Property Summary

NCBI Gene PubMed Count 44
PubMed Score 309.14
PubTator Score 219.35

Knowledge Summary

Patent

No data available

TINX Plot

  Disease Sources (6)

Disease Target Count
cystic fibrosis 1670
Disease Target Count P-value
lung carcinoma 2844 5.8724826130883E-27
juvenile dermatomyositis 1189 4.30716584561662E-14
pilocytic astrocytoma 3086 1.3020225491864E-11
posterior fossa group A ependymoma 1511 7.82982956439311E-11
Duchenne muscular dystrophy 602 5.65967845623337E-10
malignant mesothelioma 3163 2.75779364115992E-9
pituitary cancer 1972 1.32875964859622E-8
ovarian cancer 8492 1.06242419605549E-7
limb girdle muscular dystrophy 2A 156 4.89485817798906E-7
ulcerative colitis 2087 3.93813589863277E-6
lung cancer 4473 3.71750429245409E-5
intraductal papillary-mucinous carcinoma (IPMC) 2988 4.32808928118341E-5
pancreatic cancer 2300 6.64788509761124E-5
primary pancreatic ductal adenocarcinoma 1271 1.44958342874597E-4
intraductal papillary-mucinous adenoma (IPMA) 2956 2.0945465151306E-4
pediatric high grade glioma 2712 4.46805440948144E-4
Becker muscular dystrophy 187 9.75497510669577E-4
acute quadriplegic myopathy 1157 9.93375555646149E-4
invasive ductal carcinoma 2950 0.00124729839866488
intraductal papillary-mucinous neoplasm (IPMN) 3289 0.00139092637014615
dermatomyositis 967 0.00158333393775004
glioblastoma 5572 0.00386263930198118
diabetes mellitus 1663 0.00409661233842118
medulloblastoma, large-cell 6234 0.00485475208267764
interstitial cystitis 2299 0.00516790804847849
gastric carcinoma 832 0.00634064248789148
subependymal giant cell astrocytoma 2287 0.00810933905498821
sonic hedgehog group medulloblastoma 1482 0.0111096669943642
facioscapulohumeral dystrophy 286 0.0143874579226099
colon cancer 1475 0.0147976346428968
primary Sjogren syndrome 789 0.0319135193024398
adrenocortical carcinoma 1427 0.0497289169796496
Disease Target Count Z-score Confidence
Ehlers-Danlos syndrome 42 0.0 4.0

Expression

Synonym

Accession P00736 A6NJQ8 Q68D77 Q8J012
Symbols EDSPD1

Gene

C1R

PDB

1APQ   1GPZ   1MD7   1MD8   2QY0  

  Ortholog (8)

Species Source
Chimp OMA Inparanoid
Mouse OMA Inparanoid
Rat OMA Inparanoid
Dog OMA Inparanoid
Cow OMA Inparanoid
Pig OMA Inparanoid
Opossum OMA Inparanoid
Chicken OMA Inparanoid

Gene RIF (15)

PMID Text
26709396 We confirmed increased levels of C1R and VTN in sera from patients with Joint hypermobility syndrome by western blot analyses
26231209 C1q exists as the C1 complex (C1qC1r2C1s2), and C1q binding to ligands activates the C1r/C1s proteases. Incubation of nucleoli with C1 caused degradation of the nucleolar proteins nucleolin and nucleophosmin 1. T
23650384 Analysis of its interaction properties by surface plasmon resonance shows that rC1q retains the ability of serum C1q to associate with the C1s-C1r-C1r-C1s tetramer, to recognize physiological C1q ligands such as IgG and pentraxin 3
23589288 C1r specificity is well suited to its cleavage targets and that efficient cleavage of C1s is achieved through both active site and exosite contributions.
20970424 a structural rearrangement as a switch between functional states of human C1r
20796027 The modular C1r protein is the first protease activated in the classical complement pathway, a key component of innate immunity.
20592021 These results provide further structural insights into the architecture of the C1 complex, and the interactions between C1r and C1s.
20178990 Using a recombinant CUB2-CCP1 domain pair and the individual CCP1 module, we showed that binding of Ca(2+) induces the folding of the CUB2 domain and stabilizes its structure.
20008834 Detailed mapping of C1q post-translational modifications and insights into the C1r/C1s binding sites.
17996945 The activated CCP1-CCP2-SP fragment makes up a dimer in a head-to-tail fashion similarly to the previously characterized zymogen.
More...

AA Sequence

MWLLYLLVPALFCRAGGSIPIPQKLFGEVTSPLFPKPYPNNFETTTVITVPTGYRVKLVFQQFDLEPSEG      1 - 70
CFYDYVKISADKKSLGRFCGQLGSPLGNPPGKKEFMSQGNKMLLTFHTDFSNEENGTIMFYKGFLAYYQA     71 - 140
VDLDECASRSKSGEEDPQPQCQHLCHNYVGGYFCSCRPGYELQEDTHSCQAECSSELYTEASGYISSLEY    141 - 210
PRSYPPDLRCNYSIRVERGLTLHLKFLEPFDIDDHQQVHCPYDQLQIYANGKNIGEFCGKQRPPDLDTSS    211 - 280
NAVDLLFFTDESGDSRGWKLRYTTEIIKCPQPKTLDEFTIIQNLQPQYQFRDYFIATCKQGYQLIEGNQV    281 - 350
LHSFTAVCQDDGTWHRAMPRCKIKDCGQPRNLPNGDFRYTTTMGVNTYKARIQYYCHEPYYKMQTRAGSR    351 - 420
ESEQGVYTCTAQGIWKNEQKGEKIPRCLPVCGKPVNPVEQRQRIIGGQKAKMGNFPWQVFTNIHGRGGGA    421 - 490
LLGDRWILTAAHTLYPKEHEAQSNASLDVFLGHTNVEELMKLGNHPIRRVSVHPDYRQDESYNFEGDIAL    491 - 560
LELENSVTLGPNLLPICLPDNDTFYDLGLMGYVSGFGVMEEKIAHDLRFVRLPVANPQACENWLRGKNRM    561 - 630
DVFSQNMFCAGHPSLKQDACQGDSGGVFAVRDPNTDRWVATGIVSWGIGCSRGYGFYTKVLNYVDWIKKE    631 - 700
MEEED                                                                     701 - 705
//

Text Mined References (50)

PMID Year Title
26709396 2016 Proteomic analysis for the identification of serum diagnostic markers for joint hypermobility syndrome.
26231209 2015 C1q protein binds to the apoptotic nucleolus and causes C1 protease degradation of nucleolar proteins.
24275569 2014 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
23650384 2013 Expression of recombinant human complement C1q allows identification of the C1r/C1s-binding sites.
23589288 2013 Molecular determinants of the substrate specificity of the complement-initiating protease, C1r.
23533145 2013 In-depth proteomic analyses of exosomes isolated from expressed prostatic secretions in urine.
22516433 2012 Proteomic analysis of microvesicles from plasma of healthy donors reveals high individual variability.
22028381 2011 Quantitative detection of single amino acid polymorphisms by targeted proteomics.
20970424 2010 Interaction between separated consecutive complement control modules of human C1r: implications for dimerization of the full-length protease.
20796027 2010 Intermodule cooperativity in the structure and dynamics of consecutive complement control modules in human C1r: structural biology.
More...