Population Genetics and Human Disease

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Using genetics to study human history and natural selection David Reich Harvard Medical School Depatment of Genetics Broad Institute t tttctccatttgtcgtgacacctttgttgacaccttcatttctgcattctcaattctatttcactggtctatgg c g cagagaacacaaaatatggccagtggcctaaatccagcctactaccttttttttttttttgtaacattttacta a g t t acatagccattcccatgtgtttccatgtgtctgggctgcttttgcactctaatggcagagttaagaaattgtag a c c cagagaccacaatgcctcaaatatttactctacagccctttataaaaacagtgtgccaactcctgatttatgaa cttatcattatgtcaataccatactgtctttattactgtagttttataagtcatgacatcagataatgtaaatc g ctccaactttgtttttaatcaaaagtgttttggccatcctagatatactttgtattgccacataaatttgaaga a g tcagcctgtcagtgtctacaaaatagcatgctaggattttgatagggattgtgtagaatctatagattaattag c t aggagaatgactatcttgacaatactgctgcccctctgtattcgtgggggattggttccacaacaacacccacc c ccccactcggcaacccctgaaacccccacatcccccagcttttttcccctgctaccaaaatccatggatgctca g agtccatataaaatgccatactatttgcatataacctctgcaatcctcccctatagtttagatcatctctagat a t t t tacttataatactaataaaatctaaatgctatgtaaatagttgctatactgtgttgagggttttttgttttgtt c c c ttgttttatttgtttgtttgtttgtattttaagagatggtgtcttgctttgttgcccaggctggagtgcagtgg g tgagatcatagcttactgcagcctcaaactcctggactcaaacagtcctcccacctcagcctcccaaagtgctg a ggatacaggtgtgacccactgtgcccagttattattttttatttgtattattttactgttgtattatttttaat tattttttctgaatattttccatctatagttggttgaatcatggatgtggaacaggcaaatatggagggctaac g t g tgtattgcatcttccagttcatgagtatgcagtctctctgtttatttaaagttttagtttttctcaaccatgtt a c a tacttttcagtatacaagactttgacgttttttgttaaatgtatttgtaagtattttattatttgtgatgttat ttaaaaagaaattgttgactgggcacagtggctcacgcctgtaatcccagcactttgggaggctgaggcgggca t g gatcacgaggtcaggagatcaagaccatcctggctaacatggtaaaaccccgtctctactaaaaatagaaaaaa c a g attagccaggcgtggtggcgagtgcctgtagtcccagctactcgggaggctgaggcaggagaatggtgtgaacc c g tgggaggcggagcttgcagtgagctgagatcgtgccactgcattccagcctgcgtgacagagcgagactctgtc c g aaaaaaataaataaaatttaaaaaaagaagaagaaattattttcttaatttcattttcaggttttttatttatt a g t tctactatatggatacatgattgatttttgtatattgatcatgtatcctgcaaactagctaacatagtttatta a c tttctctttttttgtggattttaaaggattttctacatagataaataaacacacataaacagttttacttcttt cttttcaacctagactggatgcattttttgtttttgtttgtttgtttgctttttaacttgctgcagtgactaga g g g gaatgtattgaagaatatattgttgaacaaaagcagtgagagtggacatccctgctttccccctgattttaggg a c a g ggaatgttttcagtctttcactatttaatatgattttagctataggtttatcctagatccctgttatcatgttg a aggaaattcccttctatttctagtttgttgagattttttaattcatgtgattgcgctatctggctttgctctca A 2-part talk: Section 1: How human history affects human genetic variation Section 2: Detecting selection by the pattern of genetic variation and finding disease genes Section 1 How does human history affect genetic variation? A genome-wide survey of Linkage Disequilibrium Linkage disequilibrium is a phenomenon whereby genetic variants are associated: people who have one tend to have a second as well Section 1 Linkage Disequilibrium Explained Emergence of Variations Over Time Variations in Chromosomes Within a Population Common Ancestor Disease Mutation time present What Determines Extent of LD? Section 1 Disease-Causing Mutation 2,000 gens. ago 1,000 gens. ago Time = present Section 1 How Far Does Association (LD) Extend Between Neighboring Common Sites? • Theoretical: Range of uncertainty 3-8 kb 160kb 0kb 5kb 10kb 20kb 40kb 80kb Section 1 Strategy for Assessing Extent of LD 5 5 10 20 40 80 160kb 0kb 5kb 10kb 20kb 40kb 80kb Distance from core single nucleotide polymorphism (SNP) • 19 regions • 44 Caucasian samples from Utah • a great deal of DNA sequencing per sample Section 1 Section 1 A Genome-Wide Assessment of Linkage Disequilibrium Disease Gene Mapping Human history Section 1 MYSTERY: What explains the long-range LD?  Important event in population history? Section 1 Positive Control: 48 Swedes 1 0.9 0.8 Utah LD Curve Linkage Disequilibrium D' 0.7 0.6 0.5 0.4 0.3 0.2 0.1 0 3.5 5kb 10kb 20kb 40kb 80kb 160kb Sweden LD Sweden LD With Sign of D' set by Utah Identical pattern to Utah Distance Between SNPs (Base Pairs) Section 1 96 Nigerians (Yoruba) 1 0.9 0.8 0.7 Utah LD Curve Linkage Disequilibrium D' 0.6 0.5 0.4 0.3 0.2 0.1 0 -0.1 -0.2 3.5 Nigeria LD with sign of D' set by Utah Nigeria LD Much Less LD Associations in Africans a SUBSET of those in Caucasians 5kb 10kb 20kb 40kb 80kb 160kb Distance Between SNPs (Base Pairs) MUST be influenced by population history Confirmation of less LD in Africans from Direct DNA Sequencing 1 0.9 0.8 0.7 Mean |D'| Section 1 313 101 174 86 6 48 4 63 20 83 98 16 Nigerian Utah 67 56 0.6 0.5 0.4 0.3 0.2 0.1 0 500bp 5kb 10kb 20kb 40kb 80kb 160kb Anna DiRienzo also shows this pattern More evidence from Genotyping ~5,000 SNPs (Gabriel et al. 2002) 1 0.9 0.8 0.7 Section 1 Caucasian African-American Asian Yoruban Mean |D'| 0.6 0.5 0.4 0.3 0.2 0.1 0 0 50,000 100,000 Distance (bp) 150,000 K. Kidd, J. Kidd, Sarah Tishkoff also show this Explanation: Bottleneck or „Founder Section 1 Effect‟ in History of North Europeans Ancestral Population • likely <10 founding chromosomes What was this event? ~100,000 years ago (1) Out of Africa? (2) Founding of Europe? North Europeans Yoruba Ancestors Section 1 Open Mysteries • what caused the bottleneck event? “Out of Africa” migration? • how many people involved? When did it occur? • can we better understand when the founder event occurred, and how many people involved? Acknowledgements for Section 1 Collaborators: Michele Cargill Stacey Bolk James Ireland Pardis C. Sabeti Daniel J. Richter Thomas Lavery Rose Kouyoumjian Shelli F. Farhadian Ryk Ward Eric S. Lander Samples: Leif Groop Richard Cooper Charles Rotimi Section 2 Using Long-Range Linkage Disequilibrium to Detect Positive Selection in the Genome Overview Section 2 1. The difficulty of detecting genomic regions affected by natural selection 2. The long-range haplotype test 3. Results for two genes: G6PD and CD40 ligand Existing formal tests for selection DNA Sequence analysis Tajima‟s D HKA test Mcdonald and Kreitman Fu and Li‟s D Ka/Ks ratio Section 2 Weak Genotyping-based tests Not general at present Our test is based on the relationship between Section 2 allele frequency and extent of linkage disequilibrium No selection Young alleles: • low frequency • long-range LD Old alleles: • low or high frequency • short-range LD Positive Selection Young alleles: • high frequency • long-range LD The signal of selection Linkage Disequilibrium (Homozygosity) Section 2 Positive Selection Neutrality frequency Paradigm of the Core Region Section 2 gene 1 2 3 4 5 Core Haplotypes Long-range multi-SNP haplotypes Core markers Long-range markers C/T A/G A/G C/T C/T Section 2 C/T gene 1 2 3 4 5 Decay of LD Long-range multi-SNP haplotypes Core markers gene Section 2 Long-range markers C/T A/G A/G C/T C/T C/T C T A C T T C G G C G C C T T 3 Decay of homozygosity (probability, at any distance, that any two haplotypes that start out the same have all the same SNP genotypes) T T C 100% 75% 35% 18% Section 2 Two genes associated with malaria resistance G6PD (1960‟s) • well established association to malaria resistance • selection demonstrated in 2001 by Tishkoff et al. CD40 ligand (2002): • Recent association by Sabeti et al. • involved in immune regulation Experimental Design -480kb Section 2 G6PD +220kb telomere G6PD -480kb -180kb (11 SNPs in core, 14 at long distances) Gene TNFSF5 +220kb +520kb telomere CD40 ligand -180kb (7 SNPs in core, 14 at long distances) +520kb Gene Experimental Design DNA samples from 231 African men Yoruba (Nigeria) Beni (Nigeria) Shona (Zimbabwe) Perfect phase (X chromosome) Section 2 Core haplotypes G6PD Africans non-Africans (230) (95) 1 2 3 4 5 6 7 8 9 Section 2 CD40 ligand Africans non-Africans (231) (91) 1 2 3 4 5 6 38 72 4 28 28 14 41 5 4 61 13 5 91 9 78 30 1 77 21 7 7 “A-” protective haplotype 17 G6PD: long-range haplotype diversity G6PD-corehap1 G6PD-corehap6 Section 2 G6PD-corehap3 G6PD-corehap7 G6PD-corehap4 G6PD-corehap8 “A-” protective haplotype G6PD-corehap5 G6PD-corehap Section 2 G6PD: homozygosity vs. distance EHH Distance from the core region (kb) Section 2 G6PD: computer simulation vs. data Core haplotype 8 P << 0.0008 Relative EHH Core haplotype frequency Section 2 G6PD: P-values from simulation P- value Distance from the core region ( kb) G6PD also stands out in comparison to 7 control regions Section 2 Relative EHH Core haplotype frequency CD40 ligand: long-range haplotype diversity corehap1 corehap4 Section 2 corehap2 corehap5 corehap3 Section 2 CD40 ligand: homozygosity vs. distance EHH Distance from the core region (kb) Section 2 CD40 ligand: computer simulation vs. data Core haplotype 4 P << 0.0011 Relative EHH Core haplotype frequency Section 2 CD40 ligand: P-values from simulation P- value Distance from the core region ( kb) CD40 ligand also stands out in comparison to 7 control regions Section 2 Relative EHH Core haplotype frequency Malaria resistance arose in last 10,000 years in Africa Section 2 Long-range linkage disequilibrium also gives a direct estimate of the date ~2,500 years ago for G6PD ~6,500 years ago for CD40 ligand Traditional tests fail to detect the effect Tajima‟s D HKA test Mcdonald and Kreitman Fu and Li‟s D Ka/Ks ratio Section 2 Not significant in our data. This test is a powerful way to detect selection in last 10,000 years Section 2 Conclusions: Powerful general approach for detecting selection 1 2 3 4 Section 2 Conclusions: Powerful general approach for detecting selection 1 2 3 4 5 Section 2 Conclusions: Powerful general approach for detecting selection 1 2 3 4 Screen the genome for Postive Selection Section 2 Conclusions: Genome-wide screen for natural selection We can find disease genes without patients! What‟s coming… Section 2 1. Generalization of the long-range haplotype test 2. Application of the approach genome-wide • Haplotype map data set • Disease gene screen data sets Acknowledgements for Section 2 Pardis C. Sabeti John Higgins Haninah Z.P. Levine Daniel J. Richter Stephen F. Schaffner Stacey Gabriel Jill V. Platko Nicholas J. Patterson Gavin J. McDonald Hans C. Ackerman Sarah J. Campbell David Altshuler Richard Cooper Ryk Ward Eric S. Lander Note The 3rd section of the talk is not included here because it presents data that have not yet been published.

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