The American Journal of Human Genetics - 2014

196 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
This Month in Genetics
Gene Age Predicts the Strength of Purifying Selection Acting on Gene Expression Variation in Humans
This Month in The Journal
Homozygous and Compound-Heterozygous Mutations in TGDS Cause Catel-Manzke Syndrome
Genetic Association Analysis under Complex Survey Sampling: The Hispanic Community Health Study/Study of Latinos
Biallelic Truncating Mutations in FMN2, Encoding the Actin-Regulatory Protein Formin 2, Cause Nonsyndromic Autosomal-Recessive Intellectual Disability
MCM9 Mutations Are Associated with Ovarian Failure, Short Stature, and Chromosomal Instability
A Rare Functional Noncoding Variant at the GWAS-Implicated MIR137/MIR2682 Locus Might Confer Risk to Schizophrenia and Bipolar Disorder
Mutations in RAB39B Cause X-Linked Intellectual Disability and Early-Onset Parkinson Disease with α-Synuclein Pathology
Compound Heterozygosity of Low-Frequency Promoter Deletions and Rare Loss-of-Function Mutations in TXNL4A Causes Burn-McKeown Syndrome
Mutations in GTPBP3 Cause a Mitochondrial Translation Defect Associated with Hypertrophic Cardiomyopathy, Lactic Acidosis, and Encephalopathy
MFAP5 Loss-of-Function Mutations Underscore the Involvement of Matrix Alteration in the Pathogenesis of Familial Thoracic Aortic Aneurysms and Dissections
Absence of BiP Co-chaperone DNAJC3 Causes Diabetes Mellitus and Multisystemic Neurodegeneration
Loss-of-Function Mutations in WDR73 Are Responsible for Microcephaly and Steroid-Resistant Nephrotic Syndrome: Galloway-Mowat Syndrome
Mutations Affecting the BHLHA9 DNA-Binding Domain Cause MSSD, Mesoaxial Synostotic Syndactyly with Phalangeal Reduction, Malik-Percin Type
Partitioning Heritability of Regulatory and Cell-Type-Specific Variants across 11 Common Diseases
Mutations in SNX14 Cause a Distinctive Autosomal-Recessive Cerebellar Ataxia and Intellectual Disability Syndrome
Mutations in CKAP2L, the Human Homolog of the Mouse Radmis Gene, Cause Filippi Syndrome
This Month in Genetics
Dosage Changes of a Segment at 17p13.1 Lead to Intellectual Disability and Microcephaly as a Result of Complex Genetic Interaction of Multiple Genes
This Month in The Journal
A Peroxisomal Disorder of Severe Intellectual Disability, Epilepsy, and Cataracts Due to Fatty Acyl-CoA Reductase 1 Deficiency
Truncating and Missense Mutations in IGHMBP2 Cause Charcot-Marie Tooth Disease Type 2
PRIMUS: Rapid Reconstruction of Pedigrees from Genome-wide Estimates of Identity by Descent
Cross-Tissue and Tissue-Specific eQTLs: Partitioning the Heritability of a Complex Trait
A Selective Sweep on a Deleterious Mutation in CPT1A in Arctic Populations
Mutations in PURA Cause Profound Neonatal Hypotonia, Seizures, and Encephalopathy in 5q31.3 Microdeletion Syndrome
A Novel Test for Recessive Contributions to Complex Diseases Implicates Bardet-Biedl Syndrome Gene BBS10 in Idiopathic Type 2 Diabetes and Obesity
Molecular Convergence of Neurodevelopmental Disorders
Structural Architecture of SNP Effects on Complex Traits
Biased Gene Conversion Skews Allele Frequencies in Human Populations, Increasing the Disease Burden of Recessive Alleles
Ascertainment Bias Causes False Signal of Anticipation in Genetic Prion Disease
Characteristics of Neutral and Deleterious Protein-Coding Variation among Individuals and Populations
Effective Genetic-Risk Prediction Using Mixed Models
Mutations in LAMA1 Cause Cerebellar Dysplasia and Cysts with and without Retinal Dystrophy
Genome-wide Association Study Identifies Five Susceptibility Loci for Follicular Lymphoma outside the HLA Region
Cryptic and Complex Chromosomal Aberrations in Early-Onset Neuropsychiatric Disorders
This Month in Genetics
This Month in The Journal
Synaptotagmin 2 Mutations Cause an Autosomal-Dominant Form of Lambert-Eaton Myasthenic Syndrome and Nonprogressive Motor Neuropathy
Widespread Signals of Convergent Adaptation to High Altitude in Asia and America
Utilizing Ethnic-Specific Differences in Minor Allele Frequency to Recategorize Reported Pathogenic Deafness Variants
De Novo Mutations in Synaptic Transmission Genes Including DNM1 Cause Epileptic Encephalopathies
Genome-wide Scan of 29,141 African Americans Finds No Evidence of Directional Selection since Admixture African Americans
Parent of Origin, Mosaicism, and Recurrence Risk: Probabilistic Modeling Explains the Broken Symmetry of Transmission Genetics
Transcriptome Sequencing of a Large Human Family Identifies the Impact of Rare Noncoding Variants
Identification and Validation of Genetic Variants that Influence Transcription Factor and Cell Signaling Protein Levels
CCDC151 Mutations Cause Primary Ciliary Dyskinesia by Disruption of the Outer Dynein Arm Docking Complex Formation
Mutations in FEZF1 Cause Kallmann Syndrome
Synaptotagmin 2 Mutations Cause an Autosomal-Dominant Form of Lambert-Eaton Myasthenic Syndrome and Nonprogressive Motor Neuropathy
This Month in Genetics
This Month in The Journal
Mutations in APOPT1, Encoding a Mitochondrial Protein, Cause Cavitating Leukoencephalopathy with Cytochrome c Oxidase Deficiency
A Mutation of COX6A1 Causes a Recessive Axonal or Mixed Form of Charcot-Marie-Tooth Disease
Neu-Laxova Syndrome Is a Heterogeneous Metabolic Disorder Caused by Defects in Enzymes of the L-Serine Biosynthesis Pathway
Mutations in GRHL2 Result in an Autosomal-Recessive Ectodermal Dysplasia Syndrome
Gene-Environment Dependence Creates Spurious Gene-Environment Interaction
Mutations in NOTCH1 Cause Adams-Oliver Syndrome
This Month in Genetics
Mutations in LAMA1 Cause Cerebellar Dysplasia and Cysts with and without Retinal Dystrophy
This Month in The Journal
Exome Sequencing Identifies a Recurrent De Novo ZSWIM6 Mutation Associated with Acromelic Frontonasal Dysostosis
Pathogenic Variants for Mendelian and Complex Traits in Exomes of 6,517 European and African Americans: Implications for the Return of Incidental Results European Americans
Fine Mapping Major Histocompatibility Complex Associations in Psoriasis and Its Clinical Subtypes
SPEG Interacts with Myotubularin, and Its Deficiency Causes Centronuclear Myopathy with Dilated Cardiomyopathy
Identification and Validation of Genetic Variants that Influence Transcription Factor and Cell Signaling Protein Levels
Parental Somatic Mosaicism Is Underrecognized and Influences Recurrence Risk of Genomic Disorders
The Alu-Rich Genomic Architecture of SPAST Predisposes to Diverse and Functionally Distinct Disease-Associated CNV Alleles
ELOVL5 Mutations Cause Spinocerebellar Ataxia 38
Disruption of the Basal Body Protein POC1B Results in Autosomal-Recessive Cone-Rod Dystrophy
Rare-Variant Association Analysis: Study Designs and Statistical Tests
This Month in Genetics
Rare Variants in NR2F2 Cause Congenital Heart Defects in Humans
Genome-wide Identification and Characterization of Fixed Human-Specific Regulatory Regions
Targeted Resequencing and Systematic In Vivo Functional Testing Identifies Rare Variants in MEIS1 as Significant Contributors to Restless Legs Syndrome
Aicardi-Goutières Syndrome Is Caused by IFIH1 Mutations
Joint Analysis of Functional Genomic Data and Genome-wide Association Studies of 18 Human Traits
Examining Variation in Recombination Levels in the Human Female: A Test of the Production-Line Hypothesis
This Month in The Journal
Mutations in SLC13A5 Cause Autosomal-Recessive Epileptic Encephalopathy with Seizure Onset in the First Days of Life
Effects of Long-Term Averaging of Quantitative Blood Pressure Traits on the Detection of Genetic Associations
Integrative DNA, RNA, and Protein Evidence Connects TREML4 to Coronary Artery Calcification
Attitudes of Genetics Professionals Toward the Return of Incidental Results from Exome and Whole-Genome Sequencing
Gene-Age Interactions in Blood Pressure Regulation: A Large-Scale Investigation with the CHARGE, Global BPgen, and ICBP Consortia
PGM3 Mutations Cause a Congenital Disorder of Glycosylation with Severe Immunodeficiency and Skeletal Dysplasia
Transcriptional Consequences of 16p11.2 Deletion and Duplication in Mouse Cortex and Multiplex Autism Families
Monoallelic and Biallelic Mutations in MAB21L2 Cause a Spectrum of Major Eye Malformations
FORGE Canada Consortium: Outcomes of a 2-Year National Rare-Disease Gene-Discovery Project
Janet Davison Rowley, M.D. (1925–2013)
This Month in Genetics
This Month in The Journal
Mutations of CEP83 Cause Infantile Nephronophthisis and Intellectual Disability
An Enhancer Polymorphism at the Cardiomyocyte Intercalated Disc Protein NOS1AP Locus Is a Major Regulator of the QT Interval
Contrasting X-Linked and Autosomal Diversity across 14 Human Populations
Neu-Laxova Syndrome, an Inborn Error of Serine Metabolism, Is Caused by Mutations in PHGDH
Utilizing Population Controls in Rare-Variant Case-Parent Association Tests
Return of Genomic Results to Research Participants: The Floor, the Ceiling, and the Choices In Between
Mutations in DOCK7 in Individuals with Epileptic Encephalopathy and Cortical Blindness
Mutations in EMP2 Cause Childhood-Onset Nephrotic Syndrome
Variant Association Tools for Quality Control and Analysis of Large-Scale Sequence and Genotyping Array Data
This Month in The Journal
Biallelic Variants in TTLL5, Encoding a Tubulin Glutamylase, Cause Retinal Dystrophy
AP1S3 Mutations Are Associated with Pustular Psoriasis and Impaired Toll-like Receptor 3 Trafficking
De Novo Truncating Mutations in AHDC1 in Individuals with Syndromic Expressive Language Delay, Hypotonia, and Sleep Apnea
This Month in Genetics
Integrin Alpha 8 Recessive Mutations Are Responsible for Bilateral Renal Agenesis in Humans
Alleles of a Polymorphic ETV6 Binding Site in DCDC2 Confer Risk of Reading and Language Impairment
Mutations in ASPH Cause Facial Dysmorphism, Lens Dislocation, Anterior-Segment Abnormalities, and Spontaneous Filtering Blebs, or Traboulsi Syndrome
Convergence of Genes and Cellular Pathways Dysregulated in Autism Spectrum Disorders
Simulation of Finnish Population History, Guided by Empirical Genetic Data, to Assess Power of Rare-Variant Tests in Finland
Describing Sequencing Results of Structural Chromosome Rearrangements with a Suggested Next-Generation Cytogenetic Nomenclature
Maximizing the Power of Principal-Component Analysis of Correlated Phenotypes in Genome-wide Association Studies
Recurrent CNVs and SNVs at the NPHP1 Locus Contribute Pathogenic Alleles to Bardet-Biedl Syndrome
Mutations Affecting the SAND Domain of DEAF1 Cause Intellectual Disability with Severe Speech Impairment and Behavioral Problems
Maternal History of Oceania from Complete mtDNA Genomes: Contrasting Ancient Diversity with Recent Homogenization Due to the Austronesian Expansion
Mutations in PIEZO2 Cause Gordon Syndrome, Marden-Walker Syndrome, and Distal Arthrogryposis Type 5
Rare Variants in NR2F2 Cause Congenital Heart Defects in Humans
Phevor Combines Multiple Biomedical Ontologies for Accurate Identification of Disease-Causing Alleles in Single Individuals and Small Nuclear Families
Multicentric Carpotarsal Osteolysis Is Caused by Mutations Clustering in the Amino-Terminal Transcriptional Activation Domain of MAFB
Two Functional Lupus-Associated BLK Promoter Variants Control Cell-Type- and Developmental-Stage-Specific Transcription
Joint Analysis of Functional Genomic Data and Genome-wide Association Studies of 18 Human Traits
De Novo Mutations in the Beta-Tubulin Gene TUBB2A Cause Simplified Gyral Patterning and Infantile-Onset Epilepsy
Loss of α1β1 Soluble Guanylate Cyclase, the Major Nitric Oxide Receptor, Leads to Moyamoya and Achalasia
This Month in The Journal
This Month in Genetics
Antisense Oligonucleotide-Based Therapy in Human Erythropoietic Protoporphyria
Progesterone Antagonist Therapy in a Pelizaeus-Merzbacher Mouse Model
Whole-Exome Sequencing Identifies KIZ as a Ciliary Gene Associated with Autosomal-Recessive Rod-Cone Dystrophy
De Novo Loss-of-Function Mutations in SETD5, Encoding a Methyltransferase in a 3p25 Microdeletion Syndrome Critical Region, Cause Intellectual Disability
GeMes, Clusters of DNA Methylation under Genetic Control, Can Inform Genetic and Epigenetic Analysis of Disease
Mutations in QARS, Encoding Glutaminyl-tRNA Synthetase, Cause Progressive Microcephaly, Cerebral-Cerebellar Atrophy, and Intractable Seizures
Meta-analysis of Genome-wide Association Studies Identifies 1q22 as a Susceptibility Locus for Intracerebral Hemorrhage
Fine Mapping Seronegative and Seropositive Rheumatoid Arthritis to Shared and Distinct HLA Alleles by Adjusting for the Effects of Heterogeneity
Genetic Origins of Lactase Persistence and the Spread of Pastoralism in Africa
2013 William Allan Award Introduction: Aravinda Chakravarti
2013 Victor A. McKusick Leadership Award Addresses
2013 Victor A. McKusick Leadership Award Introduction: Kurt and Rochelle Hirschhorn
2013 Curt Stern Award Address
2013 Curt Stern Award Introduction: John Moran
2013 William Allan Award: My Multifactorial Journey
2013 ASHG Awards and Addresses
Whole-Exome Sequencing of 2,000 Danish Individuals and the Role of Rare Coding Variants in Type 2 Diabetes
Early Austronesians: Into and Out Of Taiwan
Mutations in USP9X Are Associated with X-Linked Intellectual Disability and Disrupt Neuronal Cell Migration and Growth
This Month in The Journal
An Excess of Risk-Increasing Low-Frequency Variants Can Be a Signal of Polygenic Inheritance in Complex Diseases
2013 Presidential Address: Just Another President’s Speech (but It’s All about You)
This Month in Genetics
A Higher Mutational Burden in Females Supports a “Female Protective Model” in Neurodevelopmental Disorders
Loss of α1β1 Soluble Guanylate Cyclase, the Major Nitric Oxide Receptor, Leads to Moyamoya and Achalasia
XYLT1 Mutations in Desbuquois Dysplasia Type 2
An Expressed Retrogene of the Master Embryonic Stem Cell Gene POU5F1 Is Associated with Prostate Cancer Susceptibility
A Common 16p11.2 Inversion Underlies the Joint Susceptibility to Asthma and Obesity
Exome-Based Mapping and Variant Prioritization for Inherited Mendelian Disorders
Gene-centric Meta-analysis in 87,736 Individuals of European Ancestry Identifies Multiple Blood-Pressure-Related Loci European Ancestry
Mechanism, Prevalence, and More Severe Neuropathy Phenotype of the Charcot-Marie-Tooth Type 1A Triplication
Mitochondrial Carbonic Anhydrase VA Deficiency Resulting from CA5A Alterations Presents with Hyperammonemia in Early Childhood
Whole-Exome Sequencing Identifies Rare and Low-Frequency Coding Variants Associated with LDL Cholesterol
Solving Glycosylation Disorders: Fundamental Approaches Reveal Complicated Pathways
Mutations in CSPP1 Cause Primary Cilia Abnormalities and Joubert Syndrome with or without Jeune Asphyxiating Thoracic Dystrophy
Association of Low-Frequency and Rare Coding-Sequence Variants with Blood Lipids and Coronary Heart Disease in 56,000 Whites and Blacks Whites and Blacks
This Month in Genetics
Causal Effects of Body Mass Index on Cardiometabolic Traits and Events: A Mendelian Randomization Analysis
The IL-33-ST2L Pathway Is Associated with Coronary Artery Disease in a Chinese Han Population
A Statistical Framework to Guide Sequencing Choices in Pedigrees
ERCC6L2 Mutations Link a Distinct Bone-Marrow-Failure Syndrome to DNA Repair and Mitochondrial Function
This Month in The Journal
Heterozygous Loss-of-Function Mutations in YAP1 Cause Both Isolated and Syndromic Optic Fissure Closure Defects
Causal Effects of Body Mass Index on Cardiometabolic Traits and Events: A Mendelian Randomization Analysis
A Founder Mutation in PET100 Causes Isolated Complex IV Deficiency in Lebanese Individuals with Leigh Syndrome
NR2F1 Mutations Cause Optic Atrophy with Intellectual Disability
Mutations in PGAP3 Impair GPI-Anchor Maturation, Causing a Subtype of Hyperphosphatasia with Mental Retardation
A Common Functional Regulatory Variant at a Type 2 Diabetes Locus Upregulates ARAP1 Expression in the Pancreatic Beta Cell
Integrin Alpha 8 Recessive Mutations Are Responsible for Bilateral Renal Agenesis in Humans
Revisiting the Thrifty Gene Hypothesis via 65 Loci Associated with Susceptibility to Type 2 Diabetes
Annotating DNA Variants Is the Next Major Goal for Human Genetics
Reponse to de Leeuw and Houge
An APOA5 3′ UTR Variant Associated with Plasma Triglycerides Triggers APOA5 Downregulation by Creating a Functional miR-485-5p Binding Site
Immunochip Analysis Identifies Multiple Susceptibility Loci for Systemic Sclerosis
Loss of PRDM16 Is Unlikely to Cause Cardiomyopathy in 1p36 Deletion Syndrome
Mutations in POGLUT1, Encoding Protein O-Glucosyltransferase 1, Cause Autosomal-Dominant Dowling-Degos Disease
Loss of Association of REEP2 with Membranes Leads to Hereditary Spastic Paraplegia
This Month in The Journal
This Month in Genetics
Mutations in PCYT1A, Encoding a Key Regulator of Phosphatidylcholine Metabolism, Cause Spondylometaphyseal Dysplasia with Cone-Rod Dystrophy
Mutations in PCYT1A Cause Spondylometaphyseal Dysplasia with Cone-Rod Dystrophy
Mutations in TBC1D24, a Gene Associated With Epilepsy, Also Cause Nonsyndromic Deafness DFNB86
Dominant Mutations in GRHL3 Cause Van der Woude Syndrome and Disrupt Oral Periderm Development
Rare-Variant Extensions of the Transmission Disequilibrium Test: Application to Autism Exome Sequence Data
Mutations in CSPP1 Cause Primary Cilia Abnormalities and Joubert Syndrome with or without Jeune Asphyxiating Thoracic Dystrophy
Mutations in CSPP1 Lead to Classical Joubert Syndrome
A Noncoding Expansion in EIF4A3 Causes Richieri-Costa-Pereira Syndrome, a Craniofacial Disorder Associated with Limb Defects
Exome Sequence Reveals Mutations in CoA Synthase as a Cause of Neurodegeneration with Brain Iron Accumulation
Mutations in CSPP1, Encoding a Core Centrosomal Protein, Cause a Range of Ciliopathy Phenotypes in Humans
Mutations in DNAH1, which Encodes an Inner Arm Heavy Chain Dynein, Lead to Male Infertility from Multiple Morphological Abnormalities of the Sperm Flagella
Mutations in KPTN Cause Macrocephaly, Neurodevelopmental Delay, and Seizures