title: Potential diagnostic biomarkers in serum of idiopathic pulmonary arterial hypertension.
abstract: BACKGROUND
  The pathogenesis of idiopathic pulmonary arterial hypertension (IPAH) is unknown, and the syndrome of IPAH remains a diagnostic and therapeutic challenge. The present study investigated the disease-specific proteins that aid in the diagnosis of IPAH and thus to study their role in the disease process.
  
  
  METHODS
  A comparative proteomic analysis was used for clinical screening of serum proteins in 10 patients with IPAH and compared with 10 normal subjects. Furthermore, enzyme linked immunosorbent assay (ELISA) was performed for comparison with serum proteins between individual IPAH patients and controls.
  
  
  RESULTS
  Nine proteins and their isoforms, including leucine-rich alpha-2-glycoprotein (LRG), haptoglobin precursor, albumin isoform 2, transferrin variant, C3 complement, hydroxypyruvate reductase isoform 1, RAF1, fibrinogen isoformgamma-A and fibrinogen isoformgamma-B showed significant changes in serum of IPAH patients compared with controls by proteomic analysis. And significant higher serum levels of LRG in IPAH patients compared with controls were found by ELISA. Correlation analysis disclosed a significant association between serum LRG concentrations and New York Heart Association (NYHA) functional class (r=0.71, P<0.01) and cardiac output (CO) (r=-0.65, P<0.01).
  
  
  CONCLUSIONS
  These results indicate that there are significant differences in the expression of proteins in the serum of patients with IPAH and normal subjects. And the measurement of LRG, RAF1 and C3 complement levels in the serum may be helpful for the diagnosis of IPAH. In particular, LRG may be a specific prognostical biomarker of IPAH.
authors.affiliation: Research Center for Pathology and Physiology, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Bei-Li-Shi Road, Xi Cheng District, Beijing 100037, China.
authors.forename: Jianqiang
authors.initials: J
authors.lastname: Zhang
authors.forename: Ying
authors.initials: Y
authors.lastname: Zhang
authors.forename: Ning
authors.initials: N
authors.lastname: Li
authors.forename: Zhihong
authors.initials: Z
authors.lastname: Liu
authors.forename: Changming
authors.initials: C
authors.lastname: Xiong
authors.forename: Xinhai
authors.initials: X
authors.lastname: Ni
authors.forename: Yaoli
authors.initials: Y
authors.lastname: Pu
authors.forename: Rutai
authors.initials: R
authors.lastname: Hui
authors.forename: Jianguo
authors.initials: J
authors.lastname: He
authors.forename: Jielin
authors.initials: J
authors.lastname: Pu
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D015180:Electrophoresis, Gel, Two-Dimensional; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006023:Glycoproteins / Q000097:blood; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000175:diagnosis*; D008297:Male; D011506:Proteins / Q000032:analysis*; D019908:Proto-Oncogene Proteins c-raf / Q000097:blood; D055815:Young Adult
chemical_list: D015415:Biomarkers; D006023:Glycoproteins; D011506:Proteins; D019908:Proto-Oncogene Proteins c-raf