Selected-GenAtlas references SOURCE GeneCards NCBI Gene Swiss-Prot Ensembl
HGNC UniGene Nucleotide OMIM UCSC
Home Page
FLASH GENE
Symbol LMX1A contributors: mct - updated : 29-05-2015
HGNC name LIM homeobox transcription factor 1, alpha
HGNC id 6653
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • two N terminal cysteine-rich (LIM) domains (zinc-binding)
  • an helix-turn-helix DNA binding domain (homeobox)
    • HOMOLOGY
    interspecies homolog to murine Lmx1a
    homolog to C. elegans lim-6
    Homologene
    FAMILY LIM-homeodomain (LIM-HD) transcription factor family
    CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,nucleus
    basic FUNCTION
  • involved in dorsoventral polarity of vertebrate limb (dorsal differentiation) and patterning modulator of motor axon guidance
  • transcriptional regulator of insulin gene, with the LIM-homeo domain (lmx-1)
  • acting as a transcriptional activator by binding to an A/T-rich sequence, the FLAT element, in the insulin gene promoter
  • required for development of the roof plate and, in turn, for specification of dorsal cell fates in the CNS and developing vertebrae
  • being sufficient and required to trigger dopamine cell differentiation
  • inducing the expression of MSX1 (early activity)
  • is required for segregation of sensory epithelia and normal ear histogenesis and morphogenesis
  • LMX1A and LMX1B cooperate with FOXA2 to coordinate the specification of dopaminergic neurons and control of floor plate cell differentiation in the developing mesencephalon
  • is a critical regulator of cell-fate decisions within both these germinal zones
  • its activity is critical for proper production of cells originating from both the cerebellar rhombic lip and the telencephalic cortical hem
  • LMX1A and LMX1B function cooperatively to regulate proliferation, specification, and differentiation of mDA (midbrain dopaminergic neurons) progenitors, including their floor plate-like properties
  • required for the development of midbrain dopaminergic neurons, roof plate formation, and cortical hem development
  • early regulator of midbrain dopamine neural progenitor phenotype specification, also involved in the regulation of midbrain dopaminergic maturation by regulating gene expression of the dopamine transporter
  • very potent inducer of stem cells towards dopaminergic neurons
  • key midbrain dopamine (mDA) regulators (NR4A2, PITX3, and LMX1A) play overlapping as well as distinct roles during neurogenesis and neurotransmitter phenotype determination of mDA neurons
  • is required for proper development of roof plate and cortical hem structures of the forebrain, as well as the development of floor plate and midbrain dopaminergic neurons
    • CELLULAR PROCESS nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS development
    PATHWAY
    metabolism carbohydrate
    signaling
  • WNT1-LMX1A pathway controls the midbrain dopaminergic neurons (mdDA) differentiation
    • a component
    INTERACTION
    DNA binding to the FLAT element, an A/T-rich sequence, in the insulin gene promoter
    RNA
    small molecule metal binding,
  • Zn2+
    • protein
  • SLC6A3 is a direct target of LMX1A, emphasizing a novel role of LMX1A as one of regulators of mature midbrain dopaminergic neurotransmitter phenotypes
  • is an activator of RGS4 and GRB10 and is responsible for the correct specification of rostral and medial mdDA neurons
  • novel regulatory pathway where signaling from PRKDC appears to suppress midbrain-specific LMX1A expression
  • ONECUT1 interacts with LMX1A to promote ventral midbrain neural stem cells (vmNSCs)differentiation into dopamine neuron through WNT1-LMX1A pathway
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Lmx1a/Lmx1b compound-null mutants fail to generate hindbrain roof plate