Joint Surg Am. 1990 Mar;72(3):403-8 8589888 - In Vitro Cell Dev Biol Anim. 1995 Jun;31(6):447-55 18049033 - Hypertens Res. 2007 Oct;30(10):999-1002 21236620 - J Vasc Surg. 2011 Mar;53(3):826-36.e1 7363496 - Clin Genet. 1980 Feb;17(2):115-6 1156158 - Arch Surg. 1975 Aug;110(8):922-8 1281554 - Science. 1992 Dec 11;258(5089):1798-801 8683138 - J Immunol. 1996 Jul 1;157(1):360-8 22615343 - Circulation. 2012 Jun 26;125(25):3182-90 1131001 - Arch Surg. 1975 May;110(5):561-6 11399208 - Genet Med. 2000 Sep-Oct;2(5):278-82 5553126 - Mayo Clin Proc. 1971 Mar;46(3):161-7 2398088 - J Bone Joint Surg Am. 1990 Sep;72(8):1178-84 20130469 - Genet Med. 2010 Apr;12(4):196-203 19556353 - AJNR Am J Neuroradiol. 2009 Sep;30(8):1614-9 9486668 - Circ Res. 1998 Feb 23;82(3):396-403 22070689 - Mini Rev Med Chem. 2012 Feb;12(2):175-83 16435345 - J Orthop Res. 2006 Feb;24(2):124-31 2771024 - Neurosurgery. 1989 Sep;25(3):482-3 15731757 - Nat Genet. 2005 Mar;37(3):275-81 8533811 - Am J Med Genet. 1995 Aug 28;58(2):169-76 23023332 - Nat Genet. 2012 Nov;44(11):1249-54 16344385 - Circulation. 2005 Dec 20;112(25):3884-91 15115832 - N Engl J Med. 2004 Apr 29;350(18):1862-71 3318002 - Stroke. 1987 Nov-Dec;18(6):1037-47 8248168 - Proc Natl Acad Sci U S A. 1993 Nov 15;90(22):10759-63 8047140 - Nature. 1994 Aug 4;370(6488):341-7 7974595 - Stroke. 1994 Dec;25(12):2492-6 12626597 - J Immunol. 2003 Mar 15;170(6):3369-76 12672202 - J Rheumatol. 2003 Apr;30(4):799-803 24399159 - Circ Cardiovasc Genet. 2014 Feb;7(1):80-8 2003292 - Stroke. 1991 Feb;22(2):269-71 23248062 - Circulation. 2012 Dec 18;126(25):2925-7 19303062 - Am J Hum Genet. 2009 Apr;84(4):477-82 19635970 - Circulation. 2009 Aug 11;120(6):526-32 22320226 - Amyloid. 2012 Mar;19(1):5-14 15334479 - Arthritis Rheum. 2004 Aug;50(8):2640-4 18074684 - J Neurosurg Spine. 2007 Dec;7(6):601-9 20686062 - J Bone Joint Surg Am. 2010 Aug 4;92(9):1876-83 6368158 - Dis Mon. 1984 Apr;30(7):1-62 18161745 - J Pathol. 2008 Jan;214(2):199-210 21288095 - N Engl J Med. 2011 Feb 3;364(5):432-42 23064905 - Vasc Med. 2012 Dec;17(6):371-8 2139949 - Presse Med. 1990 Apr 7;19(14):661-7 22836090 - Nat Genet. 2012 Aug;44(8):838-9 23155180 - Circulation. 2012 Dec 18;126(25):3062-9 9971870 - N Engl J Med. 1999 Feb 11;340(6):448-54 |
| References_xml | – reference: 2771024 - Neurosurgery. 1989 Sep;25(3):482-3 – reference: 2139949 - Presse Med. 1990 Apr 7;19(14):661-7 – reference: 3318002 - Stroke. 1987 Nov-Dec;18(6):1037-47 – reference: 16344385 - Circulation. 2005 Dec 20;112(25):3884-91 – reference: 22070689 - Mini Rev Med Chem. 2012 Feb;12(2):175-83 – reference: 9971870 - N Engl J Med. 1999 Feb 11;340(6):448-54 – reference: 18049033 - Hypertens Res. 2007 Oct;30(10):999-1002 – reference: 16435345 - J Orthop Res. 2006 Feb;24(2):124-31 – reference: 19635970 - Circulation. 2009 Aug 11;120(6):526-32 – reference: 19556353 - AJNR Am J Neuroradiol. 2009 Sep;30(8):1614-9 – reference: 12626597 - J Immunol. 2003 Mar 15;170(6):3369-76 – reference: 22320226 - Amyloid. 2012 Mar;19(1):5-14 – reference: 2003292 - Stroke. 1991 Feb;22(2):269-71 – reference: 15115832 - N Engl J Med. 2004 Apr 29;350(18):1862-71 – reference: 19303062 - Am J Hum Genet. 2009 Apr;84(4):477-82 – reference: 8248168 - Proc Natl Acad Sci U S A. 1993 Nov 15;90(22):10759-63 – reference: 8589888 - In Vitro Cell Dev Biol Anim. 1995 Jun;31(6):447-55 – reference: 5553126 - Mayo Clin Proc. 1971 Mar;46(3):161-7 – reference: 20130469 - Genet Med. 2010 Apr;12(4):196-203 – reference: 1281554 - Science. 1992 Dec 11;258(5089):1798-801 – reference: 23155180 - Circulation. 2012 Dec 18;126(25):3062-9 – reference: 2398088 - J Bone Joint Surg Am. 1990 Sep;72(8):1178-84 – reference: 7974595 - Stroke. 1994 Dec;25(12):2492-6 – reference: 15731757 - Nat Genet. 2005 Mar;37(3):275-81 – reference: 12672202 - J Rheumatol. 2003 Apr;30(4):799-803 – reference: 11399208 - Genet Med. 2000 Sep-Oct;2(5):278-82 – reference: 21288095 - N Engl J Med. 2011 Feb 3;364(5):432-42 – reference: 8533811 - Am J Med Genet. 1995 Aug 28;58(2):169-76 – reference: 2312537 - J Bone Joint Surg Am. 1990 Mar;72(3):403-8 – reference: 22836090 - Nat Genet. 2012 Aug;44(8):838-9 – reference: 9486668 - Circ Res. 1998 Feb 23;82(3):396-403 – reference: 23248062 - Circulation. 2012 Dec 18;126(25):2925-7 – reference: 7363496 - Clin Genet. 1980 Feb;17(2):115-6 – reference: 6368158 - Dis Mon. 1984 Apr;30(7):1-62 – reference: 22615343 - Circulation. 2012 Jun 26;125(25):3182-90 – reference: 1131001 - Arch Surg. 1975 May;110(5):561-6 – reference: 18074684 - J Neurosurg Spine. 2007 Dec;7(6):601-9 – reference: 1156158 - Arch Surg. 1975 Aug;110(8):922-8 – reference: 24399159 - Circ Cardiovasc Genet. 2014 Feb;7(1):80-8 – reference: 15334479 - Arthritis Rheum. 2004 Aug;50(8):2640-4 – reference: 23023332 - Nat Genet. 2012 Nov;44(11):1249-54 – reference: 20686062 - J Bone Joint Surg Am. 2010 Aug 4;92(9):1876-83 – reference: 8047140 - Nature. 1994 Aug 4;370(6488):341-7 – reference: 18161745 - J Pathol. 2008 Jan;214(2):199-210 – reference: 8683138 - J Immunol. 1996 Jul 1;157(1):360-8 – reference: 21236620 - J Vasc Surg. 2011 Mar;53(3):826-36.e1 – reference: 23064905 - Vasc Med. 2012 Dec;17(6):371-8 |
| SSID | ssj0001016 |
| Score | 2.4018097 |
| Snippet | Fibromuscular dysplasia (FMD) is a rare, nonatherosclerotic arterial disease for which the molecular basis is unknown. We comprehensively studied 47 subjects... |
| SourceID | pubmed |
| SourceType | Index Database |
| StartPage | 3313 |
| SubjectTerms | Adult Aged Arnold-Chiari Malformation - complications Biomarkers - blood Bone Density Bone Diseases, Metabolic - etiology Case-Control Studies Cell Cycle Cell Line Connective Tissue - pathology Dermis - pathology Dilatation, Pathologic Dura Mater - pathology Female Fibroblasts - secretion Fibromuscular Dysplasia - complications Fibromuscular Dysplasia - metabolism Fibromuscular Dysplasia - pathology Humans Inflammation - blood Inflammation - etiology Inflammation Mediators - blood Joint Instability - etiology Male Middle Aged Phenotype Renal Artery - pathology Single-Blind Method Spine - pathology Transforming Growth Factor beta1 - biosynthesis Transforming Growth Factor beta1 - blood Transforming Growth Factor beta1 - secretion Transforming Growth Factor beta2 - blood Transforming Growth Factor beta2 - secretion Young Adult |
| Title | Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF-β expression and connective tissue features |
| URI | https://www.ncbi.nlm.nih.gov/pubmed/24732132 |
| Volume | 28 |
| hasFullText | |
| inHoldings | 1 |
| isFullTextHit | |
| isPrint | |
| link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnZ1Lb9NAEIBXKQiJS8W7PLUHerIMXr99bKOECqkcIJVyq7zrXexD7Qg7h_Z_8Ec48Sv4TczsIzblIeBiRbac2J4v45nZeRDyMmBCqDgTfpyo1I-jOAE9GBV-JFnCWcRkpPsUnL5LT87it-tkPZt9nWQtbQf-Slz9sq7kf6QK-0CuWCX7D5LdfSnsgM8gX9iChGH7VzKeu7JGjH7zBodfmep_O4m79_rtR1xA8hR4xd3F1madVpf9RpdP4jjz0nZzboRbrbEFb7iODubo6s3SP5wvDo9DHAdg0mZbWw4HSlrrS2_Q8vOU1H1C-6nJiyAujz4sjr3pPWHKTwlqtjZh6XaomzHietp9wjSBwYzue489p3dnrbd6PaVuxiWkupNt3Zn2kkvMKLTE22AGi3epdPAucgoY3NnczBhwGjrMJyTmE3UbRaaQ9af3ADhmKDx8EfhowZnJuhMkNheaiTDOopCZCOufj17ryu0O7ZE98E9w4CpGiawFgBERW2QBF_J6ehnYfNqees2R0QbN6g7Zt54IPTJY3SUz2d4jt8xs0sv75LODi4Ko6QQu6uCiFi76A1x0BxdtelpSBxe1cFGEi1q4KML17QsdwdK_NoJFDVjUgfWAnC0Xq_mJb2d4-CLKscltEcB_vsx5pkAlMJ5KlrGskjkvFAuUBPM3UmCzM5mmFRdhkgvsyKg4ZwIeVBA-JDfarpUHhMZBpso4DwuVcjDyqyJQQRUGHHxiURRx-Jg8Ms_zfGMatZy7J_3kt0eektsjhs_ITQWaQT4HM3PgL7RMvwMzFoLp |
| linkProvider | National Library of Medicine |
| openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Clinical+and+biochemical+profiles+suggest+fibromuscular+dysplasia+is+a+systemic+disease+with+altered+TGF-%CE%B2+expression+and+connective+tissue+features&rft.jtitle=The+FASEB+journal&rft.au=Ganesh%2C+Santhi+K&rft.au=Morissette%2C+Rachel&rft.au=Xu%2C+Zhi&rft.au=Schoenhoff%2C+Florian&rft.date=2014-08-01&rft.eissn=1530-6860&rft.volume=28&rft.issue=8&rft.spage=3313&rft_id=info:doi/10.1096%2Ffj.14-251207&rft_id=info%3Apmid%2F24732132&rft_id=info%3Apmid%2F24732132&rft.externalDocID=24732132 |