The Experts below are selected from a list of 360 Experts worldwide ranked by ideXlab platform

Peter Mombaerts - One of the best experts on this subject based on the ideXlab platform.

  • odorant Receptor Gene choice in olfactory sensory neurons the one Receptor one neuron hypothesis revisited
    Current Opinion in Neurobiology, 2004
    Co-Authors: Peter Mombaerts
    Abstract:

    Abstract Designed for General chemical recognition, the mammalian olfactory system shares many similarities with the immune system. Among these is the popular notion that a single olfactory sensory neuron expresses a single odorant Receptor Gene, while all other ∼1000 Genes of this type remain silent. Here, I examine the evidence supporting the one Receptor–one neuron hypothesis. I conclude that, contrary to widespread belief, it is far from being proven. I propose an hypothesis of a developmental phase of oligogenic expression that is followed by positive and negative selection resulting usually in cells with one expressed Receptor. Curiously, selective processes are well established and widely accepted for lymphocytes, but these concepts are essentially ignored for olfactory sensory neurons, despite the analogies that are frequently made between these two systems. More attention must be paid to odorant Receptor Gene choice and expression during development and neuronal differentiation.

  • odorant Receptor Gene choice in olfactory sensory neurons the one Receptor one neuron hypothesis revisited
    Current Opinion in Neurobiology, 2004
    Co-Authors: Peter Mombaerts
    Abstract:

    Designed for General chemical recognition, the mammalian olfactory system shares many similarities with the immune system. Among these is the popular notion that a single olfactory sensory neuron expresses a single odorant Receptor Gene, while all other approximately 1000 Genes of this type remain silent. Here, I examine the evidence supporting the one Receptor-one neuron hypothesis. I conclude that, contrary to widespread belief, it is far from being proven. I propose an hypothesis of a developmental phase of oligogenic expression that is followed by positive and negative selection resulting usually in cells with one expressed Receptor. Curiously, selective processes are well established and widely accepted for lymphocytes, but these concepts are essentially ignored for olfactory sensory neurons, despite the analogies that are frequently made between these two systems. More attention must be paid to odorant Receptor Gene choice and expression during development and neuronal differentiation.

Mary Jeanne Kreek - One of the best experts on this subject based on the ideXlab platform.

Philip Asherson - One of the best experts on this subject based on the ideXlab platform.

Maria Teresa Landi - One of the best experts on this subject based on the ideXlab platform.

Dennis B Lubahn - One of the best experts on this subject based on the ideXlab platform.

  • estrogen resistance caused by a mutation in the estrogen Receptor Gene in a man
    The New England Journal of Medicine, 1994
    Co-Authors: Eric P Smith, Dennis B Lubahn, Jeff Boyd, Graeme R Frank, Hiroyuki Takahashi, Robert M Cohen, Bonny Specker, Timothy C Williams, Kenneth S Korach
    Abstract:

    Background and Methods Mutations in the estrogen-Receptor Gene have been thought to be lethal. A 28-year-old man whose estrogen resistance was caused by a disruptive mutation in the estrogen-Receptor Gene underwent studies of pituitary-gonadal function and bone density and received transdermal estrogen for six months. Estrogen-Receptor DNA, extracted from lymphocytes, was evaluated by analysis of single-strand-conformation polymorphisms and by direct sequencing. Results The patient was tall (204 cm [80.3 in.]) and had incomplete epiphyseal closure, with a history of continued linear growth into adulthood despite otherwise normal pubertal development. He was normally masculinized and had bilateral axillary acanthosis nigricans. Serum estradiol and estrone concentrations were elevated, and serum testosterone concentrations were normal. Serum follicle-stimulating hormone and luteinizing hormone concentrations were increased. Glucose tolerance was impaired, and hyperinsulinemia was present. The bone mineral d...

  • alteration of reproductive function but not prenatal sexual development after insertional disruption of the mouse estrogen Receptor Gene
    Proceedings of the National Academy of Sciences of the United States of America, 1993
    Co-Authors: Jeffrey S Moyer, Thomas S Golding, John F Couse, Kenneth S Korach, Dennis B Lubahn, Oliver Smithies
    Abstract:

    Abstract Estrogen Receptor and its ligand, estradiol, have long been thought to be essential for survival, fertility, and female sexual differentiation and development. Consistent with this proposed crucial role, no human estrogen Receptor Gene mutations are known, unlike the androgen Receptor, where many loss of function mutations have been found. We have Generated mutant mice lacking responsiveness to estradiol by disrupting the estrogen Receptor Gene by Gene targeting. Both male and female animals survive to adulthood with normal gross external phenotypes. Females are infertile; males have a decreased fertility. Females have hypoplastic uteri and hyperemic ovaries with no detectable corpora lutea. In adult wild-type and heterozygous females, 3-day estradiol treatment at 40 micrograms/kg stimulates a 3- to 4-fold increase in uterine wet weight and alters vaginal cornification, but the uteri and vagina do not respond in the animals with the estrogen Receptor Gene disruption. Prenatal male and female reproductive tract development can therefore occur in the absence of estradiol Receptor-mediated responsiveness.

  • androgen Receptor Gene mutations in x linked spinal and bulbar muscular atrophy
    Nature, 1991
    Co-Authors: Albert R La Spada, Dennis B Lubahn, Elizabeth M Wilson, A E Harding, Kenneth H Fischbeck
    Abstract:

    X-LINKED spinal and bulbar muscular atrophy (Kennedy's disease) is an adult-onset form of motorneuron disease which may be associated with signs of androgen insensitivity. We have now investigated whether the androgen Receptor Gene on the proximal long arm of the X chromosome is a candidate Gene for this disease. In patient samples we found androgen Receptor Gene mutations with increased size of a polymorphic tandem CAG repeat in the coding region. These amplified repeats were absolutely associated with the disease, being present in 35 unrelated patients and none of 75 controls. They segregated with the disease in 15 families, with no recombination in 61 meioses (the maximum log likelihood ratio (lod score) is 13.2 at a recombination rate of 0). The association is unlikely to be due to linkage disequilibrium, because 11 different disease alleles were observed. We conclude that enlargement of the CAG repeat in the androgen Receptor Gene is probably the cause of this disorder.