Genetics: Inheritance, Sex Linkage, Linkage Mapping, Population Genetics and Disorders

This chapter answers Section XL4.3 of XL4 Zoology, one of the five optional sections of the Life Sciences (XL) paper: “Basic Principles of inheritance, molecular basis of heredity, sex determination and sex-linked characteristics, cytoplasmic inheritance, linkage, recombination and mapping of genes in eukaryotes, population genetics, genetic disorders, roles of model organisms in understanding genetic principles.” It teaches Mendel's laws and gene interaction, the experiments that identified the genetic material, sex determination and dosage compensation, sex-linked and extranuclear inheritance, linkage and three-point mapping, Hardy–Weinberg genetics, the main human disorders with pedigree analysis, and what each model organism revealed. The numericals are cross ratios, map distances and the coefficient of coincidence, Hardy–Weinberg frequencies, and pedigree and sex-linked probabilities.

1. Mendel's principles and gene interaction

Mendel's pea crosses (published 1866) gave two principles. Segregation: the two alleles of a gene pass into different gametes, because homologues part at anaphase I, so a monohybrid F2 shows 3 : 1 phenotypes and 1 : 2 : 1 genotypes. Independent assortment: genes on different chromosomes assort independently, giving 9 : 3 : 3 : 1 in a dihybrid F2. A test cross to the recessive homozygote shows the gametes directly (1 : 1 or 1 : 1 : 1 : 1). With n independent heterozygous genes there are 2ⁿ gamete types, 3ⁿ genotypes and, with complete dominance, 2ⁿ phenotypes, and probabilities multiply across genes. In AaBbCcDd × AaBbCcDd, the fraction dominant for all four traits is (3/4)⁴ = 81/256 and the fraction aabbccdd is (1/4)⁴ = 1/256.

Departures from simple dominance and their ratios
InteractionF2 ratioExample
Incomplete dominance1 : 2 : 1Andalusian fowl: black × splashed white gives blue F1
Codominance, multiple alleles1 : 2 : 1Human ABO (Iᴬ, Iᴮ, i; IᴬIᴮ is group AB) and MN blood groups
Recessive lethal2 : 1Yellow coat in mice: AʸAʸ embryos die
Two genes, novel phenotypes9 : 3 : 3 : 1Fowl comb: walnut (R_P_), rose, pea, single (rrpp)
Recessive epistasis9 : 3 : 4Mouse coat: cc albino whatever the agouti locus; Labrador ee yellow whatever the B locus
Dominant epistasis12 : 3 : 1A dominant allele at one locus masks the other (summer squash colour)
Dominant suppression13 : 3White Leghorn × White Wyandotte fowl: inhibitor allele I suppresses colour
Complementary genes9 : 7Dominant alleles needed at both loci; two deaf parents with defects in different genes can have hearing children
Duplicate genes15 : 1A dominant allele at either locus gives the phenotype

Penetrance is the percentage of individuals with a genotype that show the phenotype; expressivity is the degree of expression among those that do. Pleiotropy is one gene affecting several traits (the sickle-cell allele; Marfan syndrome). In polygenic inheritance with n unlinked, additive gene pairs, the F2 has 2n + 1 phenotypic classes and each extreme class has frequency (1/4)ⁿ.

2. Molecular basis of heredity

  • Griffith (1928): heat-killed virulent (smooth, capsulated) pneumococci mixed with live non-virulent (rough) cells killed mice, and live smooth cells were recovered: a “transforming principle” passed between cells.
  • Avery, MacLeod and McCarty (1944): transforming activity of the purified extract survived protease and RNase but was destroyed by DNase, so the principle is DNA.
  • Hershey and Chase (1952): T2 phage labelled with ³²P (DNA) or ³⁵S (protein) infected E. coli; after blending, ³²P was inside the cells and reached progeny phage, while ³⁵S stayed in the empty coats.
  • Fraenkel-Conrat and Singer (1957): tobacco mosaic virus rebuilt from the RNA of one strain and the protein of another gave progeny of the RNA-donor type, so RNA is the genetic material of some viruses.
  • Meselson and Stahl (1958): E. coli grown in ¹⁵N and moved to ¹⁴N gave, in CsCl density gradients, only hybrid DNA after one generation and equal hybrid and light DNA after two, as semiconservative replication predicts. After n generations the hybrid fraction is 2/2ⁿ.

Chargaff's rules: in double-stranded DNA A = T and G = C, so purines equal pyrimidines, while (A + T)/(G + C) varies between species; a duplex with 22% G has 22% C and 28% each of A and T. Beadle and Tatum (1941), using nutritional mutants of Neurospora, linked one gene to one enzyme, now refined to one gene–one polypeptide.

3. Sex determination, dosage compensation and sex linkage

Sex-determining systems
SystemHeterogametic sexExamples
XX–XYMale (XY)Mammals; Drosophila
XX–XOMale (XO)Grasshoppers and many other insects
ZZ–ZWFemale (ZW)Birds; butterflies and moths
HaplodiploidyNone; males are haploid, from unfertilised eggsBees, ants, wasps
EnvironmentalNoneIncubation temperature in crocodilians and many turtles; Bonellia larvae become males on settling on a female

In mammals the Y is decisive: SRY on the Y starts testis development, so XXY persons are male (Klinefelter) and XO persons female (Turner). In Drosophila the ratio of X chromosomes to autosome sets (X : A) decides: 1.0 gives a female, 0.5 a male, intermediate values intersexes. Hence XXY flies are fertile females and XO flies sterile males; the fly Y carries only male-fertility genes. Dosage compensation equalises X-linked products. Mammalian females inactivate one X at random early in development, and it condenses into a Barr body (Barr bodies = number of X chromosomes − 1); the choice is clonally inherited, so heterozygous females are mosaics, as in tortoiseshell cats. Drosophila males double transcription from their single X, and C. elegans hermaphrodites halve it from each of their two.

X-linked recessive traits (haemophilia A, Duchenne muscular dystrophy, red–green colour blindness) show criss-cross inheritance: an affected father passes the allele to all daughters (carriers) and no sons; a carrier mother gives it to half her sons (affected) and half her daughters (carriers); affected males far outnumber females, and there is no male-to-male transmission. Morgan's white-eyed Drosophila (1910), whose reciprocal crosses differed, first tied a gene to a particular chromosome. X-linked dominant traits pass from an affected father to all daughters and no sons; Y-linked traits pass from father to every son. Two look-alikes are autosomal: sex-limited traits appear in one sex only (milk yield in cattle), and sex-influenced traits are dominant in one sex and recessive in the other (horns in some sheep breeds).

4. Cytoplasmic inheritance and maternal effect

Genes in mitochondria (and plastids in plants) or in cytoplasmic symbionts show cytoplasmic inheritance: reciprocal crosses differ, transmission is maternal because the egg supplies almost all the cytoplasm (sperm mitochondria are eliminated after fertilisation in mammals), there are no Mendelian F2 ratios, the genes map to no chromosome, and the trait persists when the nucleus is replaced by repeated backcrossing.

  • Human mitochondrial disorders (Leber hereditary optic neuropathy, MERRF, MELAS) pass from an affected mother to sons and daughters and never from an affected father. Cells often hold mixtures of normal and mutant mtDNA (heteroplasmy), and symptoms appear when the mutant share in a tissue crosses a threshold, so affected siblings differ in severity.
  • Killer *Paramecium*: killer strains harbour bacterial endosymbionts (kappa particles) that are kept only in cells carrying the dominant nuclear allele K.
  • CO₂ sensitivity in *Drosophila* is caused by the sigma virus passed through the egg cytoplasm; *Wolbachia* bacteria, also egg-borne, cause cytoplasmic incompatibility in many insects.

A maternal effect is different: the offspring's phenotype is set by the mother's nuclear genotype, through products she places in the egg. In the snail Lymnaea peregra, dextral coiling (D) is dominant to sinistral (d). A dd female × DD male gives F1 (Dd) that are all sinistral, because their mother was dd. Selfed F1 give an F2 that is all dextral, because the F1 mothers are Dd, although its genotypes are 1 DD : 2 Dd : 1 dd. The 3 : 1 appears only in the F3, among the broods of F2 snails: the dd quarter produce sinistral broods.

⚠️ Exam trap
Three patterns give different results in reciprocal crosses. Sex linkage shows sex-specific ratios; cytoplasmic traits follow the mother in every generation and never segregate in Mendelian ratios; a maternal-effect gene segregates 3 : 1 one generation late.

5. Linkage, recombination and mapping

Genes on one chromosome are linked and tend to travel together. They are separated by crossing over between non-sister chromatids at the four-strand stage of prophase I, seen as chiasmata; in 1931 Creighton and McClintock (maize) and Stern (Drosophila) showed that genetic recombination goes with physical exchange between chromosomes. Recombination frequency (RF) = recombinants/total × 100, and Sturtevant (1913) used it as distance: 1% RF = 1 map unit = 1 centimorgan (cM). RF cannot exceed 50%, because a crossover involves only two of the four chromatids; genes 50 cM or more apart look unlinked. Double crossovers restore parental combinations, so long distances are underestimated and maps are built by adding short intervals. Linkage is complete in the heterogametic sex of some species: Drosophila males and Bombyx mori (silkworm) females do not cross over.

Three-point test cross. (1) The commonest reciprocal pair of classes are the parentals and the rarest pair the double crossovers (DCO). (2) The gene that differs between the parental and DCO classes is in the middle. (3) Each interval distance = (single crossovers in that interval + DCO)/total. (4) Expected DCO = RF₁ × RF₂ × total; coefficient of coincidence (CoC) = observed/expected DCO; interference I = 1 − CoC. Positive interference means one crossover lowers the chance of another nearby. Worked example, 1,000 test-cross progeny of a female heterozygous for x, y and z:

Three-point test cross (classes listed as alleles at x, y, z)
ClassesNumbersType
x y + and + + z357 + 353Parental
x + z and + y +96 + 94Single crossover, x–y
x y z and + + +46 + 44Single crossover, y–z
x + + and + y z6 + 4Double crossover

Parental x y + against DCO x + + shows that y has switched, so the order is x – y – z. x–y = (190 + 10)/1000 = 20 cM; y–z = (90 + 10)/1000 = 10 cM; x–z = 30 cM. Expected DCO = 0.20 × 0.10 × 1000 = 20 against 10 observed, so CoC = 0.5 and I = 0.5. Other tools: in ordered Neurospora tetrads, gene–centromere distance = ½ × % second-division segregation asci; in human pedigrees, a LOD score of 3 or more (odds 1000 : 1) is accepted as linkage; physical mapping uses somatic-cell hybrids, FISH and DNA markers (RFLPs, microsatellites, SNPs).

6. Population genetics

In a large, randomly mating population without mutation, migration or selection, allele frequencies p and q (p + q = 1) stay constant and genotype frequencies reach p², 2pq and q² after one generation of random mating: the Hardy–Weinberg principle. Its chief use is estimating hidden carriers. If an autosomal recessive disease affects 1 in 10,000, q = 0.01 and carriers are 2pq = 2 × 0.99 × 0.01 ≈ 0.02, about 1 in 50, two hundred times the number of patients. For an X-linked gene, affected males occur at frequency q and affected females at q²: if 8% of males are colour-blind, about 0.64% of females are. For three alleles the genotype frequencies are the terms of (p + q + r)². Fit is tested by χ² with degrees of freedom = genotype classes − number of alleles (1 for two alleles).

Frequencies change by mutation (slowly), migration (Δq = m(q_m − q) for a migrant fraction m with frequency q_m), genetic drift (random change, strongest in small populations; a new neutral allele is fixed with probability 1/2N) and selection (treated in the Evolution chapter). Drift explains the founder effect, such as the high frequency of Ellis–van Creveld syndrome among the Old Order Amish, and bottlenecks, such as the low genetic variation of the northern elephant seal. Inbreeding raises homozygosity without changing allele frequencies: with inbreeding coefficient F, the frequency of aa is q² + Fpq. Random mating restores Hardy–Weinberg proportions but does not alter p or q.

7. Genetic disorders and pedigree analysis

Representative single-gene disorders
DisorderInheritanceMolecular defect
Sickle-cell anaemiaAutosomal recessiveβ-globin Glu6Val; HbS polymerises when deoxygenated
Cystic fibrosisAutosomal recessiveCFTR chloride channel; ΔF508 is the most frequent mutant allele
PhenylketonuriaAutosomal recessivePhenylalanine hydroxylase deficiency; managed by a low-phenylalanine diet
Huntington diseaseAutosomal dominantExpanded CAG repeat in HTT; late onset, with anticipation
AchondroplasiaAutosomal dominantGain-of-function FGFR3; most cases are new mutations
Haemophilia A; Duchenne muscular dystrophyX-linked recessiveFactor VIII; dystrophin
Fragile X syndromeX-linkedExpanded CGG repeat in FMR1

Chromosomal disorders arise mostly from nondisjunction. Down syndrome (47,+21) is usually a free trisomy from a maternal meiotic error, with risk rising with maternal age; a minority carry a Robertsonian translocation (often 14;21), and a phenotypically normal carrier parent with 45 chromosomes has a high recurrence risk. Others: Edwards (trisomy 18), Patau (trisomy 13), Turner (45,X), Klinefelter (47,XXY), cri-du-chat (deletion of 5p) and the Philadelphia chromosome t(9;22) of chronic myeloid leukaemia, which makes the BCR–ABL fusion. With genomic imprinting parental origin matters: loss of 15q11–q13 from the paternal chromosome causes Prader–Willi syndrome, and from the maternal chromosome Angelman syndrome.

Pedigree rules. Autosomal recessive: unaffected parents have affected children (risk 1/4), often with consanguinity, both sexes alike. Autosomal dominant: each affected person has an affected parent (barring new mutation), with risk 1/2 to each child of a heterozygote. X-linked recessive: mostly males, through carrier mothers, never father to son. Mitochondrial: through mothers only. Worked example: an unaffected man whose sister has cystic fibrosis (parents unaffected) is a carrier with probability 2/3, not 1/2, because among unaffected children of two carriers AA : Aa = 1 : 2. If his unaffected wife also has an affected sibling, the risk to their first child is 2/3 × 2/3 × 1/4 = 1/9.

8. Model organisms and the principles they revealed

A good genetic model breeds fast, gives many progeny and allows controlled crosses and mutant screens
OrganismUseful featuresPrinciples established
Garden pea (Pisum sativum)True-breeding varieties with contrasting traits; controlled pollinationSegregation and independent assortment (Mendel)
Drosophila melanogasterAbout 10-day generation at 25 °C; four chromosome pairs; polytene chromosomes; balancersSex linkage (Morgan), first linkage map (Sturtevant), nondisjunction (Bridges), X-ray mutagenesis (Muller), homeotic genes
Neurospora crassaHaploid; ordered tetrads; grows on minimal mediumOne gene–one enzyme (Beadle and Tatum); gene–centromere mapping
Saccharomyces cerevisiaeStable haploid and diploid phases; tetrads; easy gene replacementGenes controlling the cell cycle; mating-type switching
Caenorhabditis elegansSelf-fertilising hermaphrodites plus males; transparent; invariant lineage of 959 somatic cells in the adult hermaphroditeGenes of programmed cell death; RNA interference (Fire and Mello, 1998)
Mus musculusMammal; inbred strains; gene targeting in embryonic stem cellsGenomic imprinting, X inactivation, gene knockouts
Danio rerio (zebrafish)Transparent external embryos; large forward-genetic screensGenes of vertebrate development
E. coli and its phagesGeneration of minutes; haploidDNA as genetic material, the operon, fine-structure mapping (Benzer)

Key takeaways

  • Segregation gives 3 : 1 and independent assortment 9 : 3 : 3 : 1; probabilities multiply across genes. Epistasis turns 9 : 3 : 3 : 1 into 9 : 3 : 4, 12 : 3 : 1, 13 : 3, 9 : 7 or 15 : 1.
  • DNA is the genetic material (Griffith, Avery–MacLeod–McCarty, Hershey–Chase), RNA in some viruses, and replication is semiconservative: all hybrid after one generation, half hybrid after two.
  • SRY makes the Y decisive in mammals, the X : A ratio decides in Drosophila, and birds have ZW females; Barr bodies = X − 1. X-linked recessive alleles pass from affected fathers to carrier daughters, never to sons.
  • Cytoplasmic traits follow the mother and never segregate; a maternal effect (Lymnaea coiling) segregates 3 : 1 one generation late.
  • Map distance = % recombinants, never above 50%; in a three-point cross the DCO class marks the middle gene, CoC = observed/expected DCO and I = 1 − CoC; Drosophila males and silkworm females do not cross over.
  • Hardy–Weinberg: p², 2pq, q²; rare-recessive carriers ≈ 2q; X-linked affected males = q; an unaffected sib of an affected child is a carrier with probability 2/3.

Practice questions (18)

Attempt each one before opening the answer. Every explanation names the tempting wrong option as well as the right one, because that is where marks are lost.

  1. In Labrador retrievers, allele B (black pigment) is dominant to b (brown, called chocolate). At a second, unlinked locus, genotype ee prevents pigment deposition in the coat, so ee dogs are yellow whatever their B genotype, while E_ dogs are black or chocolate as set by the B locus. A BbEe dog is mated with a Bbee dog. Among 48 puppies, the expected number of chocolate puppies is ____.

    Numerical answer — type the value.

    Show answer

    Answer: 6

    Chocolate needs bb together with at least one E. From Bb × Bb, P(bb) = 1/4; from Ee × ee, P(Ee) = 1/2. P(chocolate) = 1/4 × 1/2 = 1/8, and 48 × 1/8 = 6. Yellow (ee) puppies make up half the litter (24) and black ones 3/8 (18). Applying the 9 : 3 : 4 ratio, which holds only for BbEe × BbEe, wrongly gives 9.
  2. Two mice heterozygous at two unlinked coat-colour loci are crossed. The progeny are 92 agouti, 29 black and 39 albino. Which kind of gene interaction best explains this result?

    1. Dominant epistasis, which gives a 12 : 3 : 1 ratio
    2. Complementary gene action, which gives a 9 : 7 ratio
    3. Recessive epistasis, which gives a 9 : 3 : 4 ratio
    4. Dominant suppression, which gives a 13 : 3 ratio
    Show answer

    Answer: C — Recessive epistasis, which gives a 9 : 3 : 4 ratio

    With 160 progeny one sixteenth is 10, so 92 : 29 : 39 is close to 90 : 30 : 40, i.e. 9 : 3 : 4. This is recessive epistasis: cc mice are albino whatever their agouti-locus genotype, so the A_cc (3/16) and aacc (1/16) classes merge into 4/16 albino. A 12 : 3 : 1 ratio needs a dominant masking allele, and 9 : 7 and 13 : 3 give only two phenotypes, not three.
  3. In the cross AaBbCcDd × AaBbCcDd, with all four genes unlinked, what fraction of the progeny is heterozygous at exactly two of the four loci?

    1. 1/16
    2. 1/4
    3. 81/256
    4. 3/8
    Show answer

    Answer: D — 3/8

    At each locus Aa × Aa gives a heterozygote with probability 1/2 and a homozygote with probability 1/2. There are C(4,2) = 6 ways to choose which two loci are heterozygous, each with probability (1/2)⁴ = 1/16, so the fraction is 6/16 = 3/8. The value 1/16 counts only one particular pair of loci, and 81/256 is the fraction showing all four dominant phenotypes.
  4. Which of the following statements about the classic experiments on the genetic material are correct?

    1. In the Avery–MacLeod–McCarty experiment, treating the transforming extract with DNase abolished transformation
    2. In the Hershey–Chase experiment, most of the ³⁵S label entered the bacteria and appeared in progeny phage
    3. In the Meselson–Stahl experiment, after one generation in ¹⁴N medium all the DNA had hybrid density
    4. Griffith's experiment by itself identified DNA as the transforming principle
    Show answer

    Answer: A — In the Avery–MacLeod–McCarty experiment, treating the transforming extract with DNase abolished transformation; C — In the Meselson–Stahl experiment, after one generation in ¹⁴N medium all the DNA had hybrid density

    DNase, but not protease or RNase, destroyed transforming activity, and one round of semiconservative replication makes every duplex half ¹⁵N and half ¹⁴N. In the Hershey–Chase experiment it was ³²P (DNA), not ³⁵S (protein), that entered the cells and reached the progeny. Griffith showed only that a transforming principle existed; its identity as DNA came from Avery and colleagues in 1944.
  5. Which pair of observations shows that the Y chromosome determines maleness in humans but not in Drosophila?

    1. XXY humans are male, whereas XXY flies are female
    2. XO humans are female, and XO flies are also female
    3. XXY humans are female, whereas XXY flies are male
    4. XO humans are male, whereas XO flies are female
    Show answer

    Answer: A — XXY humans are male, whereas XXY flies are female

    In humans a Y (carrying SRY) makes an XXY individual male despite the two X chromosomes. In Drosophila sex follows the X : A ratio, so an XXY fly (ratio 1.0) is female and an XO fly (ratio 0.5) is male, though sterile. The pairings that make XO flies female or XXY humans female are simply wrong.
  6. Red–green colour blindness is X-linked recessive. A colour-blind man marries a woman with normal vision whose parents both have normal vision but whose brother is colour-blind. The probability that their first child is a colour-blind daughter is ____ (to three decimal places).

    Numerical answer — type the value.

    Show answer

    Answer: 0.125

    The woman's brother got his X from their mother, who must be a carrier, so the woman is a carrier with probability 1/2. A daughter always receives the father's X, which carries the allele, and is colour-blind only if she also receives the mother's mutant X. P = P(woman is a carrier) × P(child is a daughter) × P(mother transmits the allele) = 1/2 × 1/2 × 1/2 = 1/8 = 0.125. Leaving out the 1/2 for the sex of the child gives 0.25.
  7. In the snail Lymnaea peregra, shell coiling is controlled by a maternal-effect gene in which the dextral allele D is dominant to the sinistral allele d. A sinistral snail, used as the female parent, produces only dextral offspring. What is its genotype?

    1. dd, because sinistral coiling is recessive and the snail itself is sinistral
    2. DD, because every one of its offspring shows the dominant dextral coiling
    3. Dd, since its own shell reflects a dd mother and its broods reflect its own genotype
    4. It cannot be determined, because coiling direction is inherited through mitochondria
    Show answer

    Answer: C — Dd, since its own shell reflects a dd mother and its broods reflect its own genotype

    With a maternal effect, a snail's own coiling is set by its mother's genotype and its broods' coiling by its own genotype. Being sinistral, it had a dd mother and so carries at least one d; producing dextral broods, it carries D. Hence it is Dd. Reading its phenotype as its genotype (dd) is the trap, and DD is impossible because it received d from its dd mother. The gene is nuclear, not mitochondrial.
  8. A rare human disorder is caused by a mutation in mitochondrial DNA. Which of the following features are expected in pedigrees of this disorder?

    1. Affected women transmit the disorder to both sons and daughters
    2. Affected men do not transmit the disorder to any of their children
    3. Affected men transmit the disorder to all their daughters but to none of their sons
    4. Among the children of two unaffected carrier parents, about one quarter are affected
    Show answer

    Answer: A — Affected women transmit the disorder to both sons and daughters; B — Affected men do not transmit the disorder to any of their children

    Mitochondria are inherited through the egg, so an affected mother passes mutant mtDNA to children of both sexes (severity varies with heteroplasmy), and an affected father passes it to none. Transmission from affected fathers to all daughters is the X-linked dominant pattern, and a one-quarter risk from two carrier parents is the autosomal recessive pattern.
  9. A female heterozygous for three linked recessive mutations u, v and w is test-crossed to a male homozygous recessive for all three. The 2,000 progeny, classified by the alleles received from the female and listed in the order u, v, w, are: u + w 748; + v + 736; u v + 110; + + w 106; u + + 140; + v w 136; u v w 13; + + + 11. The coefficient of coincidence is ____ (to two decimal places).

    Numerical answer — type the value.

    Show answer

    Answer: 0.67

    Parentals are the commonest pair (u + w, + v +) and the double crossovers the rarest (u v w, + + +). Comparing u + w with u v w shows that v has switched, so v is in the middle: u – v – w. RF(u–v) = (110 + 106 + 24)/2000 = 0.12; RF(v–w) = (140 + 136 + 24)/2000 = 0.15. Expected DCO = 0.12 × 0.15 × 2000 = 36; observed DCO = 24. CoC = 24/36 = 0.67 (interference 0.33). Leaving the DCOs out of the interval distances gives a wrong expected value.
  10. A female heterozygous for three linked recessive mutations r, s and t is test-crossed, giving 1,000 progeny classified by the alleles received from the female (listed in the order r, s, t): r + + 375; + s t 371; r s + 88; + + t 86; r s t 38; + + + 36; r + t 4; + s + 2. Adding the two adjacent intervals, the map distance between the two genes at the ends of the map is ____ cM.

    Numerical answer — type the value.

    Show answer

    Answer: 26

    Parentals: r + + and + s t; DCOs: r + t and + s +. Comparing r + + with r + t shows that t has switched, so t is in the middle: r – t – s. r–t = (38 + 36 + 6)/1000 = 8 cM; t–s = (88 + 86 + 6)/1000 = 18 cM. The end-to-end distance is 8 + 18 = 26 cM. Counting recombinants between r and s directly gives (88 + 86 + 38 + 36)/1000 = 24.8 cM, an underestimate because each double crossover restores the parental r–s combination.
  11. In Drosophila, genes a and b are 20 map units apart as measured in females. A male heterozygous in coupling (AB/ab) is test-crossed to an ab/ab female. Which progeny are expected?

    1. 40% AB, 40% ab, 10% Ab and 10% aB, as from a female of the same genotype
    2. 50% AB and 50% ab, with no recombinant classes
    3. 25% each of AB, ab, Ab and aB, as for unlinked genes
    4. 45% AB, 45% ab, 5% Ab and 5% aB, since recombination is halved in males
    Show answer

    Answer: B — 50% AB and 50% ab, with no recombinant classes

    Drosophila males do not undergo meiotic crossing over, so linkage in males is complete and only the parental gametes AB and ab are formed. The 40 : 40 : 10 : 10 split is what a female would give at 20 cM, which makes it the tempting answer. Halved recombination and independent assortment have no basis here.
  12. An autosomal recessive disorder affects 1 in 3,600 newborns in a population at Hardy–Weinberg equilibrium. Using the exact expression 2pq, the expected number of heterozygous carriers among 3,600 people of this population is ____.

    Numerical answer — type the value.

    Show answer

    Answer: 118

    q² = 1/3600, so q = 1/60 and p = 59/60. Carrier frequency 2pq = 2 × (59/60) × (1/60) = 118/3600, so 118 carriers are expected among 3,600 people, against 1 affected person. The shortcut 2q gives 120, which is only an approximation.
  13. In a population at Hardy–Weinberg equilibrium, 10% of males show an X-linked recessive trait. What fraction of females are expected to be heterozygous carriers?

    1. 0.10
    2. 0.18
    3. 0.01
    4. 0.09
    Show answer

    Answer: B — 0.18

    For an X-linked gene the frequency of affected males equals q, so q = 0.10 and p = 0.90. Carrier females are 2pq = 2 × 0.9 × 0.1 = 0.18, and affected females q² = 0.01. Taking the male frequency, 0.10, as the carrier frequency is the usual slip.
  14. Which of the following processes, acting alone on an autosomal locus, change allele frequencies in a population?

    1. Inbreeding within a large population
    2. Genetic drift in a small population
    3. One generation of random mating in a population not at Hardy–Weinberg proportions
    4. Immigration from a population with a different allele frequency
    Show answer

    Answer: B — Genetic drift in a small population; D — Immigration from a population with a different allele frequency

    Drift changes allele frequencies at random, and gene flow from a population with different frequencies shifts them towards the donor's values. Inbreeding moves alleles from heterozygotes into homozygotes without changing their frequencies, and one round of random mating restores p², 2pq and q² while leaving p and q unchanged.
  15. A phenotypically normal man has a brother with cystic fibrosis (autosomal recessive); their parents are unaffected. He marries an unaffected woman with no family history from a population in which the carrier frequency is 1/25. The probability that their first child has cystic fibrosis is 1/N, where N = ____.

    Numerical answer — type the value.

    Show answer

    Answer: 150

    The man's parents must both be carriers. Among their unaffected children AA : Aa = 1 : 2, so his carrier probability is 2/3. The woman's carrier probability is 1/25. If both are carriers the child is affected with probability 1/4. P = 2/3 × 1/25 × 1/4 = 2/300 = 1/150, so N = 150. Using 1/2 for the man gives 1/200, the common error.
  16. A phenotypically normal woman has had two children with Down syndrome, and her own karyotype shows 45 chromosomes. What is the most likely explanation?

    1. She carries a balanced Robertsonian translocation, such as one joining chromosomes 14 and 21
    2. She has Turner syndrome, which raises the rate of nondisjunction of chromosome 21
    3. She is mosaic for trisomy 21, which appears as a missing chromosome in her karyotype
    4. Her eggs undergo nondisjunction because of advanced maternal age at each conception
    Show answer

    Answer: A — She carries a balanced Robertsonian translocation, such as one joining chromosomes 14 and 21

    A balanced Robertsonian carrier has 45 chromosomes because two acrocentric chromosomes (often 14 and 21) are fused, yet has a normal amount of genetic material and is unaffected; her gametes can carry the fused chromosome plus a free 21, giving translocation Down syndrome with a high recurrence risk. Maternal age is the tempting answer, but it explains free trisomy and not a 45-chromosome mother. Turner syndrome (45,X) has its own phenotype, and trisomy mosaicism adds a chromosome rather than removing one.
  17. A child carries a deletion of the region 15q11–q13 on the chromosome 15 inherited from the father. Which condition is expected?

    1. Angelman syndrome
    2. Prader–Willi syndrome
    3. Cri-du-chat syndrome
    4. Fragile X syndrome
    Show answer

    Answer: B — Prader–Willi syndrome

    Because of genomic imprinting, several genes in 15q11–q13 are expressed from only one parental copy; loss of the paternal region gives Prader–Willi syndrome. Angelman syndrome is the tempting answer but results from loss of the maternal copy, which carries the maternally expressed UBE3A. Cri-du-chat is a 5p deletion, and fragile X is a CGG expansion in FMR1.
  18. Which pairing of model organism and the genetic principle first demonstrated with it is correct?

    1. Neurospora crassa — one gene–one enzyme, from mutants unable to grow on minimal medium
    2. Caenorhabditis elegans — the first linkage map, from three-point crosses of eye-colour mutants
    3. Drosophila melanogaster — DNA as the genetic material, from transformation of its embryos
    4. Saccharomyces cerevisiae — semiconservative replication, from density labelling of its DNA with ¹⁵N
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    Answer: A — Neurospora crassa — one gene–one enzyme, from mutants unable to grow on minimal medium

    Beadle and Tatum used Neurospora mutants that needed a supplement to grow on minimal medium to tie single genes to single enzymes. The first linkage map came from Drosophila (Sturtevant), DNA as the genetic material from bacteria and phage, and semiconservative replication from E. coli (Meselson and Stahl).