Clinical Genetics - 2024

209 articles | Last updated: 2025-12-03 14:12:56
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A Variable Clinical Presentation of Hemoglobin City of Hope Ashkenazi Jews (70.4%)
Identification of a Novel <scp><i>FLT4</i></scp> c.<scp>3028A</scp>&gt;C Variant Associated With Milroy Disease
Concomitant Upd(14)mat and Trisomy 14 Mosaicism in a Newborn Detected by Whole Genome Sequencing
Clinical and Genetic Analysis of Digenic Muscular Dystrophy due to <i>SRPK3</i> and <i>TTN</i> Variants in Two Siblings
Diagnostic Use of Genome Sequencing in Patients With 11p15.5 Imprinting Disorder Features: A Pilot Study
Homozygous <scp>ASTN1</scp> Nonsense Variant Linked to Epileptic Encephalopathy: A Detailed Report With Unique Clinical Presentation
Expanding the Clinical and Mutational Spectrum of Biallelic <scp><i>POC1A</i></scp> Variants: Characterization of Four Patients and a Comprehensive Review of <scp>POC1A</scp>‐Related Phenotypes
Unveiling New Clinical and Genetic Insights in Ultra‐Rare Intellectual Disability Phenotypes: A Study of a Turkish Cohort
Mismatch Repair Proficient Colorectal Adenocarcinoma in Two Patients With Lynch Syndrome
A Novel De Novo Missense Variant in Netrin‐1 (<i>NTN1</i>) Associated With Chorioretinal Coloboma, Sensorineural Hearing Loss and Polydactyly
Genotype–Phenotype Correlations in <i>SYNGAP1</i>‐Related Mental Retardation Type 5
Split Hand‐Foot Malformations—Unveiling Unique Molecular Diagnosis From a Brazilian Cohort Brazilian
Unraveling the Genetic Basis of Congenital Limb Anomalies in Eight Families
Biallelic Variants in <scp><i>LRRC45</i></scp> Impair Ciliogenesis and Cause a Severe Neurological Disorder
A Novel Homozygous Synonymous Variant in <scp><i>CCDC134</i></scp> as a Cause of Osteogenesis Imperfecta Type <scp>XXII</scp> Chinese; Chinese population
Comprehensive Analysis of <scp><i>TEK</i></scp> Variants in Patients With Vascular Malformations
<scp><i>WDFY3</i></scp> Haploinsufficiency Is Associated With Autosomal Dominant Neurodevelopmental Disorders and Macrocephaly
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Unique Genetic Profiles in Hypertrophic Cardiomyopathy Patients From São Miguel Island (Azores, Portugal)
Skipping of Exon 20 in <i>EP300</i>: A Novel Variant Linked to Rubinstein–Taybi Syndrome With Atypical and Severe Clinical Manifestations
Mainstreaming Cancer Genomic Testing: A Scoping Review of the Acceptability, Efficacy, and Impact
Exploring the Cognitive and Behavioral Aspects of Shprintzen‐Goldberg Syndrome; a Novel Cohort and Literature Review
The p.(<scp>Gly111Arg</scp>) <i>ABCC8</i> Variant: A Founder Mutation Causing Congenital Hyperinsulinism in the Indian Agarwal Community Indian Agarwal Community
Mobile Element Insertion in the <i>APOB</i> Exon 3 Coding Sequence: A New Challenge in Hypobetalipoproteinemia Diagnosis
Rare Causes and Differential Diagnosis in Patients With Silver‐Russell Syndrome
Novel <scp><i>BRAT1</i></scp> Deep Intronic Variant Affects Splicing Regulatory Elements Causing Cerebellar Hypoplasia Syndrome: Genotypic and Phenotypic Expansion
Skeletal Phenotype in Mulibrey Nanism, A Monogenic Skeletal Dysplasia With Fibrous Dysplasia
Genetic, Clinical, and Biochemical Characterization of a Large Cohort of Palestinian Patients With Fanconi‐Bickel Syndrome
<scp><i>BRCC3</i></scp>‐Associated Syndromic Moyamoya Angiopathy Diagnosed Through Clinical <scp>RNA</scp> Sequencing
<scp><i>AMOTL1</i></scp>‐Associated Multiple Congenital Anomalies (Craniofaciocardiohepatic Syndrome, <scp>CFCHS</scp>): A Novel Clinical Spectrum Including Craniofacial, Heart and Liver Abnormalities
A Novel Compound Heterozygous Genotype of the <i>WDR73</i> Gene Associated With a Psychomotor Retardation Syndrome Without Cerebellar Atrophy and Other <scp>CNS</scp> Structural Abnormalities
Identification of a Rare Branch Point Variant in the <i>SMS</i> Gene in a Large Family With a Severe Form of Snyder–Robinson Syndrome
Genetic Analysis of Heterotaxy in a Consanguineous Cohort
The Excess of Carriers in Rare Disorders Suggests a Nonpathogenic Effect for Most Variants of Uncertain Significance
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<scp><i>PERCC1</i></scp>‐Related Congenital Enteropathy
Recognisable Neuroradiological Findings in Five Neurogenetic Disorders
Identification of Two Novel Missense Variants in <i>BNC1</i> in Han Chinese Patients With Non‐syndromic Premature Ovarian Insufficiency Han Chinese
Genetic Variants Supporting the Diagnosis of Primary Ciliary Dyskinesia in Japan Japanese; general Japanese population; ethnic differences; global data
Biallelic <scp><i>PIGM</i></scp> Coding Variant Causes Intractable Epilepsy and Intellectual Disability Without Thrombotic Events
A Frameshift Variant in <i>ANKRD24</i> Implicates Its Role in Human Non‐Syndromic Hearing Loss
Modified Rules for Classification of Variants Associated With Disorders of Somatic Mosaicism
<scp>RNU4‐2</scp>‐Related Neurodevelopmental Disorder Is Associated With a Recognisable Facial Gestalt
Utility of Optical Genome Mapping in Repeat Disorders
A Novel <i>PTPRQ</i> c.3697del Variant Causes Autosomal Dominant Progressive Hearing Loss in Both Humans and Mice Chinese Han
<scp>ME2</scp> Deficiency Is Associated With Recessive Neurodevelopmental Disorder
<scp>PathVar</scp>: A Customisable <scp>NGS</scp> Variant Calling Algorithm Implicates Novel Candidate Genes and Pathways in Hemiplegic Migraine
Exome Sequencing of Consanguineous Pashtun Families With Familial Epilepsy Reveals Causative and Candidate Variants in <i>TSEN54</i>, <i>MOCS2</i>, and <i>OPHN1</i> Pashtun population; Pashtun community; ethnic groups
The p. <scp>S178L</scp> mutation in <i>Tbc1d24</i> disrupts endosome‐mediated synaptic vesicle trafficking of cochlear hair cells and leads to hearing impairment in mice
Hidden Aberrant Transcripts in <scp><i>TTC37</i></scp> Cause Trichohepatoenteric Syndrome
Chromosome 8p Syndromes Clinical Presentation and Management Guidelines
A Strong Candidate Gene for Nonsyndromic Intellectual Disability Phenotype: <i>SGSM3</i> Ashkenazi Jews; different populations
A Novel Homozygous Missense <scp>ZP1</scp> Variant Result in Human Female Empty Follicle Syndrome
Consolidating the Role of Mutated <i>ATP2B2</i> in Neurodevelopmental and Cerebellar Pathologies
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Second Case of Gonadal Mosaicism and a Novel Nonsense <scp>NR2F1</scp> Gene Variant as the Cause of Bosch–Boonstra–Schaaf Optic Atrophy Syndrome
Novel <i>KIF26A</i> variants associated with pediatric intestinal pseudo‐obstruction (<scp>PIPO</scp>) and brain developmental defects
Review of susceptibility genes in developmental dysplasia of the hip: A comprehensive examination of candidate genes and pathways
Research progress of the relationship between phosphoprotein phosphatases (<scp>PPPs</scp>) and neurodevelopmental disorders
Genetics of anomalies of the kidney and urinary tract with congenital heart disease: A review
Knowledge and perceptions about fragile X syndrome and fragile X‐premutation‐associated conditions among medical doctors in Nigeria
Compound heterozygosity for two variants in <i>BMP5</i> in human skeletal dysostosis with atrioventricular septal defect
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Multiple congenital anomalies in two fetuses with glutathione‐synthetase deficit (<i>GSS</i>)
Dissecting <scp><i>CASK</i></scp>: Novel splice site variant associated with male <scp>MICPCH</scp> phenotype
Childhood glaucoma: Implications for genetic counselling
Expanding the genetic landscape of Usher syndrome type <scp>IV</scp> caused by pathogenic <i>ARSG</i> variants
Non‐immune hydrops fetalis is associated with bi‐allelic pathogenic variants in the <scp>MYB</scp> Binding Protein 1a (<scp><i>MYBBP1A</i></scp>) gene
Expanding the phenotypes of <i>ABL1</i> deficiency syndromes: When mutations in different isoforms Lead to different diseases
Novel <i>PLEC</i> variants associated with infantile cholestasis
Clinical characteristics, longitudinal adaptive functioning, and association with electroencephalogram activity in <i>PPP2R5D</i>‐related neurodevelopmental disorder
<scp>SCYL2</scp>‐related autosomal recessive neurodevelopmental disorders: Arthrogryposis multiplex congenita‐4 and beyond?
In memory of Ludwine Messiaen, Ph.D. (1956–2024)
Active spread of β‐thalassemia beyond the thalassemia belt: A study on a Russian population Caucasus region; resident subpopulation; ethnic groups; resident populations
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The clinical and genetic spectrum of mitochondrial diseases in China: A multicenter retrospective cross‐sectional study
Genetic landscape of hearing loss in prelingual deaf patients of eastern <scp>Iran</scp>: Insights from exome sequencing analysis
Exploring the role of non‐canonical splice site variants in aberrant splicing associated with reproductive genetic disorders
Clinicogenetic characterization of cerebrotendinous xanthomatosis in Brazil
Identification of copy‐number variants in patients with overgrowth disorders ethnic group; Spanish
Epilepsy due to potential loss of <i>ATP6V1B2</i> function with mechanistic insight by a <i>Drosophila</i> Vha55 model
Correction to “<scp>SAM</scp> domain variants of <scp>EPHB4</scp> associated with aberrant signaling are linked to lymphatic‐related fetal hydrops and facial dysmorphology”
Molecular genetics in 1991 arrhythmia probands and 2782 relatives in Norway: Results from 17 years of genetic testing in a national laboratory Norwegian population; Norway; Norwegian
A de novo novel variant in the <i>MT‐TD</i> gene is associated with prominent extra‐neurologic manifestations
Toward <scp>3D</scp> facial analysis for recognizing Mendelian causes of autism spectrum disorder
The c.‐<scp>265G</scp>&gt;A <i>GLA</i> gene promoter variant causes Fabry disease: The hidden culprit identified
Prenatal diagnosis of a 15q24.1 microdeletion in a fetus with cerebral and urogenital abnormalities
Clinical and molecular characterization of limb‐girdle muscular dystrophy <scp>2G</scp>/<scp>R7</scp> in a large cohort of Brazilian patients Eastern European; Brazilian; Chinese; Bulgarian; Asian populations
Compound heterozygous variants in <scp><i>SLC45A1</i></scp> might cause syndromic intellectual disability by localization failure and activity attenuation in cells
Genetics of 67 patients of suspected primary ciliary dyskinesia from India
A rare form of <scp>LIM</scp> domain‐binding protein 3 (<scp><i>LDB3</i></scp>) mutation causes hypertrophic cardiomyopathy and myofibrillar myopathy type 4
Streamlined two‐step fragment analysis <scp>PCR</scp> and exome sequencing of <scp>RFC1</scp> for diagnostic testing of suspected <scp>CANVAS</scp> patients
The phenotypic and genotypic spectrum of individuals with mono‐ or biallelic <i>ANK3</i> variants
Whole genome sequencing identifies a homozygous splicing variant in <i>TDRKH</i> segregating with non‐obstructive azoospermia in an Iranian family Iranian; North African
Low‐intensity noise exposure takes an essential part in the mechanism of late‐onset hereditary hearing loss caused by Abcc1 mutation
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<scp>HDR</scp> syndrome: Large cohort and systematic review
<scp>SETBP1</scp> haploinsufficiency and related disorders clinical and neurobehavioral phenotype study
Featuring <i>BRCA1</i> and <i>BRCA2</i> germline mutational landscape from <scp>Asturias</scp> (<scp>North Spain</scp>)
Differences in manifestations of epilepsy and developmental delay in <scp>PURA</scp> syndrome and 5q31 microdeletions
A homozygous <i>ARMC3</i> splicing variant causes asthenozoospermia and flagellar disorganization in a consanguineous family
Unraveling <i>GRIA1</i> neurodevelopmental disorders: Lessons learned from the p.(<scp>Ala636Thr</scp>) variant
New kinase‐deficient <scp>PAK2</scp> variants associated with Knobloch syndrome type 2
<scp>EZH2</scp>‐associated tumor malignancy: A prominent target for cancer treatment
Short stature and dysmorphic features in Asian Indian siblings with <scp><i>DAAM2</i></scp>‐associated steroid‐resistant nephrotic syndrome: Expansion of the phenotypic spectrum or a blended phenotype Asian Indian
Re‐analysis of whole genome sequencing ends a diagnostic odyssey: Case report of an <i>RNU4‐2</i> related neurodevelopmental disorder
Non‐dilated left ventricular cardiomyopathy with arrhythmias is commonly caused by the nonsense variant <scp><i>DSP:</i>c.3793G</scp>&gt;T in Slovenian patients Slovenian ("Slovenian patients", "Slovenian probands", "Slovenian-specific")
Expanding the spectrum of phenotypes for <i>MPDZ</i>: Report of four unrelated families and review of the literature
Non‐syndromic retinal dystrophy associated with biallelic variation of <scp><i>SUMF1</i></scp> and reduced leukocyte sulfatase activity
Bi‐allelic variants in <scp>MYH3</scp> cause recessively‐inherited arthrogryposis
A novel homozygous <i>FAM92A</i> gene (<i>CIBAR1</i>) variant further confirms its association with non‐syndromic postaxial polydactyly type <scp>A9</scp> (<scp>PAPA9</scp>)
Novel molecular, structural and clinical findings in an Italian cohort of congenital cataract
<i>SERPINA11</i> related novel serpinopathy – A perinatal lethal disorder
The impact of the European Society of Cardiology guidelines and whole exome sequencing on genetic testing in hereditary cardiac diseases
B‐cell immune deficiency in twin sisters expands the phenotype of <scp>MOPDI</scp>
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The significance of carrying <i>MEFV</i> variants in symptomatic and asymptomatic individuals
Novel biallelic <i>PISD</i> missense variants cause spondyloepimetaphyseal dysplasia with disproportionate short stature and fragmented mitochondrial morphology
Biallelic <i>FANCA</i> variants detected in sisters with isolated premature ovarian insufficiency
Copy number variants at 4q31.3 affecting the regulatory region of <scp><i>FBXW7</i></scp> associated with neurodevelopmental delay
Clinical and genetic investigation of 14 families with various forms of short stature syndromes Pakistani origin; Pakistani population
Functional studies in yeast confirm the pathogenicity of a new <i>GINS3</i><scp>Meier–Gorlin</scp> syndrome variant
Inherited thrombocytopenia associated with a variant in the <scp>FLI1</scp> binding site in the 5′ <scp>UTR</scp> of <i>ANKRD26</i>
Identification of seven variants in the col4a1 gene that alter <scp>RNA</scp> splicing by minigene assay
Genetic profile of Brazilian patients with <scp>LAMA2</scp>‐related dystrophies Latin American population; Brazilian patients
High prevalence of copy number variations in the Japanese participants with suspected <scp>MODY</scp> Japanese
A fine‐scale genetic map of the Japanese population
<i>FGF12</i> copy number variant associated with epileptic encephalopathy
Outcomes of a universal germline screening program in a community urology practice
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Abnormalities in pharyngeal arch‐derived structures in SATB2‐associated syndrome
Best practice guidelines on genetic diagnostics of facioscapulohumeral muscular dystrophy: Update of the 2012 guidelines FSHD European Trial Network
Heritability of cancers in Japanese population: Estimation from recent cohort data European descent; Japanese population; Nordic population; ethnicity
Syndromic craniosynostosis caused by a novel missense variant in <i>MAP4K4</i>: Expanding the genotype–phenotype relationship in <scp>RASopathies</scp> Korean boy
Usmani‐Riazuddin syndrome can have a recognizable phenotype: Report of a novel <i>AP1G1</i> variant
Pediatric acute promyelocytic leukemia and Fanconi anemia: Case report and literature review
Severe manifestation of <scp>Rauch‐Azzarello</scp> syndrome associated with biallelic deletion of <scp><i>CTNND2</i></scp> Syrian descent
Expanding the understanding of telomere biology disorder with reports from two families harboring variants in <i>ZCCHC8</i> and <i>TERC</i>
Genotypic and phenotypic features of 39 Chinese patients with glycogen storage diseases type I, <scp>VI</scp>, and <scp>IX</scp>
Beyond the phenotype: Exploring inherited retinal diseases with targeted next‐generation sequencing in a Turkish cohort Turkish
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Simplified detection of genetic background admixture using artificial intelligence
Possible incomplete penetrance of Xq28 int22h‐1/int22h‐2 duplication
<scp><i>Kinesin family member 12</i></scp>‐related hepatopathy: A generally indolent disorder with elevated gamma‐glutamyl‐transferase activity
Shifting the landscape: Dominant C‐terminal rare missense <scp><i>FOXL2</i></scp> variants in non‐syndromic primary ovarian failure etiology
Novel copy number variations and phenotypes of infantile epileptic spasms syndrome Chinese cohort
Clinical exome sequencing uncovers genetic disorders in neonates with suspected hypoxic–ischemic encephalopathy: A retrospective analysis
Clinical feature, <i>GALC</i> variant spectrum, and genotype–phenotype correlation in Korean Krabbe disease patients: Multicenter experience over 13 years other ethnic groups
De novo start‐loss variant in <scp><i>HIRA</i></scp> in patient with <scp>DiGeorge</scp>‐like syndrome
<i>BRCA1‐associated protein 1</i>: Tumor predisposition syndrome and Kury‐Isidor syndrome, from genotype–phenotype correlation to clinical management
Regulation of <scp>N<sup>6</sup></scp>‐methyladenosine modification in erythropoiesis and thalassemia
Autozygome‐guided exome‐first study in a consanguineous cohort with early‐onset retinal disease uncovers an isolated <i>RIMS2</i> phenotype and a retina‐enriched <i>RIMS2</i> isoform
Identification of genetic causes in children with unexplained epilepsy based on trio‐whole exome sequencing
Homozygous novel truncating variant of <i>CLPP</i> associated with severe Perrault syndrome
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Genebe.net: Implementation and validation of an automatic ACMG variant pathogenicity criteria assignment
The utility of exome sequencing in diagnosing pediatric neurodevelopmental disorders in a highly consanguineous population
Reduced kinase function in two ultra‐rare <scp><i>TNNI3K</i></scp> variants in families with congenital junctional ectopic tachycardia
Cerebral dural arteriovenous fistulas in patients with <scp><i>PTEN</i></scp>‐related hamartoma tumor syndrome
Evidence for common mechanisms of pathology between <i>SHANK3</i> and other genes of <scp>Phelan‐McDermid</scp> syndrome
ASXL3‐related disorder: Molecular phenotyping and comprehensive review providing insights into disease mechanism
A founder variant expands the phenotype of <scp><i>WNT7B</i></scp>‐related <scp>PDAC</scp> syndrome
Genome‐wide association study of cardiometabolic multimorbidity in the <scp>UK</scp> Biobank European ancestry
Clinical and genetic characteristics in a Chinese cohort of complex spastic paraplegia type 4 Chinese cohort
Allelic heterogeneity in a patient with postzygotic <scp><i>MTOR</i></scp>‐related hypomelanosis of Ito with neurodevelopmental abnormalities
Genetic and phenotypic landscape of pediatric‐onset epilepsy in 142 Indian families: Counseling and therapeutic implications
Multi‐gene panel sequencing in highly consanguineous families and patients with congenital forms of skeletal dysplasias
Refining the 9q34.3 microduplication syndrome reveals mild neurodevelopmental features associated with a distinct global <scp>DNA</scp> methylation profile
Novel variant in <i>LRP6</i> associated with unusual and severe clinical presentation: Case report
Severe isolated exudative vitreoretinopathy caused by biallelic <i>FZD4</i> variants
Expanding the phenotype of <scp><i>PPP1R21</i></scp>‐related neurodevelopmental disorder
Prevalence and phenotypes associated with <i>ALPK3</i> null variants in a large French multicentric cohort: Confirming its involvement in hypertrophic cardiomyopathy
Novel phenotype associated with homozygous likely pathogenic variant in the <scp>POP1</scp> gene
Further evidence of involvement of <i>ITSN1</i> in autosomal dominant neurodevelopmental disorder
Patient perspective in perceived comparative genetic mutation risk: An exploratory review
A hemizygous loss‐of‐function variant in <scp><i>BCORL1</i></scp> is associated with male infertility and oligoasthenoteratozoospermia
Assessing the impact of psychiatric genetic counseling on psychiatric hospitalizations
The first case of a point pathogenic variant in the <i>RREB1</i> gene in Noonan‐like Rasopathy
Feasibility of whole‐exome sequencing in fine‐needle aspiration specimens of papillary thyroid microcarcinoma for the identification of novel gene mutations
Identification of a founder variant <i>AAGAB</i> c.<scp>370C</scp>&gt;T, p.<scp>Arg124Ter</scp> in patients with punctate palmoplantar keratoderma in Southern Denmark
Contribution of genetic variants in the development of familial premature coronary artery disease in a cohort of cardiac patients
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Further description of two individuals with de novo <scp>p.(Glu127Lys)</scp> missense variant in the <scp><i>ASCL1</i></scp> gene
Clinical and molecular characteristics of 26 fetuses with lethal multiple congenital contractures
Development of disease‐specific growth charts for Korean children with <scp>Beckwith–Wiedemann</scp> syndrome Korean
Genotype–phenotype correlation in <scp>Prader‐Willi</scp> syndrome: A large‐sample analysis in China
Clinical phenotypic spectrum of <scp><i>CTNNB1</i></scp> neurodevelopmental disorder
Functional characterization of variants found in Japanese patients with hereditary hemorrhagic telangiectasia
Functional characterization of inactivating <i>ABCC8</i> variants causing congenital hyperinsulinism
Hao‐Fountain syndrome: 32 novel patients reveal new insights into the clinical spectrum
The <i>FGF14</i><scp>GAA</scp> repeat expansion in Greek patients with late‐onset cerebellar ataxia and an overview of the <scp>SCA27B</scp> phenotype across populations Greek; Greek population; previously studied populations
The clinical and genetic landscape of developmental and epileptic encephalopathies in Egyptian children
Is there a dominant‐negative effect in individuals with heterozygous disease‐causing variants in <i>COL4A3/COL4A4</i>?
Further delineation of the phenotypic and metabolomic profile of <i>ALDH1L2</i>‐related neurodevelopmental disorder
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<scp><i>CCDC88C</i></scp> variants are associated with focal epilepsy and genotype–phenotype correlation
Therapeutic strategies for aberrant splicing in cancer and genetic disorders
The Phenotypic and Genotypic Spectrum of <scp><i>BRPF1</i></scp>‐Related Disorder: 29 New Patients and Literature Review
Variant Spectrum of Renal Ciliopathies in Turkish Cohort and Genotype–Phenotype Association Specifically in Autosomal Dominant Polycystic Kidney Disease Turkish cohort
The Value of <scp>ROH</scp> Metrics for Predicting Morbidity: Insights From a Large Cohort Analysis of Chromosomal Microarray ancestral origins; various origins; consanguineous populations
Biallelic Loss of Function Variant in <scp><i>SEC31A</i></scp> Is Associated With Lethal Neurodevelopmental Disorder, Dysmorphic Features, and Skeletal Defects
Identification of the Third Patient With <scp>PAICS</scp> Deficiency Harbouring the p.(<scp>Lys53Arg</scp>) Recurrent Variant, Extending the Phenotype Diversity
Next Generation Phenotyping and Synthetic Faces in Coffin Siris Syndrome ethnicity
Hereditary Spastic Paraplegia Linked to Abnormal Splicing From an <scp><i>AIMP1</i></scp> Missense Variant
Effectiveness and Impact of Transcript Analysis in Clinical Genetics Daily Practice
Risk Factors Associated With Leber Hereditary Optic Neuropathy due to Rare Mutations in Mitochondrial <scp>DNA</scp>‐Encoded Respiratory Complex I Subunits
Ataxia With Vitamin E Deficiency: Case Series, Vitamin E Therapy Response, Founder Effect, and <i>In Silico</i> Analysis