Cockayne

B. Cockayne, ed.. Modem Oxide Materials, Prep., Prop. Device Applications, Academic Press, London, 1972. 

The genus Fuchsia consists of perhaps 100 species, enjoys a wide distribntion, and offers a variety of systematic and evolutionary challenges. In addition to attracting purely botanical interest. Fuchsia is also of considerable horticnltnral value. Our interest in the genns concerns sect. Skinnera which consists of three New Zealand species, F. excorticata L. f., F. perscandens Cockayne Allan, and F. procumbens R. Cnnn., and the Tahitian endemic F. cyrtandroides Moore. A fourth taxon, F. x colensoi Hook. f. occurs wherever the ranges of F. excorticata and F. perscandens overlap. 

McDowell P,Affas Z, Reynolds C, Holden MTG, Wood SJ, Saint S, Cockayne A, Hill PJ, Dodd CER, Bycroft BW, Chan WC, Williams P (2001) Mol Microbiol 41 503... 

Scott RJ, Lian L-Y, Muharram SH, Cockayne A, Wood SJ, Bycroft BW, Williams P, Chan WC (2003) Bioorganic Med Chem Lett 13 2449... 

Theron T, Fousteri MI, Volker M, Harries LW, Botta E, Stefanini M, Fujimoto M, Andressoo JO, Mitchell J, Jaspers NG, McDaniel LD, Mullenders LH, Lehmann AR (2005) Transcription-associated breaks in xeroderma pigmentosum group D cells from patients with combined features of xeroderma pigmentosum and Cockayne syndrome. Mol Cell Biol 25(18) 8368-8378 Thiriet C, Hayes JJ (2005) Chromatin in need of a fix phosphorylation of H2AX connects chromatin to DNA repair. Mol Cell 18(6) 617-622... 

CSB/ERCC6 CSB Human Associates with a subset of Pol II complexes Mutation causes Cockayne syndrome, a defect in transcription coupled nucleotide excision repair [321,322]. Human Rad26p homolog... 

When Pol II transcription halts at the site of a DNA lesion, TFIIH can interact with the lesion and recruit the entire nucleotide-excision repair complex. Genetic loss of certain TFIIH subunits can produce human diseases. Some examples are xeroderma pigmentosum (see Box 25-1) and Cockayne s syndrome, which is characterized by arrested growth, photosensitivity, and neurological disorders. ... 

While defects in protein XPD often cause typical XP symptoms, some defects in the same protein lead to trichothiodystrophy (TTD, brittle hair disease). The hair is sulfur deficient, and scaly skin (ichthyosis, Box 8-F), mental retardation, and other symptoms are observed.0 Like their yeast counterparts (proteins RAD3 and RAD25), XPB and XPD are both DNA helicases.0 They also constitute distinct subunits of the human transcription factor TFIIHP, which is discussed in Chapter 28. It seems likely that XPD is involved in transcription-coupled repair (TCR) of DNA.° °i-s This is a subpathway of the nucleotide excision repair (NER) pathway, which allows for rapid repair of the transcribed strand of DNA. This is important in tissues such as skin, where the global NER process may be too slow to keep up with the need for rapid protein synthesis. Transcription-coupled repair also appears to depend upon proteins CSA and CSB, defects which may result in the rare cockayne syndrome.13 0 4 11 Patients are not only photosensitive but have severe mental and physical retardation including skeletal defects and a wizened appearance. 

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