The Lesch-Nyhan syndrome is a serious metabolic disorder affecting about one in 50,000 males in the population oF the United States. A class of molecules called purines, which are biochemical precursors of DNA, accumulate in the nervous tissues and joints of people with the Lesch-Nyhan syndrome. This biochemical abnormality is caused by a deficiency for the enzyme hypoxanthine phosphoribosyltransferase (HPRT), which is encoded by a gene located on the X chromosome. Individuals deficient for this enzyme are unable to control their movements and unwillingly engage in self-destructive behaviour such as biting and scratching themselves, The males labeled IV-5 and IV6 in the following pedigree have the Lesch-Nyhan syndrome. What are the risks that V-1 and VI-2 will inherit this disorder? In about 4 in 200 pregnancies a sperm fertilizes a polar body instead of an oocyte. Why cant a polar body support the development of an entire embyro? We know that Ill-3 must be a heterozygous carrier of the mutant allele (b) because two of her sons are affected, However, because she herself does not show the mutant phenotype, we know that her other X chromosome must carry the wild-type allele (H). Given that Ill3 is genotypical Hb, there is one-half chance that she passed the mutant allele to her daughter (WV-2). If she did, there is a one-half chance that IV-2 will transmit this allele to her child (V-1), and there is a one-half chance that this child will be a male, Thus, the risk that V-1 will have the Lesch-Nyhan syndrome is, (1/2) x (1/2) x (1/2) = 1/8. For V-2, the risk of Inheriting the Lesch-Nyhan syndrome is essentially zero. This child's father (IV-3) does not have the mutant allele, and even if he did, he would not transmit it to a son, The child's mother comes from outside the Family and is very unlikely to be a carrier because the trait is rare in the general population. Thus, V-2 has virtually no chance of suffering from the Lesch-Nyhan syndrome A polar body does not have enough cytoplasm and organelles to support an embryo