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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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Gene Review: 

PFDN6  -  prefoldin subunit 6

Homo sapiens

Synonyms: H2-KE2, HKE2, KE-2, PFD6, Prefoldin subunit 6, ...
 
 
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Disease relevance of PFDN6

 

High impact information on PFDN6

  • RESULTS: The nonamyloid deposits observed in CDGGI stained intensively with KE-15 and KE-2, whereas the amyloid deposits in all analyzed CDLI corneas reacted to KE-2 but not to KE-15 [2].
  • Characterization of HKE2: an ancient antigen encoded in the major histocompatibility complex [3].
  • The localization of the HKE2 gene to the class II region, its cytoplasmic expression and putative protein-binding domain suggest that HKE2 may function in adaptive immunity and cancer [3].
  • Deposits between the epithelial layer and Bowman's layer stained with Masson's trichrome but not with Congo red in five of the seven corneas; these deposits were stained with both KE-2 and KE-15 [4].
  • By means of fluorescence microscopy the neuroblastoma cells are labeled with rhodamine only, and the false + pre-B lymphocytes are double labeled with both rhodamine (Rh) and fluorescein (FI) since they are HLA+ and react with KE2 [1].
 

Anatomical context of PFDN6

  • METHODS: KE-15 and KE-2, two rabbit antisera raised against peptides from the 69-364 and 426 - 682 amino acid regions of KE respectively, were used for immunohistology of the corneas obtained after keratoplasty in six CDLI patients, three CDGGI patients, and one CDA patient [2].
  • Two monoclonal antibodies are used, PI153/3, IgM class, recognizes NBL and some pre-B lymphocytes and KE2 IgG class recognizes HLA [1].

References

  1. A method of detecting neuroblastoma in human bone marrow by means of two monoclonal antibodies PI 153/3 and KE2. Evans, A.E., Griffin, G.C., Tartaglione, M., Kennett, R.H. Hybridoma (1985) [Pubmed]
  2. On the role of kerato-epithelin in the pathogenesis of 5q31-linked corneal dystrophies. Korvatska, E., Munier, F.L., Chaubert, P., Wang, M.X., Mashima, Y., Yamada, M., Uffer, S., Zografos, L., Schorderet, D.F. Invest. Ophthalmol. Vis. Sci. (1999) [Pubmed]
  3. Characterization of HKE2: an ancient antigen encoded in the major histocompatibility complex. Ostrov, D.A., Barnes, C.L., Smith, L.E., Binns, S., Brusko, T.M., Brown, A.C., Quint, P.S., Litherland, S.A., Roopenian, D.C., Iczkowski, K.A. Tissue Antigens (2007) [Pubmed]
  4. Immunohistology of kerato-epithelin in corneal stromal dystrophies associated with R124 mutations of the BIGH3 gene. Konishi, M., Yamada, M., Nakamura, Y., Mashima, Y. Curr. Eye Res. (2000) [Pubmed]
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