27.11.2023
Authors Wegener JW, Mitronova GY, ElShareif L, Quentin C, Belov V, Pochechueva T, Hasenfuss G, Ackermann L, Lehnart SE Journal Life Science Alliance Citation Life Sci Alliance. 2023 Nov 27;7(2):e202302278. Abstract In the heart, genetic or acquired mishandling of diastolic [Ca2+] by ryanodine receptor type 2 (RyR2) overactivity correlates with
21.11.2023
Unraveling haplotype errors in the DFNA33 locus
Authors Vona B, Regele S, Rad A, Strenzke N, Pater JA, Neumann K, Sturm M, Haack TB, Am Zehnhoff-Dinnesen AG Journal Frontiers in Genetics Citation Front. Genet. 14:1214736. Abstract Genetic heterogeneity makes it difficult to identify the causal genes for hearing loss. Studies from previous decades have mapped numerous genetic
17.11.2023
The SARS-CoV-2 protein ORF3c is a mitochondrial modulator of innate immunity
Authors Stewart H, Lu Y, O’Keefe S, Valpadashi A, Cruz-Zaragoza LD, Michel HA, Nguyen SK, Carnell GW, Lukhovitskaya N, Milligan R, Adewusi Y, Jungreis I, Lulla V, Matthews DA, High S, Rehling P, Emmott E, Heeney JL, Davidson AD, Edgar JR, Smith GL, Firth AE Journal iScience Citation iScience. 2023
17.11.2023
Prediction mismatch responses arise as corrections of a predictive spiking code
Authors van Driel K, Rudelt L, Priesemann V, Mikulasch FA Journal Biorxiv Citation bioRxiv 2023.11.16.567335. Abstract Prediction mismatch responses in cortex seem to signal the difference between an internal model of the animal and sensory observations. Often these responses are interpreted as evidence for the existence of error neurons, which
16.11.2023
Lysinuric protein intolerance caused by a homozygous SLC7A7 deletion and presented with hyperferritinemia and osteoporosis in two siblings
Authors Kalay I, Aykut H, Caliskan Z, Yigit G, Wollnik B Journal Molecular Genetics and Metabolism Reports Citation Mol Genet Metab Rep. 2023 Nov 16;37:101022. Abstract Lysinuric protein intolerance (LPI) is a rare, inherited aminoaciduria caused by biallelic pathogenic variants in the amino acid transporter gene SLC7A7 (OMIM *603593). Individuals
14.11.2023
Spliceosome malfunction causes neurodevelopmental disorders with overlapping features
Authors Li D, Wang Q, Bayat A, Battig MR, Zhou Y, Bosch DG, van Haaften G, Granger L, Petersen AK, Pérez-Jurado LA, Aznar-Laín G, Aneja A, Hancarova M, Bendova S, Schwarz M, Kremlikova Pourova R, Sedlacek Z, Keena BA, March ME, Hou C, O’Connor N, Bhoj EJ, Harr MH, Lemire
13.11.2023
Tazarotene and Bexarotene Show Efficacy as In Vitro Therapeutic Agents in Multiple Sulfatase Deficiency
Authors Schlotawa l, Matysiak K, Kettwig M, Ahrens-Nicklas RC, Baud M, Berulava T, Brunetti-Pierri N, Gagne A, Herbst ZM, Maguire JA, Monfregola J, Pena T, Radhakrishnan K, Schröder S, Waxman EA, Ballabio A, Dierks T, Fischer A, French DL, Gelb MH, Gärtner J Journal Neuropediatrics Citation Neuropediatrics 2023; 54(S 01):
10.11.2023
Bi-allelic ACBD6 variants lead to a neurodevelopmental syndrome with progressive and complex movement disorders
Authors Kaiyrzhanov R, Rad A, Lin SJ, Bertoli-Avella A, Kallemeijn WW, Godwin A, Zaki MS, Huang K, Lau T, Petree C, Efthymiou S, Ghayoor Karimiani E, Hempel M, Normand EA, Rudnik-Schöneborn S, Schatz UA, Baggelaar MP, Ilyas M, Sultan T, Alvi JR, Ganieva M, Fowler B, Aanicai R, Akay Tayfun
06.11.2023
Protein stock for the beginning of a new life
How mysterious structures in the egg cell supply the early embryo as protein storage sites When mammals have offspring, they invest a lot. Unlike fish or frogs, the embryo cannot develop on its own. It has to implant in the uterus, where it is supplied with everything it needs
06.11.2023
Protein stock for the beginning of a new life
How mysterious structures in the egg cell supply the early embryo as protein storage sites When mammals have offspring, they invest a lot. Unlike fish or frogs, the embryo cannot develop on its own. It has to implant in the uterus, where it is supplied with everything it needs to