The American Journal of Human Genetics - 2001

393 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
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Announcements11Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Unive
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The Tumor‐Necrosis‐Factor Receptor–Associated Periodic Syndrome: New Mutations in<i>TNFRSF1A,</i>Ancestral Origins, Genotype‐Phenotype Studies, and Evidence for Further Genetic Heterogeneity of Period
A Common Ancestral Origin of the Frequent and Widespread 2299delG<i>USH2A</i>Mutation
Genetic and Mutational Analysis of a Large Multiethnic Bardet‐Biedl Cohort Reveal a Minor Involvement of<i>BBS6</i>and Delineate the Critical Intervals of Other Loci
Leber Congenital Amaurosis and Retinitis Pigmentosa with Coats‐Like Exudative Vasculopathy Are Associated with Mutations in the Crumbs Homologue I (<i>CRB1</i>) Gene
Erratum
Announcements11. Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Uni
Erratum
Announcements11Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Unive
Contents of Volume 68
Periaxin Mutations Cause Recessive Dejerine‐Sottas Neuropathy
High‐Resolution Multipoint Linkage‐Disequilibrium Mapping in the Context of a Human Genome Sequence
A Genomewide Screen for Autism Susceptibility Loci
Contents of Volume 69
The Amyloid Precursor Protein Locus and Very‐Late‐Onset Alzheimer Disease
Mutations in a Novel Gene with Transmembrane Domains Underlie Usher Syndrome Type 3
Announcements11. Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Uni
Do the Four Clades of the mtDNA Haplogroup L2 Evolve at Different Rates?
Multipoint Genetic Mapping with Trisomy Data
Survival of Male Patients with Incontinentia Pigmenti Carrying a Lethal Mutation Can Be Explained by Somatic Mosaicism or Klinefelter Syndrome
Identification of a Major Susceptibility Locus for Restless Legs Syndrome on Chromosome 12q
Type III 3-Methylglutaconic Aciduria (Optic Atrophy Plus Syndrome, or Costeff Optic Atrophy Syndrome): Identification of the OPA3 Gene and Its Founder Mutation in Iraqi Jews Iraqi Jews
This Month in the Journal
Nonsense and Frameshift Mutations in ZFHX1B, Encoding Smad-Interacting Protein 1, Cause a Complex Developmental Disorder with a Great Variety of Clinical Features
Evidence for a Susceptibility Gene, SLEV1, on Chromosome 17p13 in Families with Vitiligo-Related Systemic Lupus Erythematosus
Erratum
Spinal Neurofibromatosis without Café-au-Lait Macules in Two Families with Null Mutations of the NF1 Gene
A Genomewide Search for Quantitative-Trait Loci Underlying Asthma
Origins and Divergence of the Roma (Gypsies)
A Genomewide Scan for Type 1–Diabetes Susceptibility in Scandinavian Families: Identification of New Loci with Evidence of Interactions
Parent-Specific Complementary Patterns of Histone H3 Lysine 9 and H3 Lysine 4 Methylation at the Prader-Willi Syndrome Imprinting Center
Large Upward Bias in Estimation of Locus-Specific Effects from Genomewide Scans
The Discovery of Single-Nucleotide Polymorphisms—and Inferences about Human Demographic History
A Second Leaky Splice-Site Mutation in the Spastin Gene
Editorial Reviewers for 2001
Type 2 Diabetes and Three Calpain-10 Gene Polymorphisms in Samoans: No Evidence of Association
Sporadic—but Not Variant—Creutzfeldt-Jakob Disease Is Associated with Polymorphisms Upstream of PRNP Exon 1
Large-Scale Deletions and SMADIP1 Truncating Mutations in Syndromic Hirschsprung Disease with Involvement of Midline Structures
Mutations in the Fukutin-Related Protein Gene (FKRP) Cause a Form of Congenital Muscular Dystrophy with Secondary Laminin α2 Deficiency and Abnormal Glycosylation of α-Dystroglycan
The R22X Mutation of the SDHD Gene in Hereditary Paraganglioma Abolishes the Enzymatic Activity of Complex II in the Mitochondrial Respiratory Chain and Activates the Hypoxia Pathway
Huntington Disease Phenocopy Is a Familial Prion Disease
Polysubstance Abuse–Vulnerability Genes: Genome Scans for Association, Using 1,004 Subjects and 1,494 Single-Nucleotide Polymorphisms
The Origin of Abnormalities in Recurrent Aneuploidy/Polyploidy
A Missense Mutation in a Highly Conserved Region of CASQ2 Is Associated with Autosomal Recessive Catecholamine-Induced Polymorphic Ventricular Tachycardia in Bedouin Families from Israel Bedouin Families from Israel
Genomewide Multipoint Linkage Analysis of Seven Extended Palauan Pedigrees with Schizophrenia, by a Markov-Chain Monte Carlo Method
Monogenic Dyslipidemias: Window on Determinants of Plasma Lipoprotein Metabolism
Evaluation of Linkage and Association of HPC2/ELAC2 in Patients with Familial or Sporadic Prostate Cancer
Cerebro-Oculo-Facio-Skeletal Syndrome with a Nucleotide Excision–Repair Defect and a Mutated XPD Gene, with Prenatal Diagnosis in a Triplet Pregnancy
Linkage and Association Studies of Prostate Cancer Susceptibility: Evidence for Linkage at 8p22-23
A New Locus for Autosomal Dominant Familial Exudative Vitreoretinopathy Maps to Chromosome 11p12-13
Mutations of MLC1 (KIAA0027), Encoding a Putative Membrane Protein, Cause Megalencephalic Leukoencephalopathy with Subcortical Cysts
The Importance of Genealogy in Determining Genetic Associations with Complex Traits
Genetics of Schizophrenia and the New Millennium: Progress and Pitfalls
Linkage Analysis of a Complex Pedigree with Severe Bipolar Disorder, Using a Markov Chain Monte Carlo Method
Genetics of Event-Related Brain Potentials in Response to a Semantic Priming Paradigm in Families with a History of Alcoholism
A Survey of Affected-Sibship Statistics for Nonparametric Linkage Analysis
At the Speed of Sound: Gene Discovery in the Auditory System
Friedreich Ataxia: From GAA Triplet–Repeat Expansion to Frataxin Deficiency
Factor H Mutations in Hemolytic Uremic Syndrome Cluster in Exons 18–20, a Domain Important for Host Cell Recognition
Genomewide Screen and Identification of Gene-Gene Interactions for Asthma-Susceptibility Loci in Three U.S. Populations: Collaborative Study on the Genetics of Asthma
Genomewide Search in Familial Paget Disease of Bone Shows Evidence of Genetic Heterogeneity with Candidate Loci on Chromosomes 2q36, 10p13, and 5q35
A Progressive Autosomal Recessive Cataract Locus Maps to Chromosome 9q13-q22
Spectrum of Perforin Gene Mutations in Familial Hemophagocytic Lymphohistiocytosis
Mapping of Autosomal Dominant Osteopetrosis Type II (Albers-Schönberg Disease) to Chromosome 16p13.3
Efficient Multipoint Linkage Analysis through Reduction of Inheritance Space
Mutations of the Protocadherin Gene PCDH15 Cause Usher Syndrome Type 1F
Transmission/Disequilibrium Test Meets Measured Haplotype Analysis: Family-Based Association Analysis Guided by Evolution of Haplotypes
Announcements11. Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Uni
Quantitative-Trait-Locus Analysis of Body-Mass Index and of Stature, by Combined Analysis of Genome Scans of Five Finnish Study Groups
Erratum
Reply to Kosaki et al.
Null RPGRIP1 Alleles in Patients with Leber Congenital Amaurosis
Mutations in a Novel Gene with Transmembrane Domains Underlie Usher Syndrome Type 3
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The Silver Syndrome Variant of Hereditary Spastic Paraplegia Maps to Chromosome 11q12-q14, with Evidence for Genetic Heterogeneity within This Subtype
Multifactor-Dimensionality Reduction Reveals High-Order Interactions among Estrogen-Metabolism Genes in Sporadic Breast Cancer
The Tumor-Necrosis-Factor Receptor–Associated Periodic Syndrome: New Mutations in TNFRSF1A, Ancestral Origins, Genotype-Phenotype Studies, and Evidence for Further Genetic Heterogeneity of Periodic Fe
This Month in the Journal
Mitochondria and the Quality of Human Gametes
Linkage Disequilibrium in Humans: Models and Data
p63 Gene Mutations in EEC Syndrome, Limb-Mammary Syndrome, and Isolated Split Hand–Split Foot Malformation Suggest a Genotype-Phenotype Correlation
Birt-Hogg-Dubé Syndrome, a Genodermatosis Associated with Spontaneous Pneumothorax and Kidney Neoplasia, Maps to Chromosome 17p11.2
Mutations in CGI-58, the Gene Encoding a New Protein of the Esterase/Lipase/Thioesterase Subfamily, in Chanarin-Dorfman Syndrome
Ancestral Origins of the Machado-Joseph Disease Mutation: A Worldwide Haplotype Study
HPC2 Variants and Screen-Detected Prostate Cancer
Variant 1859G→A (Arg620Gln) of the “Hairless” Gene: Absence of Association with Papular Atrichia or Androgenetic Alopecia
After BRCA1 and BRCA2—What Next? Multifactorial Segregation Analyses of Three-Generation, Population-Based Australian Families Affected by Female Breast Cancer
Multipoint Linkage-Disequilibrium–Mapping Approach Based on the Case-Parent Trio Design
The Phylogeography of Brazilian Y-Chromosome Lineages
Announcements
Axonemal Dynein Intermediate-Chain Gene (DNAI1) Mutations Result in Situs Inversus and Primary Ciliary Dyskinesia (Kartagener Syndrome)
Disorders of Peroxisome Biogenesis Due to Mutations in PEX1: Phenotypes and PEX1 Protein Levels
Mutation Analysis of the Entire PKD1 Gene: Genetic and Diagnostic Implications
Identification and Functional Consequences of a New Mutation (E155G) in the Gene for GCAP1 That Causes Autosomal Dominant Cone Dystrophy
Complexity and Power in Case-Control Association Studies
A Phase 1/2 Clinical Trial of Enzyme Replacement in Fabry Disease: Pharmacokinetic, Substrate Clearance, and Safety Studies
Three Families with Autosomal Dominant Nephrogenic Diabetes Insipidus Caused by Aquaporin-2 Mutations in the C-Terminus
A Spectrum of ABCC6 Mutations Is Responsible for Pseudoxanthoma Elasticum
Erratum
This Month in the Journal
DFNA25, a Novel Locus for Dominant Nonsyndromic Hereditary Hearing Impairment, Maps to 12q21-24
Gene Preference in Maple Syrup Urine Disease
Arginine:Glycine Amidinotransferase Deficiency: The Third Inborn Error of Creatine Metabolism in Humans
A New Locus for Autosomal Dominant Dilated Cardiomyopathy Identified on Chromosome 6q12-q16
Periaxin Mutations Cause Recessive Dejerine-Sottas Neuropathy
Homozygosity Mapping of Portuguese and Japanese Forms of Ataxia-Oculomotor Apraxia to 9p13, and Evidence for Genetic Heterogeneity
Cloning of Dimethylglycine Dehydrogenase and a New Human Inborn Error of Metabolism, Dimethylglycine Dehydrogenase Deficiency
High-Resolution Analysis of Human Y-Chromosome Variation Shows a Sharp Discontinuity and Limited Gene Flow between Northwestern Africa and the Iberian Peninsula
The Presence of Mitochondrial Haplogroup X in Altaians from South Siberia
A Common Ancestral Origin of the Frequent and Widespread 2299delG USH2A Mutation
A Quantitative-Trait Analysis of Human Plasma–Dopamine β-Hydroxylase Activity: Evidence for a Major Functional Polymorphism at the DBH Locus
Niemann-Pick C1 Disease: Correlations between NPC1 Mutations, Levels of NPC1 Protein, and Phenotypes Emphasize the Functional Significance of the Putative Sterol-Sensing Domain and of the Cysteine-Ric
Employment Opportunities; Call for Patients
Hereditary Nonpolyposis Colorectal Cancer in 95 Families: Differences and Similarities between Mutation-Positive and Mutation-Negative Kindreds
Y-Chromosome Lineages Trace Diffusion of People and Languages in Southwestern Asia
The Ising Model in Physics and Statistical Genetics
Localization of a Gene (MCUL1) for Multiple Cutaneous Leiomyomata and Uterine Fibroids to Chromosome 1q42.3-q43
Homocysteine Metabolism in Children with Down Syndrome: In Vitro Modulation
Neuronal Sodium-Channel α1-Subunit Mutations in Generalized Epilepsy with Febrile Seizures Plus
Evaluation of Candidate Genes in Case-Control Studies: A Statistical Method to Account for Related Subjects
Gene Mutations in the Succinate Dehydrogenase Subunit SDHB Cause Susceptibility to Familial Pheochromocytoma and to Familial Paraganglioma
The Autism Genetic Resource Exchange: A Resource for the Study of Autism and Related Neuropsychiatric Conditions
A Genomewide Screen for Autism Susceptibility Loci
Evidence of Linkage with HLA-DR in DRB1*15-Negative Families with Multiple Sclerosis
Comparisons of Two Methods for Haplotype Reconstruction and Haplotype Frequency Estimation from Population Data
Assessment of Parent-of-Origin Effects in Linkage Analysis of Quantitative Traits
Disruption of the Bipartite Imprinting Center in a Family with Angelman Syndrome
Erratum
Linkage of Benign Familial Infantile Convulsions to Chromosome 16p12-q12 Suggests Allelism to the Infantile Convulsions and Choreoathetosis Syndrome
Announcements11Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Unive
Genetics and Public Health in the 20th Century.
Survey of Somatic Mutations in Tuberous Sclerosis Complex (TSC) Hamartomas Suggests Different Genetic Mechanisms for Pathogenesis of TSC Lesions
The 28-kb Deletion Spanning D15S63 Is a Polymorphic Variant in the Ashkenazi Jewish Population Ashkenazi Jewish
Model-Free Linkage Analysis with Covariates Confirms Linkage of Prostate Cancer to Chromosomes 1 and 4
Random Intracellular Drift Explains the Clonal Expansion of Mitochondrial DNA Mutations with Age
The Power to Detect Linkage Disequilibrium with Quantitative Traits in Selected Samples
Genomewide Linkage Analysis of Stature in Multiple Populations Reveals Several Regions with Evidence of Linkage to Adult Height
Primate DAX1, SRY, and SOX9: Evolutionary Stratification of Sex-Determination Pathway
This Month in the
A Genetic Factor for Age-Related Cataract: Identification and Characterization of a Novel Galactokinase Variant, “Osaka,” in Asians Asians
Reporting of Linkage Results
A Mutation in the Gene for the Neurotransmitter Receptor–Clustering Protein Gephyrin Causes a Novel Form of Molybdenum Cofactor Deficiency
Bone Dysplasia Sclerosteosis Results from Loss of the SOST Gene Product, a Novel Cystine Knot–Containing Protein
The Effect That Genotyping Errors Have on the Robustness of Common Linkage-Disequilibrium Measures
Estimating the Efficacy and Efficiency of Cascade Genetic Screening
The Significance of Not Finding a Gene
A Spectrum of FOXC1 Mutations Suggests Gene Dosage as a Mechanism for Developmental Defects of the Anterior Chamber of the Eye
Phylogenetic Network for European mtDNA European mtDNA
Bilineal Disease and Trans-Heterozygotes in Autosomal Dominant Polycystic Kidney Disease
Complex HLA-DR and -DQ Interactions Confer Risk of Narcolepsy-Cataplexy in Three Ethnic Groups
Localization of a Novel Locus for Autosomal Recessive Early-Onset Parkinsonism, PARK6, on Human Chromosome 1p35-p36
CHRNB2 Is the Second Acetylcholine Receptor Subunit Associated with Autosomal Dominant Nocturnal Frontal Lobe Epilepsy*
Erratum
The Molecular Basis of 3-Methylcrotonylglycinuria, a Disorder of Leucine Catabolism
Announcements11Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Unive
A Nonsense Mutation in MSX1 Causes Witkop Syndrome
Female Patient Showing Hypohidrotic Ectodermal Dysplasia and Immunodeficiency (HED-ID)
Infantile Alexander Disease: Spectrum of GFAP Mutations and Genotype-Phenotype Correlation
A Genomewide Scan for Loci Predisposing to Type 2 Diabetes in a U.K. Population (The Diabetes UK Warren 2 Repository): Analysis of 573 Pedigrees Provides Independent Replication of a Susceptibility Lo
Alpha-B Crystallin Gene (CRYAB) Mutation Causes Dominant Congenital Posterior Polar Cataract in Humans
Studies of Association between the Gene for Calpain-10 and Type 2 Diabetes Mellitus in the United Kingdom
Novel TFAP2B Mutations That Cause Char Syndrome Provide a Genotype-Phenotype Correlation
Accounting for Unmeasured Population Substructure in Case-Control Studies of Genetic Association Using a Novel Latent-Class Model
Acheiropodia Is Caused by a Genomic Deletion in C7orf2, the Human Orthologue of the Lmbr1 Gene
Etiological Point Mutations in the Hereditary Multiple Exostoses Gene EXT1: A Functional Analysis of Heparan Sulfate Polymerase Activity
Genome Diversity: Applications in Human Population Genetics.
This Month in the Journal
Announcements11. Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Uni
A Duplication in Chromosome 4q35 Is Associated with Hereditary Benign Intraepithelial Dyskeratosis
Use of Closely Related Affected Individuals for the Genetic Study of Complex Diseases in Founder Populations
BMPR2 Haploinsufficiency as the Inherited Molecular Mechanism for Primary Pulmonary Hypertension
This Month in the Journal
Announcements11 Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Univ
Effects of 5′ Regulatory-Region Polymorphisms on Paraoxonase-Gene (PON1) Expression
A Novel Syndrome Affecting Multiple Mitochondrial Functions, Located by Microcell-Mediated Transfer to Chromosome 2p14-2p13
Localization of the Gene for Distal Hereditary Motor Neuronopathy VII (dHMN-VII) to Chromosome 2q14
Association between a Single-Nucleotide Polymorphism in the Promoter of the Human Interleukin-3 Gene and Rheumatoid Arthritis in Japanese Patients, and Maximum-Likelihood Estimation of Combinatorial E
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Mutations Identified by MS/MS-Based Prospective Screening of Newborns Differ from Those Observed in Patients with Clinical Symptoms: Identification and Chara
CNGA3 Mutations in Hereditary Cone Photoreceptor Disorders
Up-Regulation of WNT-4 Signaling and Dosage-Sensitive Sex Reversal in Humans
This Month in the Journal
Markers for Mapping by Admixture Linkage Disequilibrium in African American and Hispanic Populations
Linkage of Otopalatodigital Syndrome Type 2 (OPD2) to Distal Xq28: Evidence for Allelism with OPD1
This Month in the Journal
An Immune Defect Causing Dominant Chronic Mucocutaneous Candidiasis and Thyroid Disease Maps to Chromosome 2p in a Single Family
A Schizophrenia-Susceptibility Locus at 6q25, in One of the World's Largest Reported Pedigrees
Population Structure in Admixed Populations: Effect of Admixture Dynamics on the Pattern of Linkage Disequilibrium
Reply to Geigl
On Discovery, Genomes, The Society, and Society*
Point Mutations of the mtDNA Control Region in Normal and Neurodegenerative Human Brains
Random Genetic Drift Determines the Level of Mutant mtDNA in Human Primary Oocytes
Paget Disease of Bone: Mapping of Two Loci at 5q35-qter and 5q31
Erratum
Leber Congenital Amaurosis and Retinitis Pigmentosa with Coats-like Exudative Vasculopathy Are Associated with Mutations in the Crumbs Homologue 1 (CRB1) Gene
A Signal, from Human mtDNA, of Postglacial Recolonization in Europe
Erratum
Paternal Population History of East Asia: Sources, Patterns, and Microevolutionary Processes
Familial Dysautonomia Is Caused by Mutations of the IKAP Gene
Tissue-Specific Expression of a Splicing Mutation in the Gene Causes Familial Dysautonomia
A Novel SCN1A Mutation Associated with Generalized Epilepsy with Febrile Seizures Plus—and Prevalence of Variants in Patients with Epilepsy
Identification of a New Candidate Locus for Uric Acid Nephrolithiasis
Connexin Mutations in Skin Disease and Hearing Loss
The Frequency of Hereditary Defective Mismatch Repair in a Prospective Series of Unselected Colorectal Carcinomas
Lactase Haplotype Diversity in the Old World
Broad and Narrow Heritabilities of Quantitative Traits in a Founder Population
Correcting for a Potential Bias in the Pedigree Disequilibrium Test
Vacuoliting Megalencephalic Leukoencephalopathy
A New Statistical Method for Haplotype Reconstruction from Population Data
Mortality in Neurofibromatosis 1: An Analysis Using U.S. Death Certificates
Reply to Rothhammer and Moraga
Reply to Vasen et al.
Pseudoexon Activation as a Novel Mechanism for Disease Resulting in Atypical Growth-Hormone Insensitivity
International Collaboration Provides Convincing Linkage Replication in Complex Disease through Analysis of a Large Pooled Data Set: Crohn Disease and Chromosome 16
Patterns of Y-Chromosome Variation in South Amerindians
Conflicting Reports of Imprinting Status of Human GRB10 in Developing Brain: How Reliable Are Somatic Cell Hybrids for Predicting Allelic Origin of Expression?
Decoding Darkness: The Search for the Genetic Causes of Alzheimer’s Disease.
Seven Regions of the Genome Show Evidence of Linkage to Type 1 Diabetes in a Consensus Analysis of 767 Multiplex Families
Prevalence and Penetrance of Germline BRCA1 and BRCA2 Mutations in a Population Series of 649 Women with Ovarian Cancer
A Major Locus for Fasting Insulin Concentrations and Insulin Resistance on Chromosome 6q with Strong Pleiotropic Effects on Obesity-Related Phenotypes in Nondiabetic Mexican Americans Mexican Americans
Nemaline Myopathy Caused by Mutations in the Muscle α-Skeletal-Actin Gene
Disruption of a Novel Gene (IMMP2L) by a Breakpoint in 7q31 Associated with Tourette Syndrome
The Mitochondrial Gene Tree Comes of Age
A Genome Screen of Families with Multiple Cases of Prostate Cancer: Evidence of Genetic Heterogeneity
The Genetic Dissection of Complex Traits in a Founder Population
The Y Chromosome Pool of Jews as Part of the Genetic Landscape of the Middle East
Haploinsufficiency for One COL3A1 Allele of Type III Procollagen Results in a Phenotype Similar to the Vascular Form of Ehlers-Danlos Syndrome, Ehlers-Danlos Syndrome Type IV
Clustering of Missense Mutations in the C-Terminal Region of Factor H in Atypical Hemolytic Uremic Syndrome
Syndromic Short Stature in Patients with a Germline Mutation in the LIM Homeobox LHX4
Localization of a Susceptibility Gene for Familial Nonmedullary Thyroid Carcinoma to Chromosome 2q21
Analysis of the Prostate Cancer–Susceptibility Locus HPC20 in 172 Families Affected by Prostate Cancer
Linkage and Association Analysis of Angiotensin I–Converting Enzyme (ACE)–Gene Polymorphisms with ACE Concentration and Blood Pressure
Decrease of 3243 A→G mtDNA Mutation from Blood in MELAS Syndrome: A Longitudinal Study
Inadequate Use of Molecular Hybridization to Analyze DNA in Neanderthal Fossils
Problems in the Definition, Interpretation, and Evaluation of Genetic Heterogeneity
Origin of the Mutations in the parkin Gene in Europe: Exon Rearrangements Are Independent Recurrent Events, whereas Point Mutations May Result from Founder Effects
Sequence Variation and Linkage Disequilibrium in the Human T-Cell Receptor β (TCRB) Locus
A Genomewide Linkage-Disequilibrium Scan Localizes the Saguenay–Lac-Saint-Jean Cytochrome Oxidase Deficiency to 2p16
Reply to Mergenthaler et al.
Worldwide Genetic Analysis of the CFTR Region
Niemann-Pick C Variant Detection by Altered Sphingolipid Trafficking and Correlation with Mutations within a Specific Domain of NPC1
Reply to Zhang et al.
Mutations in the 3β-Hydroxysterol Δ24-Reductase Gene Cause Desmosterolosis, an Autosomal Recessive Disorder of Cholesterol Biosynthesis
Identification of the Gene for Oral-Facial-Digital Type I Syndrome
Announcements11Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Unive
Demethylation, Reactivation, and Destabilization of Human Fragile X Full-Mutation Alleles in Mouse Embryocarcinoma Cells
mtDNA and the Islands of the North Atlantic: Estimating the Proportions of Norse and Gaelic Ancestry
Limitations of Chromosome Classification by Multicolor Karyotyping
Genomic Divergences between Humans and Other Hominoids and the Effective Population Size of the Common Ancestor of Humans and Chimpanzees
Somatic Mosaicism in Hemophilia A: A Fairly Common Event
Familial Chordoma, a Tumor of Notochordal Remnants, Is Linked to Chromosome 7q33
A Genomewide Screen in Multiplex Rheumatoid Arthritis Families Suggests Genetic Overlap with Other Autoimmune Diseases
Transaldolase Deficiency: Liver Cirrhosis Associated with a New Inborn Error in the Pentose Phosphate Pathway
Evidence for a Susceptibility Gene for Autism on Chromosome 2 and for Genetic Heterogeneity
Identification of TSIX, Encoding an RNA Antisense to Human XIST, Reveals Differences from its Murine Counterpart: Implications for X Inactivation
Phylogenetic and Familial Estimates of Mitochondrial Substitution Rates: Study of Control Region Mutations in Deep-Rooting Pedigrees
Genetic Differentiation in South Amerindians Is Related to Environmental and Cultural Diversity: Evidence from the Y Chromosome
This Month in the Journal
Mutational Analysis in a Cohort of 224 Tuberous Sclerosis Patients Indicates Increased Severity of TSC2, Compared with TSC1, Disease in Multiple Organs
Equivalence between Haseman-Elston and Variance-Components Linkage Analyses for Sib Pairs
Nonmuscle Myosin Heavy Chain IIA Mutations Define a Spectrum of Autosomal Dominant Macrothrombocytopenias: May-Hegglin Anomaly and Fechtner, Sebastian, Epstein, and Alport-Like Syndromes
Fragile-X Carrier Screening and the Prevalence of Premutation and Full-Mutation Carriers in Israel
Homozygosity Mapping Places the Acrodermatitis Enteropathica Gene on Chromosomal Region 8q24.3
Benign Familial Infantile Convulsions: Mapping of a Novel Locus on Chromosome 2q24 and Evidence for Genetic Heterogeneity
Complete Loss of P/Q Calcium Channel Activity Caused by a CACNA1A Missense Mutation Carried by Patients with Episodic Ataxia Type 2
Precise Estimation of Allele Frequencies of Single-Nucleotide Polymorphisms by a Quantitative SSCP Analysis of Pooled DNA
Analyses of the Effects That Disease-Causing Missense Mutations Have on the Structure and Function of the Winged-Helix Protein FOXC1
Genotypic and Phenotypic Spectrum in Tricho-Rhino-Phalangeal Syndrome Types I and III
A Confidence-Set Approach for Finding Tightly Linked Genomic Regions
X-Linked Creatine-Transporter Gene (SLC6A8) Defect: A New Creatine-Deficiency Syndrome
A Narrow Segment of Maternal Uniparental Disomy of Chromosome 7q31-qter in Silver-Russell Syndrome Delimits a Candidate Gene Region
Optimized Group Sequential Study Designs for Tests of Genetic Linkage and Association in Complex Diseases
Hermansky-Pudlak Syndrome Type 3 in Ashkenazi Jews and Other Non–Puerto Rican Patients with Hypopigmentation and Platelet Storage-Pool Deficiency Ashkenazi Jews; Non–Puerto Rican
Mutations in the Sepiapterin Reductase Gene Cause a Novel Tetrahydrobiopterin-Dependent Monoamine-Neurotransmitter Deficiency without Hyperphenylalaninemia
Use of Homozygosity Mapping to Identify a Region on Chromosome 1 Bearing a Defective Gene That Causes Autosomal Recessive Homozygous Hypercholesterolemia in Two Unrelated Families
Identification, by Homozygosity Mapping, of a Novel Locus for Autosomal Recessive Congenital Ichthyosis on Chromosome 17p, and Evidence for Further Genetic Heterogeneity
This Month in the Journal
Quantitative Similarity-Based Association Tests Using Population Samples
Extent and Distribution of Linkage Disequilibrium in Three Genomic Regions
A Second Gene for Otosclerosis, OTSC2, Maps to Chromosome 7q34-36
Melanocortin-1 Receptor Gene Variants Determine the Risk of Nonmelanoma Skin Cancer Independently of Fair Skin and Red Hair
A Recurrent RNA-Splicing Mutation in the SEDL Gene Causes X-Linked Spondyloepiphyseal Dysplasia Tarda
Lower-Than-Expected Linkage Disequilibrium between Tightly Linked Markers in Humans Suggests a Role for Gene Conversion
AT-Rich Palindromes Mediate the Constitutional t(11;22) Translocation
Genetics and Analysis of Quantitative Traits
Frequency of XY Sperm Increases with Age in Fathers of Boys with Klinefelter Syndrome
Excess of Twins among Affected Sibling Pairs with Autism: Implications for the Etiology of Autism
Are Rare Variants Responsible for Susceptibility to Complex Diseases?
Genomewide-Linkage and Haplotype-Association Studies Map Intracranial Aneurysm to Chromosome 7q11
Patterns of Ancestral Human Diversity: An Analysis of Alu-Insertion and Restriction-Site Polymorphisms
Mutations in the Hepatocyte Nuclear Factor-1β Gene Are Associated with Familial Hypoplastic Glomerulocystic Kidney Disease
Hereditary Vascular Retinopathy, Cerebroretinal Vasculopathy, and Hereditary Endotheliopathy with Retinopathy, Nephropathy, and Stroke Map to a Single Locus on Chromosome 3p21.1-p21.3
MYO6, the Human Homologue of the Gene Responsible for Deafness in Snell’s Waltzer Mice, Is Mutated in Autosomal Dominant Nonsyndromic Hearing Loss
Germline Mutations in BMPR1A/ALK3 Cause a Subset of Cases of Juvenile Polyposis Syndrome and of Cowden and Bannayan-Riley-Ruvalcaba Syndromes*
Paternal Uniparental Isodisomy of Chromosome 20q—and the Resulting Changes in GNAS1 Methylation—as a Plausible Cause of Pseudohypoparathyroidism
PARK7, a Novel Locus for Autosomal Recessive Early-Onset Parkinsonism, on Chromosome 1p36
This Month in the Journal
Announcements11Announcements are published free of charge for members of The American Society of Human Genetics (ASHG). Please mail announcements to The American Journal of Human Genetics, Emory Unive
Genetic and Mutational Analyses of a Large Multiethnic Bardet-Biedl Cohort Reveal a Minor Involvement of BBS6 and Delineate the Critical Intervals of Other Loci
Compound Heterozygosity for a Recurrent 16.5-kb Alu-Mediated Deletion Mutation and Single-Base-Pair Substitutions in the ABCC6 Gene Results in Pseudoxanthoma Elasticum
Usher Syndrome 1D and Nonsyndromic Autosomal Recessive Deafness DFNB12 Are Caused by Allelic Mutations of the Novel Cadherin-Like Gene CDH23
MECP2 Mutations in Sporadic Cases of Rett Syndrome Are Almost Exclusively of Paternal Origin
Two Genes That Map to the STSL Locus Cause Sitosterolemia: Genomic Structure and Spectrum of Mutations Involving Sterolin-1 and Sterolin-2, Encoded by ABCG5 and ABCG8, Respectively
Schizophrenia and Affective Disorders—Cosegregation with a Translocation at Chromosome 1q42 That Directly Disrupts Brain-Expressed Genes: Clinical and P300 Findings in a Family
Segregation Analyses of 1,476 Population-Based Australian Families Affected by Prostate Cancer
Single-Amino-Acid Deletion in the RYR1 Gene, Associated with Malignant Hyperthermia Susceptibility and Unusual Contraction Phenotype
MC1R Genotype Modifies Risk of Melanoma in Families Segregating CDKN2A Mutations
Resetting our Educational Sights: Unconstructing the Public's Dreams and Nightmares of the Genetic Revolution*
Examinations of Methylenetetrahydrofolate Reductase C677T and A1298C Mutations—and In Utero Viability
Estimation of Sibling Recurrence-Risk Ratio under Single Ascertainment in Two-Child Families
Heteroplasmy of the Human mtDNA Control Region Remains Constant during Life
Large-Scale Deletion and Point Mutations of the Nuclear NDUFV1 and NDUFS1 Genes in Mitochondrial Complex I Deficiency
Reply to Wickramaratne and Hodge
Regression Models for Linkage Heterogeneity Applied to Familial Prostate Cancer
Melanocortin-1 Receptor Variant R151C Modifies Melanoma Risk in Dutch Families with Melanoma
Archaeogenetics: DNA and the Population Prehistory of Europe
Hereditary Sensory Neuropathy Type I: Haplotype Analysis Shows Founders in Southern England and Europe
Gene Conversion and Different Population Histories May Explain the Contrast between Polymorphism and Linkage Disequilibrium Levels
Variation in Cancer Risks, by Mutation Position, in BRCA2 Mutation Carriers
Wild-Type Huntingtin Reduces the Cellular Toxicity of Mutant Huntingtin In Vivo
Is Breast Cancer Part of the Tumor Spectrum of Hereditary Nonpolyposis Colorectal Cancer?
Localization of the Gene for the Intermediate Form of Charcot-Marie-Tooth to Chromosome 10q24.1-q25.1
Shwachman-Diamond Syndrome with Exocrine Pancreatic Dysfunction and Bone Marrow Failure Maps to the Centromeric Region of Chromosome 7
The Promoter of a Lysosomal Membrane Transporter Gene, CTNS, Binds Sp-1, Shares Sequences with the Promoter of an Adjacent Gene, CARKL, and Causes Cystinosis If Mutated in a Critical Region
Global Analysis of ATM Polymorphism Reveals Significant Functional Constraint
A Mutation in COL9A1 Causes Multiple Epiphyseal Dysplasia: Further Evidence for Locus Heterogeneity
A Second Locus for an Axonal Form of Autosomal Recessive Charcot-Marie-Tooth Disease Maps to Chromosome 19q13.3
Principles of Molecular Oncology
Niemann-Pick Disease Type C: Spectrum of HE1 Mutations and Genotype/Phenotype Correlations in the NPC2 Group
Genomewide Scans of Complex Human Diseases: True Linkage Is Hard to Find
Autosomal Dominant Craniometaphyseal Dysplasia Is Caused by Mutations in the Transmembrane Protein ANK
Many Roads Lead to a Broken Heart: The Genetics of Dilated Cardiomyopathy
Whole-Genome Screening in Ankylosing Spondylitis: Evidence of Non-MHC Genetic-Susceptibility Loci
An Extensive Analysis of Y-Chromosomal Microsatellite Haplotypes in Globally Dispersed Human Populations
Missense Mutations in the N-Terminal Domain of Human Phenylalanine Hydroxylase Interfere with Binding of Regulatory Phenylalanine
Functional Complementation of a Genetic Deficiency with Human Artificial Chromosomes
Fabry Disease: Preclinical Studies Demonstrate the Effectiveness of α-Galactosidase A Replacement in Enzyme-Deficient Mice
Atypical Forms of Incontinentia Pigmenti in Male Individuals Result from Mutations of a Cytosine Tract in Exon 10 of NEMO (IKK-γ)
Independent Histories of Human Y Chromosomes from Melanesia and Australia
In Southern Africa, Brown Oculocutaneous Albinism (BOCA) Maps to the OCA2 Locus on Chromosome 15q: P-Gene Mutations Identified
Recent Origin and Spread of a Common Lithuanian Mutation, G197del LDLR, Causing Familial Hypercholesterolemia: Positive Selection Is Not Always Necessary to Account for Disease Incidence among Ashkena
The D13S171 Marker, Misannotated to BRCA2, Links the AS3 Gene to Various Cancers
A Novel Form of “Central Pouchlike” Cataract, with Sutural Opacities, Maps to Chromosome 15q21-22
Measurement of Mutational Flow Implies Both a High New-Mutation Rate for Huntington Disease and Substantial Underascertainment of Late-Onset Cases
The Amyloid Precursor Protein Locus and Very-Late-Onset Alzheimer Disease
Dominant Intermediate Charcot-Marie-Tooth Neuropathy Maps to Chromosome 19p12-p13.2
Genomewide Genetic Linkage Analysis Confirms the Presence of Susceptibility Loci for Schizophrenia, on Chromosomes 1q32.2, 5q33.2, and 8p21-22 and Provides Support for Linkage to Schizophrenia, on Chr
Identification and Expression Analysis of Spastin Gene Mutations in Hereditary Spastic Paraplegia
A Predominantly Indigenous Paternal Heritage for the Austronesian-Speaking Peoples of Insular Southeast Asia and Oceania
Olfactory Receptor–Gene Clusters, Genomic-Inversion Polymorphisms, and Common Chromosome Rearrangements
The Molecular Basis of X-Linked Spondyloepiphyseal Dysplasia Tarda
De Novo Mutations in the Sodium-Channel Gene SCN1A Cause Severe Myoclonic Epilepsy of Infancy
Unusual Haplotypic Structure of IL8, a Susceptibility Locus for a Common Respiratory Virus
A Recessive Contiguous Gene Deletion of Chromosome 2p16 Associated with Cystinuria and a Mitochondrial Disease
Mutations in the Human Orthologue of the Mouse underwhite Gene (uw) Underlie a New Form of Oculocutaneous Albinism, OCA4
A Mutation, in the Iron-Responsive Element of H Ferritin mRNA, Causing Autosomal Dominant Iron Overload
A Genome Scan for Renal Function among Hypertensives: the HyperGEN Study
A Transmission/Disequilibrium Test That Allows for Genotyping Errors in the Analysis of Single-Nucleotide Polymorphism Data
Molecular Characterization and Gene Content of Breakpoint Boundaries in Patients with Neurofibromatosis Type 1 with 17q11.2 Microdeletions
Maple Syrup Urine Disease: Identification and Carrier-Frequency Determination of a Novel Founder Mutation in the Ashkenazi Jewish Population Ashkenazi Jewish population
Reply to Volcik et al.
Analysis of European mtDNAs for Recombination European mtDNAs
Dominant Inheritance of Sialuria, an Inborn Error of Feedback Inhibition
Genetic and Physical Mapping of the Locus for Autosomal Dominant Renal Fanconi Syndrome, on Chromosome 15q15.3
The Primary Erythermalgia–Susceptibility Gene Is Located on Chromosome 2q31-32
High Residual Activity of PMM2 in Patients’ Fibroblasts: Possible Pitfall in the Diagnosis of CDG-Ia (Phosphomannomutase Deficiency)
A Novel Frameshift Mutation in Exon 23 of ATP7A (MNK) Results in Occipital Horn Syndrome and Not in Menkes Disease
Characterization of Mutations in the CPO Gene in British Patients Demonstrates Absence of Genotype-Phenotype Correlation and Identifies Relationship between Hereditary Coproporphyria and Harderoporphy British patients
Erratum
This Month in the Journal
Founder Mutations of BRCA1 and BRCA2 in North American Families of Polish Origin That Are Affected with Breast Cancer
Transformation of Sib-Pair Values for the Haseman-Elston Method
Cracking the Genome: Inside the Race to Unlock Human DNA
Introductory Speech for F. Clarke Fraser*
Impaired Heme Binding and Aggregation of Mutant Cystathionine β-Synthase Subunits in Homocystinuria
The Human Aminophospholipid-Transporting ATPase Gene ATP10C Maps Adjacent to UBE3A and Exhibits Similar Imprinted Expression
High-Resolution Multipoint Linkage-Disequilibrium Mapping in the Context of a Human Genome Sequence
A Genomewide Screen for Autism: Strong Evidence for Linkage to Chromosomes 2q, 7q, and 16p
Maternal Folate Polymorphisms and the Etiology of Human Nondisjunction
Advances in Twin and Sib-Pair Analysis.
Disruption of the ProSAP2 Gene in a t(12;22)(q24.1;q13.3) Is Associated with the 22q13.3 Deletion Syndrome