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LIM homeobox protein 3

Lhx3, Lim3
This gene encodes a member a large protein family which carry the LIM domain, a unique cysteine-rich zinc-binding domain. The encoded protein is a transcription factor that is required for pituitary development and motor neuron specification. Mutations in this gene cause combined pituitary hormone deficiency 3. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2010] (from NCBI)
Top mentioned proteins: LIM, LHX4, Pit-1, CAN, Isl-1
Papers on Lhx3
Generation of Highly Enriched V2a Interneurons from Mouse Embryonic Stem Cells.
New
Sakiyama-Elbert et al., Saint Louis, United States. In Exp Neurol, Feb 2016
Directly after selection, approximately 80% of cells are Chx10(+), with 94% Lhx3(+); after several weeks, cultures remain free of proliferative cell types and mature into normal glutamatergic neurons as assessed by molecular markers and electrophysiological methods.
Hic-5 mediates endothelial sprout initiation by regulating a key surface metalloproteinase.
New
Bayless et al., College Station, United States. In J Cell Sci, Feb 2016
Pro-angiogenic factors enhanced co-localization and complex formation between membrane type-1 matrix metalloproteinase (MT1-MMP) and Hic-5, but not paxillin, and the LIM2 and LIM3 domains of Hic-5 were necessary and sufficient for MT1-MMP complex formation.
Recent advances in central congenital hypothyroidism.
Review
New
Dattani et al., London, United Kingdom. In J Endocrinol, Dec 2015
Genetic ascertainment is possible in a minority of cases and reveals mutations in genes controlling the TSH biosynthetic pathway (TSHB, TRHR, IGSF1) in isolated TSH deficiency, or early (HESX1, LHX3, LHX4, SOX3, OTX2) or late (PROP1, POU1F1) pituitary transcription factors in combined hormone deficits.
Two coexisting heterozygous frameshift mutations in PROP1 are responsible for a different phenotype of combined pituitary hormone deficiency.
New
Ruchała et al., Poznań, Poland. In J Appl Genet, Dec 2015
The major contributors are the PROP1, POU1F1, LHX3, LHX4 and HESX1 genes coding transcription factors implicated in pituitary organogenesis.
Genetic Defects in Thyroid Hormone Supply
Review
Macchia et al., Madagascar. In Unknown Journal, 2015
This situation can be determined by alterations in genes involved in ontogeny of the thyroid, including PIT1 and PRPO1, HESX1, LHX3, LHX4 and SOX3 as well as by mutation in the TSH-beta or in the immunoglobulin superfamily member 1 (IGSF1) genes.
Screening feature genes of lung carcinoma with DNA microarray analysis.
Yuan et al., Wuhan, China. In Int J Clin Exp Med, 2014
The transcription biding site analysis showed that these genes were regulated by LHX3, HNF3B, CDP, HFH1, FOXO4, STAT, SOX5, MEF2, FOXO3 and SRY.
Sixteen new lung function signals identified through 1000 Genomes Project reference panel imputation.
Tobin et al., London, United Kingdom. In Nat Commun, 2014
We identify 14 novel loci (P<5 × 10(-8)) in or near ENSA, RNU5F-1, KCNS3, AK097794, ASTN2, LHX3, CCDC91, TBX3, TRIP11, RIN3, TEKT5, LTBP4, MN1 and AP1S2, and two novel signals at known loci NPNT and GPR126, providing a basis for new understanding of the genetic determinants of these traits and pulmonary diseases in which they are altered.
Pituitary development: a complex, temporal regulated process dependent on specific transcriptional factors.
Review
Ortiga-Carvalho et al., Rio de Janeiro, Brazil. In J Endocrinol, 2012
Pituitary organogenesis is a highly complex and tightly regulated process that depends on several transcription factors (TFs), such as PROP1, PIT1 (POU1F1), HESX1, LHX3 and LHX4.
Symptomatic heterozygotes and prenatal diagnoses in a nonconsanguineous family with syndromic combined pituitary hormone deficiency resulting from two novel LHX3 mutations.
GeneRIF
Amselem et al., Paris, France. In J Clin Endocrinol Metab, 2012
This study of the first nonconsanguineous patient with LHX3 mutations demonstrates the pleiotropic roles of LHX3 during development and its full involvement in the complex disease phenotype of syndromic combined pituitary hormone deficiency.
Fusion protein Isl1-Lhx3 specifies motor neuron fate by inducing motor neuron genes and concomitantly suppressing the interneuron programs.
GeneRIF
Lee et al., Portland, United States. In Proc Natl Acad Sci U S A, 2012
Isl1-Lhx3, a LIM-complex mimicking fusion, induces a signature of motor neuron transcriptome & suppresses interneuron differentiation programs. An equimolar ratio of Isl1 & Lhx3 & the LIM domain of Lhx3 are crucial for this.
A distal modular enhancer complex acts to control pituitary- and nervous system-specific expression of the LHX3 regulatory gene.
GeneRIF
Rhodes et al., Indianapolis, United States. In Mol Endocrinol, 2012
This study establishes ISL1 as a novel transcriptional regulator of LHX3 and describes a potential mechanism for regulation by PITX1.
Genetic disorders of the pituitary.
Review
Cohen, Boston, United States. In Curr Opin Endocrinol Diabetes Obes, 2012
RECENT FINDINGS: Novel mutations have been found in transcription factors involved in pituitary development, HESX1; LHX3; LHX4; SOX3; Prophet of Pit-1; and POU1FI, and in some of the signaling molecules expressed in the ventral diencephalon (fibroblast growth factor 8 and GLI2).
Structural basis for partial redundancy in a class of transcription factors, the LIM homeodomain proteins, in neural cell type specification.
GeneRIF
Matthews et al., Sydney, Australia. In J Biol Chem, 2012
X-ray crystal- and small-angle x-ray scattering-derived solution structures of an Lhx4.Isl2 complex exhibit many similarities with that of Lhx3.Isl1; structural differences supported by mutagenic studies reveal differences in the mechanisms of binding.
A recessive mutation resulting in a disabling amino acid substitution (T194R) in the LHX3 homeodomain causes combined pituitary hormone deficiency.
GeneRIF
Rhodes et al., München, Germany. In Horm Res Paediatr, 2011
descriprion of pediatric patients with combined pituitary hormone deficiency with a novel mutation in LHX3; the T194R mutation affects a critical residue in the LHX3 protein; study extends understanding of phenotypic features, molecular mechanism and developmental course associated with mutations in the LHX3 gene
Pituitary transcription factors in the aetiology of combined pituitary hormone deficiency.
Review
Klammt et al., Leipzig, Germany. In Best Pract Res Clin Endocrinol Metab, 2011
Mutations within the genes encoding for the transcription factors LHX3, LHX4, PROP1, and POU1F1 (PIT1) that act at different stages of pituitary development result in unique patterns of hormonal deficiencies reflecting their differential expression during organogenesis.
LIM factor Lhx3 contributes to the specification of motor neuron and interneuron identity through cell-type-specific protein-protein interactions.
Impact
GeneRIF
Pfaff et al., Los Angeles, United States. In Cell, 2002
LIM factor Lhx3 contributes to the specification of motor neuron and interneuron identity through cell-type-specific protein-protein interactions
Mutations in LHX3 result in a new syndrome revealed by combined pituitary hormone deficiency.
Impact
Amselem et al., Créteil, France. In Nat Genet, 2000
We have isolated LHX3, a gene involved in a new syndrome, using a candidate-gene approach developed on the basis of documented pituitary abnormalities of a recessive lethal mutation in mice generated by targeted disruption of Lhx3 (ref.
A LIM-homeodomain combinatorial code for motor-neuron pathway selection.
Impact
Thomas et al., San Diego, United States. In Nature, 1999
Here we study two members of this gene family in Drosophila, namely lim3, the homologue of the vertebrate Lhx3 and Lhx4 genes, and islet, the homologue of the vertebrate Isl1 and Is12 genes.
LIM homeodomain factors Lhx3 and Lhx4 assign subtype identities for motor neurons.
Impact
Pfaff et al., Los Angeles, United States. In Cell, 1999
We show that LIM homeodomain factors Lhx3 and Lhx4 are expressed transiently in motor neurons whose axons emerge ventrally from the neural tube (v-MN).
Multistep control of pituitary organogenesis.
Impact
Westphal et al., Bethesda, United States. In Science, 1998
Lhx3 and Lhx4 (Gsh4), two closely related LIM homeobox genes, determine formation of the pituitary gland in mice.
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