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Term:idiopathic pulmonary fibrosis
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Accession:DOID:0050156 term browser browse the term
Definition:A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.
Synonyms:exact_synonym: Familial Idiopathic Pulmonary Fibrosis;   Fibrocystic Pulmonary Dysplasia;   Fibrocystic Pulmonary Dysplasias;   IPF;   Idiopathic Fibrosing Alveolitis, Chronic Form;   Idiopathic Pulmonary Fibroses;   cryptogenic fibrosing alveolitides;   cryptogenic fibrosing alveolitis;   usual interstitial pneumonia;   usual interstitial pneumonias;   usual interstitial pneumonitides;   usual interstitial pneumonitis
 narrow_synonym: Hamman Rich disease;   Hamman-Rich diseases;   UIP Hamman Rich disease
 related_synonym: idiopathic pulmonary fibrosis, susceptibility to
 primary_id: MESH:D054990
 alt_id: OMIM:178500
 xref: GARD:8609;   ICD9CM:516.31;   NCI:C35715;   NCI:C35716
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idiopathic pulmonary fibrosis term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Abca3 ATP binding cassette subfamily A member 3 JBrowse link NW_004955442 14,933,550 14,989,492 RGD:9068941
G Ahrr aryl-hydrocarbon receptor repressor JBrowse link NW_004955504 121,739 191,871 RGD:9068941
G Apoa1 apolipoprotein A1 JBrowse link NW_004955412 18,170,331 18,172,298 RGD:9068941
G Atp11a ATPase phospholipid transporting 11A JBrowse link NW_004955404 820,788 906,531 RGD:9068941
G Bmp1 bone morphogenetic protein 1 JBrowse link NW_004955403 45,660,591 45,693,724 RGD:9068941
G Brd4 bromodomain containing 4 JBrowse link NW_004955495 7,959,033 7,989,216 RGD:9068941
G Brd9 bromodomain containing 9 JBrowse link NW_004955504 416,145 434,132 RGD:9068941
G Cep72 centrosomal protein 72 JBrowse link NW_004955504 305,181 335,791 RGD:9068941
G Cflar CASP8 and FADD like apoptosis regulator JBrowse link NW_004955403 415,092 442,214 RGD:9068941
G Ctc1 CST telomere replication complex component 1 JBrowse link NW_004955467 8,673,069 8,700,730 RGD:9068941
G Dpp9 dipeptidyl peptidase 9 JBrowse link NW_004955495 4,281,262 4,313,674 RGD:9068941
G Dsp desmoplakin JBrowse link NW_004955465 6,356,970 6,391,206 RGD:9068941
G Dtnbp1 dystrobrevin binding protein 1 JBrowse link NW_004955590 276,143 382,812 RGD:9068941
G Ehmt2 euchromatic histone lysine methyltransferase 2 JBrowse link NW_004955437 396,736 411,144 RGD:9068941
G Esr1 estrogen receptor 1 JBrowse link NW_004955439 9,588,631 9,953,606 RGD:9068941
G Exoc3 exocyst complex component 3 JBrowse link NW_004955504 195,743 223,812 RGD:9068941
G F3 coagulation factor III, tissue factor JBrowse link NW_004955423 254,060 264,448 RGD:9068941
G Fam13a family with sequence similarity 13 member A JBrowse link NW_004955474 11,615,090 11,712,766 RGD:9068941
G Fas Fas cell surface death receptor JBrowse link NW_004955425 3,961,671 3,987,513 RGD:9068941
G Faslg Fas ligand JBrowse link NW_004955406 13,098,479 13,107,045 RGD:9068941
G Gper1 G protein-coupled estrogen receptor 1 JBrowse link NW_004955460 8,790,798 8,794,902 RGD:9068941
G Hif1a hypoxia inducible factor 1 subunit alpha JBrowse link NW_004955466 8,000,311 8,022,497 RGD:9068941
G Hps4 HPS4 biogenesis of lysosomal organelles complex 3 subunit 2 JBrowse link NW_004955455 1,048,946 1,076,097 RGD:9068941
G Il1rl1 interleukin 1 receptor like 1 JBrowse link NW_004955470 7,779,828 7,849,157 RGD:9068941
G Il1rn interleukin 1 receptor antagonist JBrowse link NW_004955470 1,145,022 1,159,413 RGD:9068941
G Il2ra interleukin 2 receptor subunit alpha JBrowse link NW_004955421 22,642,004 22,694,729 RGD:9068941
G Lama3 laminin subunit alpha 3 JBrowse link NW_004955402 11,980,980 12,362,143 RGD:9068941
G Mmp19 matrix metallopeptidase 19 JBrowse link NW_004955458 3,473,279 3,479,620 RGD:9068941
G Muc1 mucin 1, cell surface associated JBrowse link NW_004955545 1,657,728 1,662,112 RGD:9068941
G Muc5b mucin 5B, oligomeric mucus/gel-forming JBrowse link NW_004955476 11,021,665 11,049,239 RGD:7240710
G Nkd2 NKD inhibitor of WNT signaling pathway 2 JBrowse link NW_004955504 507,253 527,275 RGD:9068941
G Ntrk2 neurotrophic receptor tyrosine kinase 2 JBrowse link NW_004955432 2,284,283 2,629,631 RGD:9068941
G Parn poly(A)-specific ribonuclease JBrowse link NW_004955442 4,255,726 4,421,083 RGD:9068941
G Parp1 poly(ADP-ribose) polymerase 1 JBrowse link NW_004955489 520,875 557,609 RGD:9068941
G Pdcd6 programmed cell death 6 JBrowse link NW_004955504 75,378 94,654 RGD:9068941
G Plau plasminogen activator, urokinase JBrowse link NW_004955437 17,995,726 18,000,542 RGD:9068941
G Ptgs2 prostaglandin-endoperoxide synthase 2 JBrowse link NW_004955406 25,077,988 25,086,259 RGD:9068941
G Rtel1 regulator of telomere elongation helicase 1 JBrowse link NW_004955528 531,680 578,003 RGD:9068941
G Rtkn2 rhotekin 2 JBrowse link NW_004955425 15,484,287 15,563,535 RGD:9068941
G Sdha succinate dehydrogenase complex flavoprotein subunit A JBrowse link NW_004955504 36,939 66,507 RGD:9068941
G Sftpc surfactant protein C JBrowse link NW_004955403 45,649,149 45,651,421 RGD:9068941
G Slc12a7 solute carrier family 12 member 7 JBrowse link NW_004955504 529,183 589,728 RGD:9068941
G Slc6a18 solute carrier family 6 member 18 JBrowse link NW_004955504 634,615 644,667 RGD:9068941
G Slc6a19 solute carrier family 6 member 19 JBrowse link NW_004955504 617,683 633,594 RGD:9068941
G Slc9a3 solute carrier family 9 member A3 JBrowse link NW_004955504 226,192 260,632 RGD:9068941
G St14 ST14 transmembrane serine protease matriptase JBrowse link NW_004955412 31,091,313 31,129,050 RGD:9068941
G Stat3 signal transducer and activator of transcription 3 JBrowse link NW_004955451 16,320,326 16,348,505 RGD:9068941
G Stn1 STN1 subunit of CST complex JBrowse link NW_004955485 6,748,682 6,784,119 RGD:9068941
G Tert telomerase reverse transcriptase JBrowse link NW_004955504 649,248 669,796 RGD:9068941
G Tfpi tissue factor pathway inhibitor JBrowse link NW_004955403 10,925,155 11,004,229 RGD:9068941
G Thbd thrombomodulin JBrowse link NW_004955415 30,222,830 30,226,406 RGD:9068941
G Trip13 thyroid hormone receptor interactor 13 JBrowse link NW_004955504 434,187 452,636 RGD:9068941
G Wnt5a Wnt family member 5A JBrowse link NW_004955430 5,304,817 5,325,684 RGD:9068941

Term paths to the root
Path 1
Term Annotations click to browse term
  disease 11744
    Pathological Conditions, Signs and Symptoms 6852
      Pathologic Processes 5035
        Fibrosis 1187
          pulmonary fibrosis 183
            idiopathic pulmonary fibrosis 53
Path 2
Term Annotations click to browse term
  disease 11744
    disease of anatomical entity 11304
      respiratory system disease 2252
        lower respiratory tract disease 1404
          lung disease 1370
            pneumonia 204
              idiopathic interstitial pneumonia 60
                idiopathic pulmonary fibrosis 53
paths to the root


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.