PowerPoint Presentation Materials to accompany Genetics: Analysis and Principles Robert J. Brooker CHAPTER 23 DEVELOPMENTAL GENETICS Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display INTRODUCTION Multicellular organisms begins their lives as a fertilized egg As this occurs, cells divide, migrate, and change their characteristics They become highly specialized units within a multicellular individual Developmental genetics studies the genes that orchestrate the changes that occur during development From this simple organization they proceed step by step to a much more complex arrangement It is currently one of the hottest fields in molecular biology Here, we will consider several examples in which geneticists understand how genes govern the developmental process Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-2 The Early Stages of Embryonic Development Multicellular development in plants and animals follows a body plan or pattern Pattern refers to the spatial arrangement of different body regions At the cellular level, the body pattern is due to the arrangement of cells and their specialization The progressive growth of a fertilized egg into an adult organism involves four types of cellular events: Cell division Cell movement Cell differentiation Cell death The coordination of these four events leads to the formation of a body with a particular pattern Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-4 Yolk Zygote goes through a series of nuclear divisions but NOT cytoplasmic divisions Portions of the cell membrane surround each nucleus In the middle On the inside This stage involves a great deal of cell migration which forms the Figure 23.1 Drosophila development On the outside 23-5 At the end of embryogenesis In Drosophila, there are three larval stages separated by molts During molting, the larva sheds its cuticle After the third larval stage, Drosophila proceeds through a process termed metamorphosis Groups of cells called imaginal disks were produced earlier in development These imaginal disks grow and differentiate into the structures found in the adult fly The fly then emerges from its pupal case In metazoa, the final result of development is an adult body organized along three axes Even before hatching, the embryo develops the basic body plan that will be found in the adult organism Figure 23.1 Drosophila development 23-6 Imaginal discs Early in development, the segmentation genes, play a role in the formation of body segments The expression of segmentation genes in specific regions of the embryo causes it to become segmented There are three classes of segmentation genes Gap genes Pair-rule genes Segment-polarity genes Figure 23.3 shows a few phenotypic effects observed on Drosophila larvae when a segmentation gene is defective Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-8 Eight adjacent segments are missing from the larvae Anterior portion of each segment is missing from the larvae Defective gene Even-numbered parasegments are missing from the larvae Defective gene Figure 23.3 Defective gene 23-9 The Generation of a Body Pattern Depends on Positional Information Position relative to the other cells Positional information affects cells in various ways: 1. A cell may be stimulated to divide into two daughter cells 2. A cell may be stimulated to differentiate into another cell type 3. A cell may be stimulated to migrate from one region to another 4. A cell may be stimulated to die! Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-10 Morphogens are molecules that convey positional information and promote developmental changes A morphogen influences the developmental fate of a cell A key feature of morphogens is that they act in a concentration-dependent manner They often have a critical threshold concentration Above that they exert their effect and restrict a cell into a particular developmental pathway Below that, they are ineffective Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-11 This provides positional information that establishes the general polarity of an embryo The process by which a cell or group of cells governs the developmental fate of neighboring cells Is known as induction Figure 23.4 Three molecular mechanisms of positional information 23-12 In addition to morphogens, positional information is conveyed by cell adhesion Each cell makes its own collection of cell surface receptors These are known as cell adhesion molecules (CAMs) CAMs cause the cell to adhere to the extracellular matrix (ECM) and/or to other cells CAMs Figure 23.4 Three molecular mechanisms of positional information 23-13 Stripe pattern of pair-rule gene expression in Drosophila embryo. This embryo is stained to show patterns of expression of the genes even-skipped and fushitarazu; Caption: Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-14 The Establishment of the Body Axes The first stage in Drosophila embryonic pattern development is the establishment of the body axes During oogenesis, certain gene products important in early development are deposited asymmetrically within the egg After fertilization, these gene products establish independent developmental programs These govern the formation of the body axes of the embryo These gene products act as key morphogens or receptors for morphogens Refer to Figure 23.5 Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-15 Nanos is required for the formation of the abdomen Figure 23.5 The establishment of the axes of polarity in the Drosophila embryo 23-16 Let’s now take a closer look at the molecular mechanism of bicoid The bicoid gene got its name because a larva defective in this gene develops with two posterior ends Normally found only at the posterior end Figure 23.6 Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-17 Bicoid exhibits a maternal effect mode of inheritance Refer to Chapter 7 Consider a female fly that is Phenotypically normal (because its mother was heterozygous for the normal bicoid allele) But genotypically homozygous for an inactive bicoid allele (because it inherited the inactive allele from her mother and father) This fly produces 100% affected offspring even if mated to a male that is homozygous for the normal bicoid allele In other words, the genotype of the mother determines the phenotype of the offspring This occurs because the bicoid gene product is provided to the occyte via the nurse cells Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-18 In the ovaries of female flies, the nurse cells are localized asymmetrically towards the anterior end of the oocyte Thus, maternally encoded gene products enter one side of the oocyte This side will eventually become the anterior side of the embryo The bicoid gene is actively transcribed in the nurse cells Bicoid mRNA enters the anterior end of the oocyte and is trapped there Figure 23.7 Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-19 Figure 23.7b shows an in situ hybridization experiment of bicoid mRNA Bicoid mRNA is highly concentrated near the anterior end Figure 23.7c shows an immunostaining experiment of Bicoid protein When the bicoid mRNA is translated, a gradient of Bicoid protein is established The Bicoid protein functions as a transcription factor It influences gene expression based on its concentration It stimulates a gene called hunchback in the anterior part of the embryo But not in the posterior part, where its concentration is low Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-20 Fig. 23.7b (TE Art) Copyright © The McGraw-Hill Companies, Inc. Permission required for reproduction or display. In situ hybridization of bicoid mRNA Fig. 23.7c (TE Art) Copyright © The McGraw-Hill Companies, Inc. Permission required for reproduction or display. Immunostaining of Bicoid protein The Establishment of Segmentation The next developmental process after axes formation, is the organization of the embryo into segments The segmentation pattern is shown in Figure 23.8 This pattern of positional information will be maintained or “remembered” throughout the rest of development Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-21 Note that the segments and parasegments are out of register The anterior part of a segment coincides with the posterior region of a parasegment The posterior part of a segment coincides with the anterior region of a parasegment The pattern of gene expression that occurs in the anterior region of one parasegment and the posterior region of an adjacent parasegment Results in the formation of the corresponding segment Figure 23.8 A comparison of segments and parasegments in the Drosophila embryo 23-22 The genes that play a role in the formation of body segments are called segmentation genes Gap genes Pair-rule genes Segment-polarity genes Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-23 1. Maternal effect gene products, such as bicoid mRNA are deposited asymmetrically in the oocyte These will form a gradient that will later influence the formation of axes 2. After fertilization, maternal effect gene products activate zygotic genes The first set to be activated is the gap genes 3. The gap genes and maternal effect genes then activate the pair-rule genes 4. The pair-rule genes then activate the segment polarity genes Later in development, the anterior end of one parasegment and the posterior end of another parasegment will develop into a segment Each segment will have particular morphological characteristics Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-24 Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-25 Activation of a gap gene •Bicoid activates Hunchback •Bicoid activates Krüppel; •Hunchback activates Krüppel at low concentrations and represses Krüppel at high concentrations •Knirps represses Krüppel Activation of a pair-rule gene •The even-skipped gene contains seven discrete enhancers- each are sensitive to different concentrations of maternal and gap morphogens Figure 23-9 Copyright © 2006 Pearson Prentice Hall, Inc. Figure 23-10 Copyright © 2006 Pearson Prentice Hall, Inc. Homeotic Genes and Segment Phenotype The term cell fate describes the ultimate morphological features of a cell or group of cells In Drosophila, the cells in each body segment have their fate determined very early in embryological development The term homeotic refers to mutant alleles in which one body part is replaced by another It was coined by the English zoologist William Bateson Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-26 Drosophila contains two clusters of homeotic genes The antennapedia complex contains five genes Antennapedia complex Bithorax complex Both complexes are located on chromosome 3, but a large segment of DNA separates them (Figure 23.10) lab (labial) pb (proboscipedia) dfd (deformed) scr (sex combs reduced) antp (antennapedia) The bithorax complex contains three genes ubx (ultrabithorax) abdA (abdominal A) abdB (abdominal B) Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-27 The order of gene expression, from anterior to posterior, parallels the order of genes on the chromosome The expression pattern of four genes is shown Figure 23.10 Expression pattern of homeotic genes in Drosophila 23-28 Figure 23-17 Copyright © 2006 Pearson Prentice Hall, Inc. The role of homeotic genes has been revealed by mutations that alter their function For example, Figure 23.11 shows the antennapedia mutation in Drosophila This is a gain-of-function mutation in the antp gene It causes it to be expressed in an additional place in the embryo In this case, it is also expressed in the anterior segment that normally gives rise to the antennae The abnormal expression of the antp gene in this region causes the antennae to be converted into legs! Investigators have also studied many loss-of-function alleles in homeotic genes When a particular homeotic gene is defective, its function is replaced by the gene that acts in the adjacent anterior region Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-29 Fig. 23.11b Gain of function Fig. 23.2 loss of function The homeotic genes are regulated by segmentation genes Homeotic genes encode transcription factors The coding sequence of homeotic genes contains a 180 bp consensus sequence, termed the homeobox This has been found in all homeotic boxes and in other genes affecting pattern development, such as bicoid The protein domain encoded by the homeobox is called a homeodomain The arrangement of a-helices promotes the binding of the protein to the major groove in DNA In addition, homeotic protein also contain a transcriptional activation domain Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-31 The DNA-binding sites are found within genetic regulatory elements (i.e., enhancers) Figure 23.12 Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-32 The transcription factors encoded by homeotic genes activate the next set of genes, the realizator genes These genes produce the morphological characteristics of each segment Realizator genes encode 1. Transcription factors 2. Proteins involved in cell-to-cell signaling pathways Future research will shed more light on how the realizator genes control morphological changes Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-33 23.2 VERTEBRATE DEVELOPMENT Historically, development was extensively studied in amphibians and birds Eggs are rather large and easy to manipulate Vertebrate species that have been studied include Chicken Frog (Xenopus laevis) Small aquarium zebrafish (Brachydanio rerio) Among mammals, the most extensive genetic analyses have been performed in the mouse Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-45 Researchers Have Identified Homeotic Genes in Vertebrates Vertebrates typically have long generation times and produce relatively few offspring Therefore, it is not practical to screen large numbers of embryos or offspring in search of developmental mutants Rather, cloned Drosophila genes are used as probes to identify homologous vertebrate genes Using this method, researchers have found complexes of homeotic genes in many vertebrate species In the mouse, the groups of adjacent homeotic genes are called Hox complexes Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-46 This suggests that there is a “universal body plan” for animal development Orthologous genes Chromosome 6 Chromosome 11 Chromosome 15 Chromosome 2 Thirteen different types of homeotic genes are found in the mouse However, none of the four Hox complexes contains all 13 Figure 23.15 A comparison of homeotic genes in Drosophila and the mouse 23-47 The arrangement of Hox genes along the mouse chromosome reflects their pattern of expression from the anterior to the posterior end Figure 23.16 This phenomenon is seen in more detail in Figure 23.16b The expression pattern for a group of HoxB genes is shown Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-48 Figure 23-18 Copyright © 2006 Pearson Prentice Hall, Inc. In mice, few natural mutations affect development This makes it tough to understand the role genes play in the development of the mouse and other vertebrates To circumvent this problem, geneticists are using reverse genetics 1. The Hox genes are first cloned using Drosophila genes as probes 2. A mutant version of a Hox gene is created in vitro 3. The mutant allele is the re-introduced into a mouse 4. A gene knockout is generated when the function of the wild type gene is eliminated This allows the geneticist to determine how the mutant allele affects the phenotype of the mouse Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-49 In recent years, a reverse genetics approach has been used to understand the role of the Hox genes Overall, the indication is that they play a key role in patterning the antero-posterior axis in vertebrates Nevertheless, additional research is necessary to clarify the individual roles of each of the 38 Hox genes Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-50 Cell Differentiation Cell determination Cell differentiation A cell is destined (predetermined) to become a particular cell type A cell’s morphology and function have changed, usually permanently, into a highly specialized cell type At the molecular level, the profound difference between cell types arises from gene regulation Though different cells contain the same set of genes, they regulate the expression of their genes in different ways Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-51 In Drosophila a hierarchy of gene regulation is responsible for establishing the body pattern Researchers have identified specific genes that cause cells to differentiate into particular cell types A similar type of hierarchy is though to underlie cell differentiation These genes trigger undifferentiated cells to differentiate into their proper cell fates In 1987, Harold Weintraub and his colleagues identified a gene, which they called MyoD MyoD plays a key role in skeletal muscle cell differentiation Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-52 4 genes that initiate muscle development: All four genes encode transcription factors that contain a MyoD, Myogenin, Myf5, and Mrf4 basic domain and a helix-loop-helix domain (bHLH) Binds DNA and activates skeletalmuscle specific genes Necessary for dimer formation between transcription factor proteins The four genes are called myogenic bHLH proteins They are found in all vertebrates and even some invertebrates (Drosophila and C. elegans) They are activated during skeletal muscle cell development Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-53 Molecularly, two key features enable myogenic bHLH proteins to promote muscle cell differentiation 1. The basic domain binds specifically to a muscle-cell- specific enhancer sequence This is adjacent to genes that are only expressed in muscle cells 2. Their activity is regulated by dimerization Heterodimers may be activating or inhibitory Copyright ©The McGraw-Hill Companies, Inc. Permission required for reproduction or display 23-54 At later stages, the levels of the Id protein fall Inhibitor of differentiation The Id protein is produced during early stages of development Figure 23.17 Myogenic bHLH can now combine with the E proteins to induce muscle differentiation It prevents myogenic bHLH from promoting muscle differentiation too soon 23-55 Fruit fly with extra eyes Figure 23-3 Copyright © 2006 Pearson Prentice Hall, Inc.