C9

Summary

Gene Symbol: C9
Description: complement component 9
Alias: complement component C9
Species: human

Top Publications

  1. ncbi The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain of C9
    H Ninomiya
    Oklahoma Medical Research Foundation, Oklahoma City 73104
    J Biol Chem 267:13675-80. 1992
  2. ncbi Hereditary complement (C9) deficiency associated with dermatomyositis
    E Ichikawa
    Department of Dermatology, Institute of Clinical Medicine, University of Tsukuba, 1 1 1 Tenno Dai, Tsukuba, Ibaraki 305 8575, Japan
    Br J Dermatol 144:1080-3. 2001
  3. ncbi Founder effect of the C9 R95X mutation in Orientals
    Vahid Khajoee
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Hum Genet 112:244-8. 2003
  4. ncbi Deficiency of complement defense protein CD59 may contribute to neurodegeneration in Alzheimer's disease
    L B Yang
    L J Roberts Center for Alzheimer s Research, Sun Health Research Institute, Sun City, Arizona 85351, USA
    J Neurosci 20:7505-9. 2000
  5. ncbi The human complement C9 gene: structural analysis of the 5' gene region and genetic polymorphism studies
    K Witzel-Schlömp
    Institute of Legal Medicine, Johannes Gutenberg University, Mainz, Germany
    Eur J Immunogenet 28:515-22. 2001
  6. ncbi Binding of human complement C8 to C9: role of the N-terminal modules in the C8 alpha subunit
    Jeffery J Scibek
    Department of Chemistry and Biochemistry and School of Medicine, University of South Carolina, Columbia, South Carolina 29208, USA
    Biochemistry 41:14546-51. 2002
  7. ncbi Functional studies of the MACPF domain of human complement protein C8alpha reveal sites for simultaneous binding of C8beta, C8gamma, and C9
    Daniel J Slade
    Department of Chemistry and Biochemistry and School of Medicine, University of South Carolina, Columbia, South Carolina 29208, USA
    Biochemistry 45:5290-6. 2006
  8. ncbi Defining the CD59-C9 binding interaction
    Yuxiang Huang
    Department of Microbiology and Immunology, Medical University of South Carolina, South Carolina 29403, USA
    J Biol Chem 281:27398-404. 2006
  9. ncbi Levels of complement components iC3b, C3c, C4, and SC5b-9 in peritoneal fluid and serum of infertile women with endometriosis
    Jacek Kabut
    Department of Immunology and Serology, Medical University of Silesia, Katowice, Poland
    Fertil Steril 88:1298-303. 2007
  10. ncbi Live cell imaging of outward and inward vesiculation induced by the complement c5b-9 complex
    Oren Moskovich
    Department of Cell and Developmental Biology, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    J Biol Chem 282:29977-86. 2007

Scientific Experts

  • Takahiko Horiuchi
  • E Ichikawa
  • J Hofsteenge
  • Zvi Fishelson
  • Joon Seol Bae
  • Stephanie E Wickham
  • Takayoshi Miura
  • Véronique Rossi
  • Osama A Hamad
  • David Pilzer
  • Dana Gancz
  • Matthew Fosbrink
  • Stephen Tomlinson
  • K Witzel-Schlömp
  • Jacek Kabut
  • Oren Moskovich
  • Yuxiang Huang
  • Daniel J Slade
  • James M Sodetz
  • Vahid Khajoee
  • Jeffery J Scibek
  • Jin Sol Lee
  • Ichiei Narita
  • Byung Lae Park
  • Galina Polekhina
  • Eileen M Hotze
  • In Song Koh
  • Tae Joon Park
  • Shin Goto
  • Hyun Sub Cheong
  • Michael W Parker
  • Jeong Hyun Kim
  • Charisse Flerida A Pasaje
  • Shin ichi Nishi
  • Rodney K Tweten
  • Seitaro Iguchi
  • Hisaki Shimada
  • Hyo Suk Lee
  • Yoon Jun Kim
  • Mitsuhiro Ueno
  • Tracy L Nero
  • Jason Yongha Kim
  • Hyoung Doo Shin
  • Allison J Farrand
  • L B Yang
  • Kristina N Ekdahl
  • Per H Nilsson
  • Yunxia Wang
  • Diana Wouters
  • ALFRED F ESSER
  • Bo Nilsson
  • Keizo Koya
  • John D Lambris
  • Moran Saar
  • Kateryna Soloviova
  • Florin Niculescu
  • Violeta Rus
  • Takahiro Ito
  • Horea Rus
  • Cosmin A Tegla
  • Cornelia Cudrici
  • Sonia Vlaicu
  • Natalie Donin
  • P M Schneider
  • C Rittner
  • Justyna Sikora
  • Aleksandra Mielczarek-Palacz
  • Zdzisława Kondera-Anasz
  • Brian Chiswell
  • R Kira
  • Starr Hazard
  • Fei Qiao
  • Ruben Abagyan
  • Ryutaro Kira
  • Katsushi Tokunaga
  • Kenji Ihara
  • Jia Guanjun
  • Yasunari Sakai
  • Park Myoung Hee
  • Toshiro Hara
  • Megumi Takemoto
  • Hiroyuki Torisu
  • S Lengweiler
  • C Susal
  • Mnason E Plumb
  • S Meri
  • R Li
  • J Rogers
  • Y Shen
  • J Tschopp

Detail Information

Publications37

  1. ncbi The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain of C9
    H Ninomiya
    Oklahoma Medical Research Foundation, Oklahoma City 73104
    J Biol Chem 267:13675-80. 1992
    ..between 125I-CD59 and the isolated components of human complement membrane attack complex, C5b6, C7, C8, and C9. By density gradient analysis, we were unable to detect interaction of 125I-CD59 with any of these isolated ..
  2. ncbi Hereditary complement (C9) deficiency associated with dermatomyositis
    E Ichikawa
    Department of Dermatology, Institute of Clinical Medicine, University of Tsukuba, 1 1 1 Tenno Dai, Tsukuba, Ibaraki 305 8575, Japan
    Br J Dermatol 144:1080-3. 2001
    A 28-year-old Japanese woman with hereditary complement (C9) deficiency and dermatomyositis is reported. She had a 3-year history of facial erythema and a 1-month history of progressive muscle weakness...
  3. ncbi Founder effect of the C9 R95X mutation in Orientals
    Vahid Khajoee
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Hum Genet 112:244-8. 2003
    A nonsense mutation at codon 95 (R95X) in the C9 gene is responsible for most Japanese C9 deficiency (C9D) cases, with a carrier frequency of 6.7%...
  4. ncbi Deficiency of complement defense protein CD59 may contribute to neurodegeneration in Alzheimer's disease
    L B Yang
    L J Roberts Center for Alzheimer s Research, Sun Health Research Institute, Sun City, Arizona 85351, USA
    J Neurosci 20:7505-9. 2000
    ..Taken together, these data suggest that CD59 deficits may play a role in the neuritic losses characteristic of AD...
  5. ncbi The human complement C9 gene: structural analysis of the 5' gene region and genetic polymorphism studies
    K Witzel-Schlömp
    Institute of Legal Medicine, Johannes Gutenberg University, Mainz, Germany
    Eur J Immunogenet 28:515-22. 2001
    b>C9 is the last of the human complement components creating the membrane attack complex. The single chain serum protein is encoded by a gene located on chromosome 5p13 that is composed of 11 exons...
  6. ncbi Binding of human complement C8 to C9: role of the N-terminal modules in the C8 alpha subunit
    Jeffery J Scibek
    Department of Chemistry and Biochemistry and School of Medicine, University of South Carolina, Columbia, South Carolina 29208, USA
    Biochemistry 41:14546-51. 2002
    ..During MAC formation, C8alpha mediates binding and self-polymerization of C9 to form a pore-like structure on the membrane of target cells...
  7. ncbi Functional studies of the MACPF domain of human complement protein C8alpha reveal sites for simultaneous binding of C8beta, C8gamma, and C9
    Daniel J Slade
    Department of Chemistry and Biochemistry and School of Medicine, University of South Carolina, Columbia, South Carolina 29208, USA
    Biochemistry 45:5290-6. 2006
    ..C8alpha, C8beta, and complement components C6, C7, and C9 form the MAC family of proteins...
  8. ncbi Defining the CD59-C9 binding interaction
    Yuxiang Huang
    Department of Microbiology and Immunology, Medical University of South Carolina, South Carolina 29403, USA
    J Biol Chem 281:27398-404. 2006
    ..It functions by binding to C8 (alpha chain) and C9 after their structural rearrangement during MAC assembly...
  9. ncbi Levels of complement components iC3b, C3c, C4, and SC5b-9 in peritoneal fluid and serum of infertile women with endometriosis
    Jacek Kabut
    Department of Immunology and Serology, Medical University of Silesia, Katowice, Poland
    Fertil Steril 88:1298-303. 2007
    ..To assess whether complement components iC3b, C3c, C4, and SC5b-9 may be involved in the pathogenesis of endometriosis...
  10. ncbi Live cell imaging of outward and inward vesiculation induced by the complement c5b-9 complex
    Oren Moskovich
    Department of Cell and Developmental Biology, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    J Biol Chem 282:29977-86. 2007
    ..To gain an insight into the route of MAC removal, human C9 was tagged with Alexa Fluor 488 and traced within live cells...
  11. ncbi Response gene to complement 32 is required for C5b-9 induced cell cycle activation in endothelial cells
    Matthew Fosbrink
    Department of Neurology, University of Maryland, School of Medicine, Baltimore, MD 21201, USA
    Exp Mol Pathol 86:87-94. 2009
    ..All these data together suggest that cell cycle induction by C5b-9 in AEC is RGC-32 dependent and this is in part through regulation of Akt and growth factor release...
  12. ncbi Mortalin inhibitors sensitize K562 leukemia cells to complement-dependent cytotoxicity
    David Pilzer
    Department of Cell and Developmental Biology, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    Int J Cancer 126:1428-35. 2010
    ..In the presence of MKT-077, the direct binding of mortalin to complement C9, the major MAC component, is inhibited. The tumor suppressor protein p53 is a known mortalin client protein...
  13. ncbi Involvement of the c-jun N-terminal kinases JNK1 and JNK2 in complement-mediated cell death
    Dana Gancz
    Department of Cell and Developmental Biology, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    Mol Immunol 47:310-7. 2009
    ..Our results demonstrate that complement C5b-9 induce a JNK/Bid-dependent and JNK-independent necrotic cell death. Both JNK1 and JNK2 have cytotoxic potential, however JNK2 is the primary signal transducer...
  14. ncbi Complement component C3 binds to activated normal platelets without preceding proteolytic activation and promotes binding to complement receptor 1
    Osama A Hamad
    Division of Clinical Immunology, Rudbeck Laboratory C5, Uppsala University, Uppsala, Sweden
    J Immunol 184:2686-92. 2010
    ..C1q, C4, C3, and C9 were found to bind to thrombin receptor-activating peptide-activated platelets in lepirudin-anticoagulated platelet-..
  15. ncbi Topology of the membrane-bound form of complement protein C9 probed by glycosylation mapping, anti-peptide antibody binding, and disulfide modification
    Véronique Rossi
    Division of Cell Biology and Biophysics, School of Biological Sciences, University of Missouri Kansas City, 5100 Rockhill Road, Kansas City, MO 64110, USA
    Mol Immunol 47:1553-60. 2010
    The two N-linked oligosaccharides in native human C9 were deleted by site-specific mutagenesis. This aglycosyl-C9 did not differ from its native form in hemolytic and bactericidal activity...
  16. ncbi Membranoproliferative pattern of glomerular injury associated with complement component 9 deficiency due to Arg95Stop mutation
    Takayoshi Miura
    Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Asahimachi Dori, Niigata 951 8510, Japan
    Clin Exp Nephrol 15:86-91. 2011
    Arg95Stop mutation of exon 4 in complement component 9 (C9) gene is common in individuals in Japan with C9 deficiency (C9D); however, understanding of the influences of C9D on human glomerulonephritis remains elusive.
  17. ncbi Genetic analysis of complement component 9 (C9) polymorphisms with clearance of hepatitis B virus infection
    Joon Seol Bae
    Laboratory of Genomic Diversity, Department of Life Science, Sogang University, Shinsu dong, Mapo gu, Seoul 121 742, Republic of Korea
    Dig Dis Sci 56:2735-41. 2011
    The complement component 9 (C9), a major cytolytic protein in the complement system, plays an important role in the immunological process...
  18. ncbi The four terminal components of the complement system are C-mannosylated on multiple tryptophan residues
    J Hofsteenge
    Friedrich Miescher Institut, Switzerland
    J Biol Chem 274:32786-94. 1999
    ..and to learn more about its mechanism, the terminal components of the human complement system (C6, C7, C8,and C9), which contain multiple and complex recognition motifs, were examined...
  19. ncbi Nonsense mutation in exon 4 of human complement C9 gene is the major cause of Japanese complement C9 deficiency
    R Kira
    Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan
    Hum Genet 102:605-10. 1998
    Deficiency of the ninth component of human complement (C9) is the most common complement deficiency in Japan but is rare in other countries...
  20. ncbi DNA polymorphisms and linkage relationship of the human complement component C6, C7, and C9 genes
    E Coto
    Servicio de Immunologia, Hospital Covadonga, Oviedo, Spain
    Immunogenetics 33:184-7. 1991
    In this report we describe the linkage between genes encoding human complement components C6, C7, and C9. Polymorphisms have been described at the DNA level for the C7 and C9 genes...
  21. ncbi Localization and molecular modelling of the membrane-inserted domain of the ninth component of human complement and perforin
    M C Peitsch
    Institute of Mathematical Biology, National Cancer Institute, Frederick, MD 21701
    Mol Immunol 27:589-602. 1990
    Upon interaction with the membrane-bound C5b-8 complex, the ninth component of complement (C9) unfolds and inserts into the membrane of cells on which surface complement has been activated...
  22. ncbi The ninth component of human complement (C9). Functional activity of the b fragment
    J W Shiver
    J Biol Chem 261:9629-36. 1986
    The domain structure of human complement protein C9 was investigated by determining the functional activities of the NH2-terminal (C9a) and COOH-terminal (C9b) fragments obtained by cleavage of C9 with alpha-thrombin...
  23. ncbi The gene for human complement component C9 mapped to chromosome 5 by polymerase chain reaction
    C Abbott
    MRC Human Biochemical Genetics Unit, Galton Laboratory, University College London, United Kingdom
    Genomics 4:606-9. 1989
    The gene for human complement component C9 has been mapped to chromosome 5...
  24. ncbi Relationships between the gene and protein structure in human complement component C9
    D Marazziti
    European Molecular Biology Laboratory, Heidelberg, FRG
    Biochemistry 27:6529-34. 1988
    Human complement component C9 is a multidomain protein for which a large number of surface topographical features have been determined...
  25. ncbi Evidence that C5b recognizes and mediates C8 incorporation into the cytolytic complex of complement
    J L Stewart
    J Immunol 139:1960-4. 1987
    ..Simultaneous interaction of C8 with C5 and C9 in solution suggests that during assembly of the cytolytic C5b-9 complex on membranes, C8 binds to C5b-7 through ..
  26. ncbi The sequence and topology of human complement component C9
    K K Stanley
    EMBO J 4:375-82. 1985
    A partial nucleotide sequence of human complement component C9 cDNA representing 94% of the coding region of the mature protein is presented...
  27. ncbi Analysis of the specific association of the eighth and ninth components of human complement: identification of a direct role for the alpha subunit of C8
    J L Stewart
    Biochemistry 24:4598-602. 1985
    The basis for the physical association between C8 and C9 in solution was examined by isolating the noncovalently associated alpha-gamma and beta subunits of C8 and determining their respective affinities for C9...
  28. ncbi Nucleotide sequence of cDNA and derived amino acid sequence of human complement component C9
    R G DiScipio
    Proc Natl Acad Sci U S A 81:7298-302. 1984
    The nucleotide sequence coding for the ninth component of human complement (C9) has been determined and the corresponding amino acid sequence has been derived...
  29. ncbi Identity of a peptide domain of human C9 that is bound by the cell-surface complement inhibitor, CD59
    C P Chang
    Blood Research Institute, Blood Center of Southeastern Wisconsin, Milwaukee 53233
    J Biol Chem 269:26424-30. 1994
    ..in the alpha-chain subunit of human C8, as well as within the C9b domain (amino acid residues 245-538) of human C9, during assembly of the C5b-9 complex on the target membrane (Ninomiya, H., and Sims, P. J. (1992) J. Biol. Chem...
  30. ncbi Clusterin, the human apolipoprotein and complement inhibitor, binds to complement C7, C8 beta, and the b domain of C9
    J Tschopp
    Institute of Biochemistry, University of Lausanne, Epalinges, Switzerland
    J Immunol 151:2159-65. 1993
    ..the binding interactions between [125I]clusterin and the isolated components of the complex, C5b-6, C7, C8, and C9 and vitronectin...
  31. ncbi The expression of hemolytically active human complement protein C9 in mammalian, insect, and yeast cells
    S Tomlinson
    Department of Comparative and Experimental Pathology, University of Florida, Gainesville 32610
    Protein Expr Purif 4:141-8. 1993
    The cDNA sequence encoding mature human C9 protein and its signal peptide was cloned into three expression vectors for expression in COS-7 (mammalian), Spodoptera frugiperda IPLB-SF-21AE (insect), and Saccharomyces cerevisiae (yeast) ..
  32. ncbi Identification of disulfide bonds in the ninth component (C9) of human complement
    S Lengweiler
    Institute of Biochemistry, University of Bern, Switzerland
    FEBS Lett 380:8-12. 1996
    b>C9 is the most abundant protein of the membrane attack complex of complement...
  33. ncbi Identification of complement activation sites in human immunodeficiency virus type-1 glycoprotein gp120
    C Susal
    Department of Transplantation Immunology, Institute of Immunology, University of Heidelberg, Germany
    Blood 87:2329-36. 1996
    ..In the current study, the complement proteins C4,C3,C5,C9, and properdin were found to bind to a synthetic peptide covering positions 233-251 of the gp120BRU sequence on ..
  34. ncbi The human complement C9 gene: identification of two mutations causing deficiency and revision of the gene structure
    K Witzel-Schlömp
    Institute of Legal Medicine, Johannes Gutenberg University, Mainz, Germany
    J Immunol 158:5043-9. 1997
    The ninth component of human complement (C9) is the last of the terminal complement components creating the membrane attack complex. C9 is a single-chain serum protein that is encoded by a gene located on chromosome 5p...
  35. ncbi A non-sense mutation at Arg95 is predominant in complement 9 deficiency in Japanese
    T Horiuchi
    First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan
    J Immunol 160:1509-13. 1998
    ..As a screening step for mutations, exons 2 to 11 of the C9 gene were analyzed using exon-specific PCR/single-strand conformation polymorphism analysis, which demonstrated ..
  36. ncbi Mapping the intermedilysin-human CD59 receptor interface reveals a deep correspondence with the binding site on CD59 for complement binding proteins C8alpha and C9
    Stephanie E Wickham
    Department of Microbiology and Immunology, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, USA
    J Biol Chem 286:20952-62. 2011
    ..a remarkably deep correspondence between the hCD59 binding site for ILY and that for the MAC proteins C8? and C9. ILY disengages from hCD59 during the prepore to pore transition, suggesting that loss of this interaction is ..