269 In the collection of the Capodimonte. For the history and iconography of the Gonsalvus family see Aldrovandi (1642); Siebold (1878); Zapperi (1995); Haupt et al. (1990) pp.92–7 and, especially, Hertel (2001).
273 In 1826 John Crawfurd, British diplomat and naturalist. For the history of Shwe-Maong and his family see Crawfurd (1827); Yule (1858) and Bondeson and Miles (1996)M.
276 We are born with about five million hair follicles. For a general review of hair (and feather) specification see Oro and Scott (1998). For the role of BMPs and FGFs, Jung et al. (1998) and Noramly and Morgan (1998). Reynolds et al. (1999) carry out the trans-gender transplantation experiment.
280 The one thing that many of us. Most of the anecdotal material here comes from Segrave (1996) – a delightful social history of balding. Male pattern balding or androgenetic alopecia (109200). See Cotsarelis and Millar (2001) for a general biology of the dying hair follicle, and Kuester and Happle (1984) for a review of the genetics of the androgenetic alopecia.
282 One fact is, however, known: to go bald you need testosterone. See Aristotle Historia animalium in Collected works pp.983–4. Hamilton (1942) recounts the experiments with testosterone. Knussmann et al. (1992) discuss the relationship between testosterone levels, virility and balding.
283 Is there any hope for the bald? Trotter (1928) discusses the relationship between hair growth and shaving. Sato et al. (1999) and Callahan and Oro (2001) discuss the role of sonic hedgehog in rejuvenating hair follicles; Huelsken et al. (2001) discuss (?-catenin.
285 One can still, occasionally. The portraits of the Ambras family were first described in the modern scientific literature by the physiologist C. Th. Siebold (1878). He proposed that they were atavistic, a claim echoed by Brandt (1897), who points out that the Burmese family have the same disorder. Both men recognised that the surplus hair in the two families was lanugo (Siebold explicitly compares Petrus Gonsalvus’s hair to that of a foetal orangutan), but suppose that lanugo is more ‘primitive’ – a conflation between phylogeny and ontogeny that is typical of German workers of the time, who were deeply influenced by Haeckel. Felgenhauer (1969) gives a summary of nineteenth-century views on hairy people. More recently, there has been a great deal of debate about just how many surplus-hair syndromes there are, and who had what (see Garcia-Cruz et al. 2002 for one point of view). I argue that Petrus Gonsalvus’s and Shwe-Maong’s families both have the same condition: hypertrichosis lanuginosa (145700), the mutant gene of which may reside on chromosome 8. The hair of at least one man with this syndrome (a Russian named Adrian Jewtichjew) has been examined microscopically and seems to have been lanugo. The most famous modern pedigree of hairy people, the Gomez family of Mexico, have another, unrelated, disorder: X-linked hypertrichosis terminalis (145701); Figuera et al. (1995). See this paper and Hall (1995), recent – and perhaps reasonable – claims that this latter kind of hairiness is indeed atavistic.
286 Darwin himself knew of the Burmese hairy family. See Darwin (1871; 1981) volume 2. p.378 for his account of sexual selection and hairiness of the Burmese family; see Darwin (1859; 1968) pp.183–4 and Darwin (1882) volume 2, pp.319–21 for the homology between skin organs, the Burmese family and the ‘Hindoos of Scinde’. See Thadani (1935) for a later account of the same pedigree (the ‘Bhudas’) who have a syndrome called ectodermal dysplasia 1, anhydrotic or ED1 (305100) caused by a mutation in ectodysplasin (EDA) (Kere et al. 1996). The Mexican hairless dog’s mutation is still unknown (Schnaas 1974; Goto et al. 1987) but is probably this gene or its receptor, EDAR (224900; 604095) (Headon and Overbeek 1999; Monreal et al. 1999). The scaleless variety of Medaka has a mutation in the EDAR gene (Kondo et al. 2001). For ectodysplasin’s proposed role in establishing hair papillae see Barsh (1999). See Sharpe (2001) on the evolutionary history of the hair follicle.
288 The use of a single molecule in the making. For hens’ teeth see the classic experiments by Kollar and Fisher (1980), a commentary by Gould (1983) pp.177–86, and recent experiments showing that chicken mandibles are BMP4-defective (Chen et al. 2000).
289 Perhaps it is also the retrieval of an ancient signalling system. Nipples, supernumerary or polymastia (163700). For a review see Cockayne (1933) pp.341–5; Japanese polymastia, Iwai (1907). I thank Alan Ashworth and Beatrice Howard for telling me about Scaramanga.
290 Breasts bring us back to Linnaeus. The ancient iconography of Artemis Ephesia is discussed by Fleischer (1984) and Linnaeus’ use of it by Gertz (1948) – for the translation of which I am indebted to Lisbet Rausing. Nosce te ipsum – the slogan that meant so much to Linnaeus is rarely attributed to Solon, but rather (as in Plato) to the seven wise men of Protagorous who wrote it on the temple of Apollo at Delphi. The Oxford dictionary of quotations gives its source as ‘Anonymous’.
CHAPTER IX: THE SOBER LIFE
297 Huntington disease is one of the nastier. Huntington disease, also Huntington’s Chorea or HD (143100), is caused by dominant mutations in the huntingtin gene. Rubinsztein (2002) reviews the molecular basis of the pathology; Bruyn and Went (1986) review the history and spread of the disease.
298 How can so lethal a disorder? See Haldane (1941) pp.192–4.
300 Were it not for ageing’s pervasive effects. Ricklefs and Finch (1995) give estimates of longevity in the absence of ageing.
302 But it was another British scientist. See Medawar (1952) and Williams (1957) for the seminal papers on the evolutionary theory of ageing. Rose (1991) gives an incisive historical review. Albin (1988) discusses the fecundity of women with Huntington’s based on data collected by Reed and Neel (1959).
304 In his declining years, flush with cash and fame. Alexander Graham Bell (1918) analyses the Hyde family; Quance (1977) discusses Bell’s interests in the genetics of longevity.
306 In the 1980s the evolutionary account of ageing. See Rose (1984) for the original experiment; Rose (1991) for a review; and Sgrò and Partridge (1999)for a more detailed analysis of a similar experiment.
308 Since Aristotle. Aristotle On length and shortness of life in Complete Works volume 1 p.743. See Diamond (1982) for the cost of reproduction in marsupial mice and Westendorp and Kirk wood (1998) for the cost of reproduction in British aristocrats. See Leroi (2001) for a sceptical treatment of cost of reproduction data.
309 Is there a recipe for long life? See Cornaro (1550,1903) for a translation of the Vita sobria, and Gruman (1966) for a review of Cornaro’s thought and its influence.
311 The worst of it is that there is an element of truth. See Finch (1990) pp.506–37 for a review of the earlier literature on caloric restriction; ibid. pp.20–1 for mortality rates of the Dutch during the Hongerwinter. See Holliday (1989) and Chapman and Partridge (1996) on reproductive costs and caloric restriction. Several experiments in flies and mice have been done to look at the effects of caloric restriction on ‘whole genome expression profiles’. The best is a study on flies (Pletcher et al. 2002); the mouse studies (Lee et al. 1999) are more difficult to interpret.