Alright, it’s high time I review this hermaphroditic little masterpiece.
Being a pseudo-biochemist (pseudo in the sense that I only pretend
to be a biochemist, whereas in reality I write scientific development reports and other documents that no one will ever read but which I’ve convinced myself are just as fulfilling as doing real science), I find the premise of this novel to be incredibly interesting.
5α-Reductase deficiency is an autosomal recessive disorder; autosomal meaning that the gene coding for 5α-Reductase is not
located on a sex chromosome (X or Y), and recessive meaning that one would need two
copies of a mutated form of the gene in order to express the disease trait. Since we as a biological species inherit one copy of every gene from each of our parents, it would not be enough to have only one mutated form of this gene because a single “good” copy is all that’s required for proper function. Because of this, the proper-functioning gene is considered to be completely dominant
over the mutated form in terms of phenotypic expression.
Here is a Punnett square showing basic concepts of Mendelian genetics:
Each form of the gene is called an allele: “B” represents the dominant allele, or the healthy gene form; “b” represents the recessive allele.
If both parents are phenotypically “normal,” the only way they would be able to have any offspring with this disease is if they were both carriers, meaning they each have one dominant and one recessive allele. In this way, they are said to be heterozygous for this trait, the genotype of which is represented as “Bb.” For any child they conceive, there would exist a 25% chance of that child inheriting two recessive alleles. This is referred to as being homozygous recessive, the genotype of which is represented as “bb.” Only homozygous recessive children will express the disease.
Since the protagonist of this novel has unluckily inherited both recessive alleles, one from each of his parents, he ends up with the disorder. So what is
this disorder, exactly? The 5α-Reductase gene codes for an enzyme which converts testosterone into a potent sex steroid called dihydrotestosterone
, or DHT, which plays a fundamental role in the formation of the male sex organs. Since disease subjects do not have the ability to convert testosterone into DHT, they end up with too much testosterone and not enough DHT, which in some cases leads to the formation of ambiguous genitalia.
These ambiguous genitalia form one of the many, but probably the most interesting, subjects of the novel. The author begins by tracing the history of these recessive alleles back through the family lineage before elegantly leading us to the budding of the protagonist’s crocus: his ambiguous little penis stub
(yes, you should click there; and yes, you should see that movie). Perhaps not surprisingly, the historical tracing reveals some ancestral inbreeding, as well. And since the protagonist is still genotypically male (even though he doesn’t know it and neither do his parents or anybody else), the real fun begins when he enters puberty.
When I met with my book club to talk about this fantastic novel, a few pronoun choices were used for describing the protagonist: he, she, he-she-it, etc. But all joking aside, the protagonist is male. He is male by genotypic definition (he has two healthy sex chromosomes, one of which is a Y), and he sexually identifies himself as male which is consistent with other real-life sufferers of 5α-Reductase deficiency.