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Gene responsible for severe facial defects identified

A team of researchers from UNIGE and Beihang University has identified the FOXI3 gene as responsible for one form of Goldenhar syndrome, a rare congenital disorder. Pathogenic variants in both copies of the FOXI3 gene are necessary for the disease to develop, following an autosomal recessive inheritance pattern.

SourceUniversité de Genève·JournalNature Communications·TypeNews article·DateMay 4, 2023

Stay CALM when the heart skips a beat

Researchers at Kyoto University have discovered a genetic mutation that causes lethal arrhythmia in humans. The study found that a novel variant of the CALM2 gene produces robust arrhythmogenicity in human-induced pluripotent stem cell-derived cardiomyocytes.

SourceKyoto University·JournalCirculation Arrhythmia and Electrophysiology·TypeExperimental study·DateApr 13, 2023

5,500 people diagnosed with rare genetic diseases in major research study

A nationwide UK research study has diagnosed around 5,500 children with severe developmental disorders, identifying the genetic cause of their condition. The Deciphering Developmental Disorders study used genomic sequencing technology to provide diagnoses for families from across the UK and Ireland.

SourceUniversity of Exeter·JournalNew England Journal of Medicine·TypeRandomized controlled/clinical trial·DateApr 12, 2023

Communication may guide family members’ decisions after sudden cardiac death

A new study finds that clear and accurate information about the cause of death, provided in multiple formats, can influence family members' decisions to seek follow-up screening for inherited heart conditions. The study suggests that communication from death investigators and health care professionals is crucial in addressing families'...

SourceAmerican Heart Association·JournalCirculation Cardiovascular Quality and Outcomes·DateApr 4, 2023

Specific modifier genes determine the effect of mutations that cause non-compaction cardiomyopathy

Researchers found that the presence of one Mindbomb1 mutation does not always lead to non-compaction cardiomyopathy, but depends on genetic context provided by other gene mutations. The study identified modifier genes contributing to disease severity and diversity in affected individuals.

SourceCentro Nacional de Investigaciones Cardiovasculares Carlos III (F.S.P.)·JournalCirculation·TypeExperimental study·DateNov 7, 2022

On the trail of missing genes and cancer clues

Researchers at La Jolla Institute for Immunology discovered a direct link between TET protein loss of function and missing genes in embryonic stem cells, which can lead to cancer growth. The study found that TET proteins are crucial for maintaining genome stability, and their loss results in aneuploidies, a common feature of cancer cells.

SourceLa Jolla Institute for Immunology·JournalNature Communications·TypeExperimental study·DateOct 27, 2022

Discovery of 119-million-year-old selfish genes in yeast potentially alters our understanding of how parasitic DNA impacts genome evolution

Researchers have discovered a family of selfish genes, wtf, that have survived for over 100 million years in yeast, contradicting established beliefs on their longevity. These 'killer meiotic drivers' transmit themselves to half of offspring and destroy reproductive cells without being suppressed by natural selection.

SourceStowers Institute for Medical Research·JournaleLife·TypeObservational study·DateOct 19, 2022

Tiny sea creature’s genes shed light on evolution of immunity

Researchers have identified a complex of proteins in a tiny marine invertebrate that share similarities with the human immune system, suggesting an earlier origin for the building blocks of our immune system. The study could ultimately guide the development of new immunotherapies and improve understanding of transplant rejection.

SourceUniversity of Pittsburgh·JournalProceedings of the National Academy of Sciences·DateSep 26, 2022

Some hard-to-crack genome areas carry genes that make us distinctly humans

The completed human genome assembly has revealed new insights into human evolution and diseases. Researchers found that highly repetitive regions, including segmental duplications, contain genes critical for brain development and function. These findings shed light on the genetic factors that make humans distinct from other primates.

SourceUniversity of Washington School of Medicine/UW Medicine·JournalScience·TypeExperimental study·DateMar 31, 2022

ACMG Foundation/David L. Rimoin Inspiring Excellence Award honors Catherine A. Ziats, MD

Catherine A. Ziats, MD, received the David L. Rimoin Inspiring Excellence Award for her platform presentation on alterations in respiratory epithelial gene SPDEF and severe disease responses to COVID-19 infection. The award recognizes her contributions to advancing our understanding of host genetic factors associated with severe COVID-19.

Understanding unexplained low blood sugar in children: More than normal variation

Researchers have identified four novel genes related to glucose metabolism that appear to explain unexplained low blood sugar in at least four families. The discovery may lead to the development of a novel drug against diabetes. However, more studies are needed to confirm the exact functional significance of these genetic mutations.

Microglia turned on

Researchers have developed a microglia-selective fluorogenic probe that triggers fluorescence through gene expression, enabling selective labeling and imaging of microglia cells in live brain tissue. This probe has the potential to detect developing neural diseases such as Alzheimer's disease and stroke.

SourceWiley·JournalAngewandte Chemie International Edition·DateMay 27, 2019

First patient affected by a mutation in the nucleoside transporter SLC28 gene family

Researchers have identified a mutation in the SLC28A1 gene, affecting the synthesis of the hCNT1 protein and altering pyrimidine metabolism. The study provides insights into the potential role of nucleoside transporters in regulating cellular processes and may lead to new therapeutic approaches for cancer treatment.

SourceUniversity of Barcelona·JournalBiochimica et Biophysica Acta (BBA) - Molecular Basis of Disease·DateFeb 5, 2019

Axon guidance gene influences reward system

A study found that individuals with a DCC gene mutation had reduced connectivity between brain regions involved in reward processing, leading to less impulsive behavior and lower cigarette smoking. The researchers also observed decreased volume in the striatum, a brain region critical for motivation and pleasure.

Gene mutation linked to retinitis pigmentosa in Southwestern US Hispanic families

A study has identified a common gene mutation linked to retinitis pigmentosa in Hispanic families from the Southwestern US, with over 70 genes now known to cause the disease. The researchers found that a dominant mutation in the arrestin-1 gene is responsible for 36% of cases, offering hope for future treatments and therapies.

SourceUniversity of Texas Health Science Center at Houston·JournalInvestigative Ophthalmology & Visual Science·DateJun 26, 2017

Discovery yields answers for family with thoracic aortic aneurysm and dissection

Researchers identify LOX gene mutations as cause of thoracic aortic aneurysm and dissection in family study, providing biological mechanism and possible therapeutic targets. The discovery illuminates the genetic basis of a rare disease and may lead to improved diagnosis and treatment for patients with this condition.

SourceBrigham and Women's Hospital·JournalProceedings of the National Academy of Sciences·DateJul 18, 2016

Is nature mostly a tinkerer or an inventor?

The University of Miami study reveals that the KLF/SP gene family expanded and diversified during animal evolution through domain shuffling, gene duplication, and de novo domain evolution. This 'tinkering' process led to an increase in repressor domains, which may have contributed to the development of complex cell types and tissues.

SourceUniversity of Miami·JournalGenome Biology and Evolution·DateAug 18, 2015

Octopus genome sequenced

The California two-spot octopus genome was fully sequenced, revealing widespread genomic rearrangements and a dramatic expansion of genes involved in neuronal development. The findings provide an important foundation for evolutionary studies and deeper investigations into the genetic mechanisms underlying cephalopod-specific traits.