Abstract
We analyzed the whole-genome sequences of a family of four, consisting of two siblings and their parents. Family-based sequencing allowed us to delineate recombination sites precisely, identify 70% of the sequencing errors (resulting in > 99.999% accuracy), and identify very rare single-nucleotide polymorphisms. We also directly estimated a human intergeneration mutation rate of approximately 1.1 x 10(-8) per position per haploid genome. Both offspring in this family have two recessive disorders: Miller syndrome, for which the gene was concurrently identified, and primary ciliary dyskinesia, for which causative genes have been previously identified. Family-based genome analysis enabled us to narrow the candidate genes for both of these Mendelian disorders to only four. Our results demonstrate the value of complete genome sequencing in families.
MeSH Terms
Abnormalities, Multiple/genetics
Algorithms
Alleles
Axonemal Dyneins/genetics
Ciliary Motility Disorders/genetics
Crossing Over, Genetic
Dihydroorotate Dehydrogenase
Female
Genes, Dominant
Genes, Recessive
Genetic Association Studies
Genome, Human
Humans
Inheritance Patterns
Limb Deformities, Congenital/genetics
Male
Mandibulofacial Dysostosis/genetics
Mutation
Nuclear Family
Oxidoreductases Acting on CH-CH Group Donors/genetics
Pedigree
Polymorphism, Single Nucleotide
Sequence Analysis, DNA
Syndrome
Chemicals
Dihydroorotate Dehydrogenase
Oxidoreductases Acting on CH-CH Group Donors
Axonemal Dyneins
DNAH5 protein, human
Authors & Affiliations
15 authors, click to expand affiliations / ORCID
Roach Jared C
Institute for Systems Biology, Seattle, WA 98103, USA.
Glusman Gustavo
Smit Arian F A
Huff Chad D
Hubley Robert
Shannon Paul T
Rowen Lee
Pant Krishna P
Goodman Nathan
Bamshad Michael
Shendure Jay
Drmanac Radoje
Jorde Lynn B
Hood Leroy
Galas David J
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