Clinical Genetics - 2023

191 articles | Last updated: 2025-12-03 14:12:56
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A bi‐allelic <scp><i>REC114</i></scp> loss‐of‐function variant causes meiotic arrest and nonobstructive azoospermia Chinese
Epidemiological and clinical features of malignant hyperthermia: A scoping review
Identifying candidate genes underlying isolated congenital anosmia
<scp>SAM</scp> domain variants of <scp>EPHB4</scp> associated with aberrant signaling are linked to lymphatic‐related fetal hydrops and facial dysmorphology
Spermatozoa in mice lacking the nucleoporin <scp>NUP210L</scp> show defects in head shape and motility but not in nuclear compaction or histone replacement
<scp><i>RUNX2</i></scp>‐related metaphyseal dysplasia with maxillary hypoplasia: A rare skeletal disorder resembling <scp><i>SFRP4</i></scp>‐related Pyle disease
A mother and her daughter carrying a pathogenic expansion of the <scp><i>HTT</i></scp> gene with a phenotype encompassing motor neuron disease and <scp>Huntington's</scp> disease
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Exome sequencing identifies homozygous variants in <i>MBOAT7</i> associated with neurodevelopmental disorder Pakistani
Diagnostic exome identifies a novel <scp>PRKG2</scp> mutation in a proband with skeletal dysplasia
<scp>CAMTA1‐related</scp> disorder: Phenotypic and molecular characterization of 26 new individuals and literature review
Characterization of <i>D4Z4</i> alleles and assessment of de novo cases in Facioscapulohumeral dystrophy (<scp>FSHD</scp>) in a cohort of Italian families Italian families
Genetic identification of familial hypercholesterolemia within whole genome sequences in 6820 newborns Chinese; Qingdao of China; Chinese population
A de novo variant in <i>RERE</i> causes autistic behavior by disrupting related genes and signaling pathway
A novel homozygous variant in <i>PMVK</i> is associated with enhanced <scp>IL1β</scp> secretion and a <scp>hyper‐IgD</scp> syndrome‐like phenotype
Functional analysis of the <scp>CTNS</scp> gene exonic variants predicted to affect splicing
<i>ERBB3</i> deficiency causes a multisystemic syndrome in human patient and zebrafish
Assessing <scp>type I</scp> collagen expression and quality in cellular models of osteogenesis imperfecta
<scp>CATSHL</scp> syndrome, a new family and phenotypic expansion
Inheritance of c.<scp>628‐6G</scp>&gt;A <i>GNB5</i> hypomorphic allele uncovers another challenge in the pathogenic prediction of genomic variants
Parental request for familial carrier testing in early childhood: The genetic counseling perspective
Gene mutations as predictors of central lymph mode metastasis in <scp>cN0 PTC</scp>: A meta‐analysis
A novel <i>RNF125</i> variant associated with Tenorio syndrome alters ubiquitin chain binding
<scp><i>CCDC65</i></scp>, encoding a component of the axonemal <scp>Nexin‐Dynein</scp> regulatory complex, is required for sperm flagellum structure in humans
Association between genetic variants of transmembrane transporters and susceptibility to anthracycline‐induced cardiotoxicity: Current understanding and existing evidence
A splice donor variant of <i>GAS8</i> induces structural disorganization of the axoneme in sperm flagella and leads to nonsyndromic male infertility
Digenic inheritance accounts for phenotypic variability in amelogenesis imperfecta
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Genetic counselling considerations with genetic/genomic testing in Neonatal and Pediatric Intensive Care Units: A scoping review
Genetic and phenotypic findings in 34 novel Spanish patients with <scp><i>DDX3X</i></scp> neurodevelopmental disorder
Progressive ataxia, ophthalmoparesis, and hypogonadotropic hypogonadism in a family with a novel variant in the <i>KIFBP</i> gene
<scp>DNA</scp>‐pools targeted‐sequencing as a robust cost‐effective method to detect rare variants: Application to dilated cardiomyopathy genetic diagnosis
De novo enhancer deletion of LMX1B produces a mild nail‐patella clinical phenotype
Rigorous evaluation of genetic and epigenetic effects on clinical laboratory measurements using Japanese monozygotic twins Japanese
Clinical findings in individuals with duplication of genes associated with X‐linked intellectual disability
<scp>RIPOR2</scp>: A new gene of non‐syndromic cochleovestibular dysfunction, discrepancy between human pathology and animal models
Clinical exome sequencing reveals a novel pathogenic variant in <i>KIF12</i> underlying cholestasis with highly variable phenotypes
The third case of Marbach‐Rustad progeroid syndrome caused by a <i>de novo LEMD2</i> variant
Perspectives of carriers of X‐linked retinal diseases on genetic testing and gene therapy: A global survey
The best of both worlds: Blending cutting‐edge research with clinical processes for a productive exome clinic
Genetic backgrounds and diagnosis of familial hypercholesterolemia
Adding to the evidence of gene‐disease association of <i>RAP1B</i> and syndromic thrombocytopenia
Variants in <scp><i>DOK7</i></scp> results in fetal akinesia deformation sequence: A case report and review of literature
A homozygous frameshift variant expands the clinical spectrum of <i>SAMD9</i> gene defects Lebanese
A novel non‐recurrent <scp>CNV</scp> deletion involving <scp><i>TBX4</i></scp> and leaving <scp><i>TBX2</i></scp> intact causes congenital alveolar dysplasia
CLDN1 Arg81His founder variant causes ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis (ILVASC) syndrome in Moroccan Jews Moroccan Jewish descent; North African Jewish ancestry; Jews of Moroccan descent
Exploring factors impacting <scp>haplotype‐based</scp> noninvasive prenatal diagnosis for <scp>single‐gene</scp> recessive disorders
<scp>LRRC23</scp> deficiency causes male infertility with idiopathic asthenozoospermia by disrupting the assembly of radial spokes
Sequence variants in <i>DLX5</i>, <i>HOXD13</i> and 445 kb‐microduplication surrounding <i>BTRC</i> cause split‐hand/foot malformation in three different families
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Novel <scp><i>WEE2</i></scp> homozygous mutations c.<scp>1346C</scp>&gt;T and c.<scp>949A</scp>&gt;T identified in primary infertile women due to unexplained fertilization failure
Identification of nonfunctional <scp>PABPC1L</scp> causing oocyte maturation abnormalities and early embryonic arrest in female primary infertility
Novel <scp><i>MEIOB</i></scp> pathogenic variants including a homozygous non‐canonical splicing variant, cause meiotic arrest and human non‐obstructive azoospermia Chinese
Prospects and challenges of <scp>CRISPR</scp>/Cas9 gene‐editing technology in cancer research
Spectrum of variants in a large Chinese Gitelman syndrome cohort Chinese
Lamb–Shaffer syndrome: 20 Spanish patients and literature review expands the view of neurodevelopmental disorders caused by <i>SOX5</i> haploinsufficiency Spanish
Delineating the phenotype of <i>PNPLA8</i>‐related mitochondriopathies
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A X‐linked nonsense <i>APOO/MIC26</i> variant causes a lethal mitochondrial disease with progeria‐like phenotypes
Compound heterozygous variants in <i>RAB34</i> in a rare skeletal ciliopathy syndrome
Recurrence of a <scp><i>BBS1</i></scp> variant in <scp>Bardet–Biedl</scp> patients from Prince Edward Island
From pollakiuria to Donnai‐Barrow syndrome diagnosis in pediatric age
Low risk of embryonic and other cancers in <i>PIK3CA</i><scp>‐related</scp> overgrowth spectrum: Impact on screening recommendations
Infantile onset encephalomyopathy, retinopathy, optic atrophy, and mitochondrial DNA depletion associated with a novel pathogenic <i>DHX16</i> variant
Pathogenic variants in <i>SOX11</i> mimicking <scp>Pitt‐Hopkins</scp> syndrome phenotype
Genetic testing and gene therapy in retinal diseases: Knowledge and perceptions of optometrists in <scp>Australia</scp> and <scp>New Zealand</scp>
A novel in‐frame deletion in <i>MYOT</i> causes an early adult onset distal myopathy
Issue Information
Biallelic <i>ANKS6</i> null variants cause notable extrarenal phenotypes in a nephronophthisis patient and lead to hepatobiliary abnormalities by <scp>YAP1</scp> deficiency
A deep intronic <scp><i>DLG4</i></scp> variant resulting in <scp><i>DLG4</i></scp>‐related synaptopathy
Genetic findings in <scp>Czech</scp> patients with limb girdle muscular dystrophy Czech ("Czech LGMD population")
Human phenotype caused by biallelic <i>KDM4B</i> frameshift variant
Genetic and phenotypic diversities of nevus spilus phenotypes: Case series and a proposed diagnostic algorithm
Inherited <scp>CSNK2A1</scp> variants in families with <scp>Okur‐Chung</scp> neurodevelopmental syndrome
Motivations to learn genomic information are not exceptional: Lessons from behavioral science
<scp>GPI</scp>‐anchoring disorders and the heart: Is cardiomyopathy an overlooked feature?
Novel Tu translation elongation factor, mitochondrial (<i>TUFM</i>) homozygous variant in a consanguineous family with premature ovarian insufficiency
Congenital heart defects in <i>CTNNB1</i> syndrome: Raising clinical awareness
<scp>Genotype‐phenotype</scp> analysis of selective failure of tooth eruption—A systematic review
Noninvasive prenatal diagnosis of Mendelian disorders for consanguineous couples by relative genotype dosage
<scp><i>DST</i></scp> variants are responsible for neurogenic arthrogryposis multiplex congenita enlarging the spectrum of type <scp>VI</scp> hereditary sensory autonomic neuropathy
Mutation profile of <scp>Bardet‐Biedl</scp> syndrome patients from India: Implicative role of multiallelic rare variants and oligogenic inheritance pattern
A novel homozygous missense variant in <i>TBC1D31</i> in a consanguineous family with congenital anomalies of the kidney and urinary tract (<scp>CAKUT</scp>)
Filling the gap: Genetic risk assessment in hypercholesterolemia using <scp>LDL‐C</scp> and <scp>LPA</scp> genetic scores
Issue Information
Severe trichothiodystrophy and cardiac malformation in a newborn carrying a novel <scp><i>GTF2H5</i></scp> homozygous truncating variant
Performance of the <scp>ACMG‐AMP</scp> criteria in a large familial renal glucosuria cohort with identified <scp><i>SLC5A2</i></scp> sequence variants
Spanish mental health residents' perspectives about residency education on the genetics of psychiatric disorders: A cross‐sectional survey
Medical and psychosocial outcomes of state‐funded population genomic screening
Intratumor heterogeneity in colorectal cancer: Distribution of tumor suppressor gene variants with regard to patient lymph node status
Identification of copy number variants with genome sequencing: Clinical experiences from the <scp>NYCKidSeq</scp> program
A homozygous frameshift variant in <i>SYCP2</i> caused meiotic arrest and non‐obstructive azoospermia
Nonsense variant in a consanguineous family expands the phenotype of <i>KPTN</i> gene‐related syndrome to include hearing impairment
<scp><i>MIR204</i></scp> n.<scp>37C</scp>&gt;T variant as a cause of chorioretinal dystrophy variably associated with iris coloboma, early‐onset cataracts and congenital glaucoma Czech
A homozygous founder variant in <scp><i>PDE2A</i></scp> causes paroxysmal dyskinesia with intellectual disability
Biallelic loss of function variant in <scp><i>ZNF808</i></scp> is associated with <scp>non‐syndromic</scp> neonatal diabetes
De‐novo “germline second hit” loss‐of‐heterozygosity <i>RBP3</i> deletion mutation causing recessive high myopia
Development and validation of a novel nomogram to predict the impact of the polymorphism of the <i>ICAM‐1</i> gene on the prognosis of ischemic cardiomyopathy
A recessive <scp><i>ACTL7A</i></scp> founder variant leads to male infertility due to acrosome detachment in <scp>Pakistani Pashtuns</scp>
Genetic diagnosis of kidney disease by whole exome sequencing and its clinical application
Pathogenic bi‐allelic variants of meiotic <scp>ZMM</scp> complex gene <scp><i>SPO16</i></scp> in premature ovarian insufficiency
Analysis of a non‐lethal biallelic frameshift mutation in <scp><i>ZMPSTE24</i></scp> reveals utilization of alternative translation initiation codons Pakistani
Issue Information
<scp>GNAS</scp> gene mutations affecting <scp><i>XLαs</i></scp> and bone health: A long neglected relationship
Functional characterization of a novel <scp><i>PRRT2</i></scp> variant found in a Portuguese patient with hemiplegic migraine Portuguese
Investigating genotype‐to‐phenotype correlation in <scp>CHARGE</scp> syndrome by deep phenotyping and multiparametric clustering
Lymphedema is associated with <i>CELSR1</i> in <scp>Phelan–McDermid</scp> syndrome
Morphological and genetic causes of fetal cardiomyopathies
A novel homozygous variant in <i>ZFP36L2</i> cause female infertility due to oocyte maturation defect
Increased diagnostic yield from negative whole genome‐slice panels using automated reanalysis
Sleep disturbances in Phelan‐McDermid syndrome: Clinical and metabolic profiling of 56 individuals
Characterization of novel <scp><i>CACNA1A</i></scp> splice variants by <scp>RNA</scp>‐sequencing in patients with episodic or congenital ataxia
A founder <i>PPIL1</i> variant underlies a recognizable form of microlissencephaly with pontocerebellar hypoplasia
Novel biallelic variants expand the phenotype of <scp>NAA20</scp>‐related syndrome
Pigmentation abnormalities in <scp>Coffin‐Siris</scp> syndrome
<scp>POU3F3</scp>‐related disorder: Defining the phenotype and expanding the molecular spectrum
Functional characterization of novel or yet uncharacterized <i>ATP7B</i> missense variants detected in patients with clinical Wilson's disease
Genome sequencing identifies <i>KMT2E</i>‐disrupting cryptic structural variant in a female with O'Donnell‐Luria‐Rodan syndrome
Novel <i>LSS</i> variants in alopecia and intellectual disability syndrome: New case report and clinical spectrum of <i>LSS</i>‐related rare disease traits
Octapeptide repeat alteration mutations of the prion protein gene in clinically diagnosed Alzheimer's disease and frontotemporal dementia
Issue Information
Expanding the phenotype of <scp><i>PIGP</i></scp> deficiency to multiple congenital anomalies‐hypotonia‐seizures syndrome
Evolution of clinical and radiological presentations of spondyloepimetaphyseal dysplasia, <scp>RPL13</scp>‐related: Description of 11 further cases
A synonymous codon variant altering splicing of <i>RBCK1</i> expands the phenotype and genotype spectra of polyglucosan body myopathy 1
Possible biallelic inheritance in <i>TIE1</i> in a family with congenital lymphedema, intestinal lymphangiectasia and cutis aplasia
Genetic risk for Huntington Disease and reproductive decision‐making: A systematic review
Support to caregivers who have received genetic information about neurodevelopmental and psychiatric vulnerability in their young children: A narrative review
<i>RNASEH2C</i> c.<scp>194G</scp>&gt;A is a Chinese‐specific founder mutation in three unrelated patients with <scp>Aicardi‐Goutières</scp> syndrome 3 Chinese-specific; South Asian; other ethnicities
Discovering the <scp><i>ANK2</i></scp>‐related autism phenotype
Revisiting <i>TOP2B</i>‐related phenotypes: Three new cases and literature review
Detection of male 2+0 and 1+0 carriers for spinal muscular atrophy by digital <scp>PCR</scp>
Clinical and molecular spectrum of a large Egyptian cohort with <scp><i>ALS2</i></scp>‐related disorders of infantile‐onset of clinical continuum <scp>IAHSP</scp>/<scp>JPLS</scp> Egyptian; single ethnic cohort
Steroid‐resistant nephrotic syndrome caused by nuclear pore gene <scp>NUP133</scp> variation
Identification of bi‐allelic <i>LFNG</i> variants in three patients and further clinical and molecular refinement of spondylocostal dysostosis 3
Survival, surveillance, and genetics in patients with Peutz–Jeghers syndrome: A nationwide study
Compound heterozygous variants in <scp><i>WLS</i></scp> gene causes <scp>Zaki</scp> syndrome Chinese population
A recurrent de novo variant in <scp><i>NUSAP1</i></scp> escapes nonsense‐mediated decay and leads to microcephaly, epilepsy, and developmental delay
Issue Information
Direct‐to‐consumer genetic tests providing health risk information: A systematic review of consequences for consumers and health services
Missense mutation of c.<scp>635 T</scp> &gt; C in <scp><i>CAPN3</i></scp> impairs muscle injury repair in a <scp>Limb‐Girdel</scp> Muscular Dystropy Model
Hereditary cancer predispositions: Comparison of multigene panel sequencing on fresh‐frozen breast/ovarian tumor versus blood
The genetics of incontinence: A scoping review
Use of a web‐based portal to return normal individual research results in Early Check: Exploring user behaviors and attitudes
Clinical heterogeneity of <scp><i>NADSYN1</i></scp>‐associated <scp>VCRL</scp> syndrome
Clinical characteristics and genotypes of 201 patients with mucopolysaccharidosis type <scp>II</scp> in China: A retrospective, observational study
Missense variant in <i>RBM10</i> associated with mild and non‐lethal form of <scp>TARP</scp> syndrome
<i>HRAS</i>‐related epidermal nevus syndromes: Expansion of the spectrum with first branchial arch defects
The prevalence and phenotypic range associated with biallelic <scp><i>PKDCC</i></scp> variants
An <i>FBN1</i> deep intronic variant is associated with pseudoexon formation and a variable Marfan phenotype in a five generation family
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Systematic evaluation of narrow‐sense validity of polygenic risk score for prostate cancer in a Chinese prostate biopsy cohort Chinese; ChinaPCa dataset; Chinese prostate biopsy cohort
Constitutional mosaicism for a pathogenic variant in <i>MEN1</i> associated with multiple endocrine neoplasia type <scp>I</scp> syndrome
Free carnitine concentrations and biochemical parameters in medium‐chain <scp>acyl‐CoA</scp> dehydrogenase deficiency: Genotype–phenotype correlation
Progress on the study of Popeye domain‐containing 3 (<scp>POPDC3</scp>) in malignancies and striated muscle function and homeostasis
Origins of <scp>SOPH</scp> syndrome: A study of 93 Yakut patients with review of C‐terminal phenotype
Homozygous frameshift variant in desmoglein 2 gene causes biventricular arrhythmogenic right ventricular cardiomyopathy
A novel de novo variant in the <i>PHF21A</i> causes craniofacial abnormalities, intellectual disability and skeletal manifestations
Two new cases with novel pathogenic variants reflecting the clinical diversity of <scp>Schaaf‐Yang</scp> syndrome Turkish
Economic evaluation of next‐generation sequencing techniques in diagnosis of genetic disorders: A systematic review
Variants of <scp><i>LRP2</i></scp>, encoding a multifunctional cell‐surface endocytic receptor, associated with hearing loss and retinal dystrophy
Compound heterozygous splicing variants expand the genotypic spectrum of <scp><i>EMC1</i></scp>‐related disorders
Diagnostic yield from prenatal exome sequencing for non‐immune hydrops fetalis: A systematic review and meta‐analysis
Broad spectrum of anomalies including quadricuspid aortic valve associated with a novel frameshift <scp><i>SALL4</i></scp> variant
Further case of enlarged spinal nerve roots in <i>KRAS</i>‐related Noonan syndrome
Updated protocol for genetic testing, screening and clinical management of individuals at risk of <i>NF2</i>‐related schwannomatosis
Issue Information
Biallelic intragenic tandem duplication of <i>CPLANE1</i> in Joubert syndrome: A case report
<scp>DNA</scp> methylation signature classification of rare disorders using publicly available methylation data
Overcoming the challenges associated with identification of deep intronic variants by whole genome sequencing
A progressive <scp>KY</scp> myopathy could be caused by a missense pathogenic variant
Further delineation of dosage‐sensitive K/L mediated Xq28 duplication syndrome includes incomplete penetrance
The family reported to have X‐linked <scp>Dyggve–Melchior–Clausen</scp> syndrome instead has X‐linked <scp>SEDT</scp> caused by a novel <scp>TRAPPC2</scp> frameshift variant
Identification of <i>POLR3B</i> biallelic mutations‐associated hypomyelinating leukodystrophy‐8 in two siblings
Genetic spectrum and clinical features in a cohort of Chinese patients with isolated dystonia
Possible association of trichorhinophalangeal syndrome I and intracranial subependymoma
Imagawa–Matsumoto syndrome: <i>SUZ12</i>‐related overgrowth disorder
Mosaic variegated aneuploidy syndrome 2 with biallelic novel <scp><i>CEP57</i></scp> splice site variation in Indian siblings: Expanding the clinical and molecular spectrum Indian
Issue Information
<scp>DKK1</scp> is a strong candidate for mesiodens and taurodontism
Molecular basis of carotid body tumor and associated clinical features in Japan identified by genomic, immunohistochemical, and clinical analyses Japanese
Novel variants in <scp><i>ACTL7A</i></scp> and <scp><i>PLCZ1</i></scp> are associated with male infertility and total fertilization failure
Familial transmission of chromoanagenesis leads to unpredictable unbalanced rearrangements through meiotic recombination
Expanding the spectrum of <i>EEF1D</i> neurodevelopmental disorders: Biallelic variants in the guanine exchange domain
Three <scp>KINSSHIP</scp> syndrome patients with mosaic and germline <scp><i>AFF3</i></scp> variants Japanese ("three unrelated Japanese patients")
Genomic characterization of thymic epithelial tumors reveals critical genes underlying tumorigenesis and poor prognosis
A novel <scp>PLS1</scp> c.981+<scp>1G</scp>&gt;A variant causes autosomal‐dominant hereditary hearing loss in a family Chinese
Copy number variations in <i>SPAST</i> and <i>ATL1</i> are rare among <scp>Brazilians</scp>
Expanding the sperm phenotype caused by mutations in <scp><i>SPATA20</i></scp>: A novel splicing mutation in an infertile patient with partial globozoospermia
Novel homozygous variant of <scp><i>CCIN</i></scp> causes male infertility owing to the abnormal sperm head with a nuclear subsidence phenotype
Evaluation of out‐of‐pocket pay genetic testing in a publicly funded healthcare system
Expansion of the phenotypic and molecular spectrum of <scp>CWF19L1</scp>‐related disorder