Mendelian Genetics, Chapter 14

 

 

•      Mendel’s work

–  The Law of Segregation

–  The Law of Independent Assortment

•      Rules of probability

•      Extensions of Mendelian Genetics

•      Mendelian Inheritance in Humans

 

 

Heritable Variation & Patterns of Inheritance

•     Until the 20th century, most biologist thought that offspring were a blend of both parents (blending hypothesis).

•     Gregor Mendel was the first to analyze patterns of inheritance in a systematic, scientific way

Heritable Variation & Patterns of Inheritance

•     Breeding garden peas during the 1860’s his experimental and mathematically rigorous research is a classic in the history of biology

 


Mendel’s ‘Particulate’ Model of Inheritance

•      Parents pass on to their offspring discrete factors that are responsible for inherited traits Mendel’s Principles

•      The discrete factors (genes) retain their individuality across generations.

•      An organisms collection of genes is more like a bucket of marbles than a pail of paint.

 

 

Predictions of the blending model:

•      Over many generations, a freely mating population would give rise to a uniform population of individuals.

–  Think pail of paint: blue plus yellow would make green, so all subsequent individuals would be green.

•      Everyday observations and breeding experiments do not support this hypothesis.

 

 

Predictions of the particulate model:

•      Over many generations, variation would be maintained.

•      Traits could skip a generation.

•      Everyday observations and breeding experiments support this hypothesis.

 

Table 14.1

 

Fig. 14.2

Mendel used different true-breeding varieties to study what would happen when they were crossed with each other.

He removed the stamen from one parent so that it would not self fertilize and then brushed on pollen from a second parent.

 

•      Offspring of 2 different true-breeding varieties are hybrids

•      Cross-fertilization is called hybridization or a cross

•      Parental plants are called P generation (parental)

•      Their hybrid offspring are the F1 generation (filial)

•      When F1 plants self-fertilize or are crossed their offspring are called F2 generation

 

 

In his scientific study Mendel chose seven pea characteristics that displayed two distinct forms

In his scientific study Mendel chose seven pea characteristics that displayed two distinct forms

 

 

Fig. 14.3

 

 

Mendel’s Monohybrid Cross

•      Mate purebreds

–    Mendel found that F1 plants (monohybrids) were all purple, not a blend of purple & white

–    So was the heritable factor in the white flowers lost due to hybridization?

 

•      Mate F1s to each other

•      Examine offspring (F2 - ‘grandkids’)

 

•      Always found approximately 3:1 ratio of both original phenotypes in F2

•      Ratio calculations:

–    705/929 = 76% = 3/4

–    224/929 = 24% = 1/4

 

 

Figure 14.5  Mendel’s law of segregation

 

 

Mendel concluded that:

The heritable factor for white flowers did not disappear in F1 plants

 

Mendel developed four hypotheses:

•      There are alternate forms of genes (alleles)

•      For each inherited characteristic, an organism has two genes, one from each parent. These genes may be the same alleles or different alleles

•      A sperm or an egg only carries one allele for each inherited characteristic

•      When the alleles of a gene pair are different & one is fully expressed and the other no effect, the alleles are called Dominant and Recessive respectively

 

 

Principle Of Segregation

•    This led Mendel to develop his Principle of Segregation

–   Pairs of alleles segregate (separate) during gamete formation; the fusion of gametes at fertilization creates allele pairs again

 

•    This Principle of Segregation applies to all sexually reproducing organisms

 

 

Some Definitions

•      A genes is a segment of DNA that codes for a particular trait.

–   It gives instructions for the manufacture of a protein or RNA molecule.

•      A locus is the physical location of a gene on the chromosome.

•      A character is heritable feature that varies between individuals.

–   i.e. flower color

•      Each variant for a character is called a trait.

–   i.e. purple or white flowers.

 

 

Figure 14.4  Alleles, alternative versions of a gene

 

 

Phenotype versus Genotype

An organism’s:

•     Collection of expressed, or physical, traits, is its Phenotype

•     Genetic make up is it’s Genotype

•     For the F2 plants:

–   The phenotypic ratio is 3:1

–   The genotypic ratio is 1:2:1

•     Mendel found that one parent trait disappeared in the F1 generation only to reappear in a quarter of the F2 generation

 

Figure 14.6  Genotype versus phenotype

 

 

Genetics, Alleles & Homologous Chromosomes

•     Every diploid individual has two sets of homologous chromosomes: one from each parent

•     Alleles of a gene reside at the same locus on homologous chromosomes (Fig 14.3)

•     Homologous chromosomes may have either the same alleles or different ones at a given locus

•     For any gene:

–   When the alleles are identical, it is homozygous

–   When the alleles are different, it is heterozygous

 

 

Other Monohybrid Crosses

•     Know that brown eyes are dominant to blue eyes

 

•     Can start with any type of parent and predict kids

–   Predict offspring if heterozygous brown-eyed parent and blue eyed parent.

–   Predict offspring of two blue eyed parents.

 

•     Start with kids and figure out what parents must have been

–   Two brown-eyed parents have 2 kids with brown eyes and one with blue; determine all genotypes or genotype possibilities.

–   Mum has blue eyes, kid has brown eyes; what must be true for dad?

 


Using a Test Cross to Determine an Unknown Genotype

•    A test cross is a mating between an individual of unknown genotype and a homozygous recessive

 

•    The phenotype (appearance) of the offspring reveals the genotype

 

•    Mendel used test crosses to determine whether he had true-breeding varieties of plants

 

 

Figure 14.7  A testcross

 

 

Test Cross

•     Used ONLY when have dominant phenotype & don’t know genotype

•     Cross with recessive phenotype (since know genotype)

•     3 offspring

 

 

 

Mendel's Principle of Independent Assortment

 

•           What would result from a mating of parental varieties differing in two characteristics – a dihybrid cross?

•           Mendel crossed homozygous plants having round yellow seeds (genotype RRYY) with plants having wrinkled green seeds (rryy), producing:

•           F1:  all yellow and spherical (establishes dominance of both traits)

 

 

Were the two characteristics transmitted as a package or independently of each other?

•            If the genes were inherited together:

–          The F1 hybrids would produce the same two kinds (genotypes) that they received from their parents

–          The F2 generation would show a 3:1 phenotype ratio

•            If the two seed characteristics segregated independently:

–          F1 generation would produce 4 gamete genotypes – RY, Ry, rY, ry in equal quantities

–          Nine different genotypes producing four different phenotypes in a ratio of 9:3:3:1 (two simultaneous monohybrid crosses)

 

 

Figure 14.8  Testing two hypotheses for segregation in a dihybrid cross

 

 

Mendel's Principle of Independent Assortment

•     Mendel tried his seven pea characteristics in various dihybrid combinations and always observed a 9:3:3:1 ratio (or two simultaneous 3:1 ratios) of phenotypes in the F2 generation

 

•     These results supported hypothesis (b) that:

–    each pair of alleles segregates independently of the other pairs during gamete formation

Figure 14.9  Segregation of alleles and fertilization as chance events

 

 

Mendelian inheritance reflects rules of probability.

•      A heterozygote, Pp has a probability of ½ for passing down each allele into a gamete.

•      According to the rule of multiplication, the probability of two Pp parents producing a pp gamete is ½ x ½ = Ό.

•      The rule of multiplication can be applied to dihybrid crosses.  What is the probability of two YyRr parents producing a yyrr offspring? 

–   Ό x Ό = 16.

 

 

The Rule of Addition

•      What is the probability that two heterozygous parents produce a heterozygous offspring?

•      First:

–   Probability of dominant allele from sperm is ½.

–   Probability of a recessive allele from the egg is ½.

–   ½ x ½ = Ό.

•      Second

–   Probability of dominant allele from sperm is ½.

–   Probability of a recessive allele from the egg is ½.

–   ½ x ½ = Ό.

•      Third

–   Both of these events are possible, so the probabilities for each are added:

–   Ό + Ό = ½.

 

 

More on Dominance

•      An allele can be completely dominant, so that the phenotype of a homozygous dominant individual is indistinguishable from that of a heterozygous individual.

 

•      An allele can be incompletely dominant, so that the phenotype of a heterozygous individual is intermediate between than that of a homozygous dominant individual and a homozygous recessive individual.

 

•      An allele can be codominant, in which two allele have affect the phenotype in separate, distinguishable ways and both influence the phenotype equally.  Note – this is not an intermediate phenotype.

 

 

Figure 14.9  Incomplete dominance in snapdragon color

 

 


Multiple Alleles and Codominance

•      The ABO blood group is an example of multiple alleles of a single gene.

•      There are three alleles of this gene, IA, IB or i.

–   The IA allele codes for the ‘A’ red blood cell surface marker.

–   The IB allele codes for the ‘B’ red blood cell surface marker.

–   The i allele does not code for a functional protein.

•      The IA and IB alleles are codominant, because in individuals with the genotype, both A and B surface markers are produced.

•      There are four possible genotypes. A persons blood group may be A, B, AB or O.

 

 

Figure 14.10  Multiple alleles for the ABO blood groups

 

Table 14.2

 

 

Pleiotropy

•      The ability of a gene to affect an organism in may ways is called pleiotropy.

•      This is because there are many intricate molecular mechanisms within a cell, and many genes influence more than one characteristic in an organism.

 

 

Epistasis

•      A gene at one locus influence the phenotypic expression of a gene at another locus.

•      Usually an epistatic gene is one of the first enzymes within a metabolic pathway.  If the first enzyme is non-functional, then the pathway is shut down, even of the following enzymes are functional.

 

 

An example of epistasis

•      In mice, the color locus has a gene that codes for an enzyme at the beginning of a pigment making pathway.

•      There are two alleles, C which allows the coat to be colored, and c which produces a non-functional enzyme.

•      The black/brown locus codes for an enzyme further down in the pigment making pathway.

–    The dominant B allele give a black coat color.

•    A BB or Bb mouse is black.

–    The recessive b allele codes for a version of the enzyme that is less robust.

•    A bb mouse is brown.

•      A cc mouse is white, regardless of the genotype at the brown/black locus.

 

 

Figure 14.11  An example of epistasis

 

 


Polygenic Inheritance

•      Many traits, such as human height or skin color can be classified along a continuum.

•      These are called quantitative characters.

•      This continuous inheritance usually indicated polygenic inheritance, and additive effect of two or more genes.

•      Skin color is determined by at least three genes which show incomplete dominance.

•      Height is determine by many more genes.

•      Intelligence, another continuous trait is determined by many many more genes.

 

 

Figure 14.12  A simplified model for polygenic inheritance of skin color

 

 

Multifactorial Characters

•      Phenotype can depend on the environment as well as on genes.

–  Nutrition, sun tanning of skin, stimulating environment promotes intelligence.

•      So a genotype gives a potential range of phenotypes, called a norm of reaction.

–  Can be very narrow, ex. ABO blood group.

–  Can be very broad, ex. Count of red and white blood cells.

•   Altitude, physical activity, presence of infection

 

 

Figure 14.13  The effect of environment of phenotype

 

 

Family Pedigrees

•    In genetics dominance does not mean a phenotype is normal or more common than a recessive phenotype

–   Dominance means that a heterozygote displays the dominant phenotype

–   Recessive traits are often more common in a population than dominant ones. (freckles)

•    We know how particular human traits are inherited by collecting information on a family history and creating a family tree or pedigree

 

 

Figure 14.14  Pedigree analysis

 

 

 

Human Disorders controlled by a Single Gene

 

•    There are over a thousand known human genetic disorders that can be inherited as dominant or recessive traits controlled by a single gene locus

 

 


Recessive Disorders

•     Most human genetic disorders are recessive

•     Using Mendel’s principles we can predict the fraction of affected offspring likely to result from a marriage of two carriers

•     Pedigree analyses & prediction applies to any genetic trait controlled by a single gene locus

 

 

A family with an inherited form of deafness

Deafness did not appear in the first generation

Only 2 of the 7 children in the 3rd generation had the condition

 

 

Common Recessive Disorders

•      Cystic Fibrosis:

•      Strikes 1/2,500 white of European descent.

•      1/25 (4%) of white carry the disease.

•      The normal allele for the gene codes for a chloride ion channel transporter.

•      Lack of this gene causes chloride ions to build up the extracellular fluid, and the production of a thick mucus.

–   Blocks respiratory passages (poor breathing and frequent infections), ducts from pancreas to the small intestine (poor digestion), seminal tubes (male sterility)

 

 

Common Recessive Disorders

•      Tay-Sachs Disease:

•      Strikes 1/3,600 Ashkenazic Jews.

•      The normal allele for the gene codes for an enzyme that breaks down lipids in the brain.

•      Lack of this gene causes degeneration of the brain and nervous system

–   Results in seizures, blindness, poor mental and motor function, death within a few years of birth.

 

 

Common Recessive Disorders

•      Sickle Cell Disease:

•      Strikes 1/400 African Americans.

•      Caused by the substitution of a single amino acid in the hemoglobin gene.

•      Causes red blood cells to sickle when blood oxygen levels fall due to altitude, exercise, etc.

•      Heterozygotes are protected against malaria and some individuals experience some of the symptoms of those homozygous for the sickle cell allele.

 

 

Dominant Disorders

•      Many dominant disorders are non lethal, such as extra fingers and toes, or with webbing

•      An example of a more serious disorder is Achondroplasia, a form of dwarfism, which affects about 1 in 25,000 people

–   Only heterozygotes have the disorder. Homozygotes for the dominant cause death in the embryo

–   Given that 99.99% of the population have the recessive alleles it shows that a dominant allele is not necessarily more plentiful in a population

Dominant Disorders

•     Lethal dominant alleles are a lot less common than lethal recessives

–   Afflicted individuals often die before they reproduce

–   More common lethal recessive mutations are perpetuated across generations by the reproduction of recessive carriers

•     Lethal dominants that do not cause death to late on in development can perpetuate through reproduction

–   Huntingdon’s Disease, a progressive degeneration of the nervous system. Death follows 10-20 years after onset of symptoms

 

 

Figure 14.16  Large families provide excellent case studies of human genetics

 

 

Fetal Testing

•    Many genetic disorders can be detected before birth

 

•    Methods used to gain samples for testing are:

–   Amniocentesis

–   Chorionic villus sampling

–   Fetoscopy

–   Ultrasound imaging

 

 

Chorionic Villus Sampling involves taking a small piece of fetal tissue from the placenta

•      These cells multiply rapidly and give back karyotyping data in a matter of hours

•      Compared to amniocentesis, CVS is faster & can be performed earlier (8th-10th week)

•      CVS is not suitable for tests requiring amniotic fluid and is not as widely available

 

 

Fetal Testing cont./

 

•     Fetoscopy involves a needle-thin tube containing a viewing scope being inserted into the uterus

 

•     The risk of complications, such as maternal bleeding, miscarriage or premature bleeding resulting from these techniques varies:

–   Fetoscopy (10%)

–   Amniocentesis (1%)

–   CVS (2%)

•     These tests are reserved for pregnancy in which the possibility of a genetic disorder may exist

 

 

The End