Search results for "Nkx2.1"

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NKX2-1 New Mutation Associated With Myoclonus, Dystonia, and Pituitary Involvement

2018

Background: NKX2-1 related disorders (also known as brain-lung-thyroid syndrome or benign hereditary chorea 1) are associated with a wide spectrum of symptoms. The core features are various movement disorders, characteristically chorea, less frequently myoclonus, dystonia, ataxia; thyroid disease; and lung involvement. The full triad is present in 50% of affected individuals. Numerous additional symptoms may be associated, although many of these were reported only in single cases. Pituitary dysfunction was ambiguously linked to NKX2-1 haploinsufficiency previously. Case Presentation: We examined two members of a family with motor developmental delay, mixed movement disorder (myoclonus, dyst…

0301 basic medicinePediatricsmedicine.medical_specialtycongenital hereditary and neonatal diseases and abnormalitiesMovement disordersAtaxialcsh:QH426-470NKX2-1 geneCase Reportbenign hereditary choreapituitary03 medical and health sciences0302 clinical medicineBenign hereditary choreamyoclonus dystoniaHypogonadotropic hypogonadismmedicineGeneticschoreaGenetics (clinical)Dystoniabusiness.industryChoreabrain-lung-thyroid syndromemedicine.diseasenervous system diseaseslcsh:Genetics030104 developmental biologyNKX2-1 related disordersempty sellaMolecular Medicinemedicine.symptombusinessHaploinsufficiencyMyoclonus030217 neurology & neurosurgeryFrontiers in Genetics
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Topography of somatostatin gene expression relative to molecular progenitor domains during ontogeny of the mouse hypothalamus

2010

The hypothalamus comprises alar, basal, and floor plate developmental compartments. Recent molecular data support a rostrocaudal subdivision into rostral (terminal) and caudal (peduncular) halves. In this context, the distribution of neuronal populations expressing somatostatin (Sst) mRNA was analyzed in the developing mouse hypothalamus, comparing with the expression pattern of the genes Orthopedia (Otp), Distal-less 5 (Dlx5), Sonic Hedgehog (Shh), and Nk2 homeobox 1 (Nkx2.1). At embryonic day 10.5 (E10.5), Sst mRNA was first detectable in the anterobasal nucleus, a Nkx2.1-, Shh-, and Otp-positive basal domain. By E13.5, nascent Sst expression was also related to two additional Otp-positiv…

endocrine systemBasal plate (neural tube)forebrain[SDV]Life Sciences [q-bio]OtpNeuroscience (miscellaneous)Shhlcsh:RC321-571lcsh:QM1-69503 medical and health sciencesCellular and Molecular Neuroscience0302 clinical medicineArcuate nucleusmedicine[SDV.BBM]Life Sciences [q-bio]/Biochemistry Molecular BiologySonic hedgehoghypothalamuslcsh:Neurosciences. Biological psychiatry. Neuropsychiatry[SDV.BDD]Life Sciences [q-bio]/Development BiologyOriginal Research030304 developmental biologyFloor plate0303 health sciencesAlar platebiologyDlk5forebrain;hypothalamus;Sst;Otp;Dlk5;Nkx2.1;Shh;in situ hybridization;CONTAINING NEURON SYSTEM;SONIC-HEDGEHOG;FOREBRAIN DEVELOPMENT;VENTRAL FOREBRAIN;DEVELOPMENTAL EXPRESSION;BRAIN-DEVELOPMENT;RAT HYPOTHALAMUS;GROWTH-HORMONE;CELL LINEAGES;DIENCEPHALONlcsh:Human anatomyCiencias naturales y ciencias de la saludSstNkx2.1medicine.anatomical_structureHypothalamusForebrainembryonic structuresNeuranatomybiology.protein[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]in situ hybridizationAnatomyNucleusNeuroscience030217 neurology & neurosurgery
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