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  1. Artikel ; Online: The homeobox gene TGIF1 is required for chicken ovarian cortical development and generation of the juxtacortical medulla.

    Estermann, Martin Andres / Hirst, Claire Elizabeth / Major, Andrew Thomas / Smith, Craig Allen

    Development (Cambridge, England)

    2021  Band 148, Heft 16

    Abstract: During early embryogenesis in amniotic vertebrates, the gonads differentiate into either ovaries or testes. The first cell lineage to differentiate gives rise to the supporting cells: Sertoli cells in males and pre-granulosa cells in females. These key ... ...

    Abstract During early embryogenesis in amniotic vertebrates, the gonads differentiate into either ovaries or testes. The first cell lineage to differentiate gives rise to the supporting cells: Sertoli cells in males and pre-granulosa cells in females. These key cell types direct the differentiation of the other cell types in the gonad, including steroidogenic cells. The gonadal surface epithelium and the interstitial cell populations are less well studied, and little is known about their sexual differentiation programs. Here, we show the requirement of the homeobox transcription factor gene TGIF1 for ovarian development in the chicken embryo. TGIF1 is expressed in the two principal ovarian somatic cell populations: the cortex and the pre-granulosa cells of the medulla. TGIF1 expression is associated with an ovarian phenotype in estrogen-mediated sex reversal experiments. Targeted misexpression and gene knockdown indicate that TGIF1 is required, but not sufficient, for proper ovarian cortex formation. In addition, TGIF1 is identified as the first known regulator of juxtacortical medulla development. These findings provide new insights into chicken ovarian differentiation and development, specifically cortical and juxtacortical medulla formation.
    Mesh-Begriff(e) Animals ; Cell Differentiation ; Cell Lineage/genetics ; Chick Embryo ; Chickens/genetics ; Embryo, Mammalian/metabolism ; Female ; Gene Expression Regulation, Developmental ; Gene Knockdown Techniques ; Genes, Homeobox ; Gonads/metabolism ; Homeodomain Proteins/genetics ; Homeodomain Proteins/metabolism ; Male ; Ovary/cytology ; Ovary/embryology ; Ovary/metabolism ; Repressor Proteins/genetics ; Repressor Proteins/metabolism ; Sertoli Cells/metabolism ; Sex Determination Processes/genetics ; Sex Differentiation/genetics ; Testis/metabolism
    Chemische Substanzen Homeodomain Proteins ; Repressor Proteins
    Sprache Englisch
    Erscheinungsdatum 2021-08-23
    Erscheinungsland England
    Dokumenttyp Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 90607-4
    ISSN 1477-9129 ; 0950-1991
    ISSN (online) 1477-9129
    ISSN 0950-1991
    DOI 10.1242/dev.199646
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  2. Artikel: Sporadic Creutzfeldt-Jakob disease presenting as a stroke mimic590.

    Hirst, Claire L

    British journal of hospital medicine (London, England : 2005)

    2011  Band 72, Heft 10, Seite(n) 590–591

    Abstract: Creutzfeldt-Jakob disease is a rare neurodegenerative disease in which there is an abnormal accumulation of prion protein. It occurs with an incidence of approximately 1 per million per year. Sporadic Creutzfeldt-Jakob disease occurs in approximately 85% ...

    Abstract Creutzfeldt-Jakob disease is a rare neurodegenerative disease in which there is an abnormal accumulation of prion protein. It occurs with an incidence of approximately 1 per million per year. Sporadic Creutzfeldt-Jakob disease occurs in approximately 85% of cases, with familial, variant and iatrogenic forms less common. Typically sporadic Creutzfeldt-Jakob disease presents with a rapidly progressive dementia, but sub-variants include the Heidenhain and Oppenheimer-Brownell variants. The former presents with visual disturbance and the latter with ataxia. This article describes a 75-year-old man with a Heidenhain variant of sporadic Creutzfeldt-Jakob disease who presented with a sudden onset of homonymous hemianopia mimicking a stroke.
    Mesh-Begriff(e) Aged ; Creutzfeldt-Jakob Syndrome/diagnosis ; Diagnosis, Differential ; Disease Progression ; Fatal Outcome ; Female ; Humans ; Stroke/diagnosis
    Sprache Englisch
    Erscheinungsdatum 2011-11-01
    Erscheinungsland England
    Dokumenttyp Case Reports ; Journal Article
    ISSN 1750-8460
    ISSN 1750-8460
    DOI 10.12968/hmed.2011.72.10.590
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  3. Artikel ; Online: Sex determination and gonadal sex differentiation in the chicken model.

    Hirst, Claire E / Major, Andrew T / Smith, Craig A

    The International journal of developmental biology

    2018  Band 62, Heft 1-2-3, Seite(n) 153–166

    Abstract: Our understanding of avian sex determination and gonadal development is derived primarily from the studies in the chicken. Analysis of gynandromorphic chickens and experimental chimeras indicate that sexual phenotype is at least partly cell autonomous in ...

    Abstract Our understanding of avian sex determination and gonadal development is derived primarily from the studies in the chicken. Analysis of gynandromorphic chickens and experimental chimeras indicate that sexual phenotype is at least partly cell autonomous in the chicken, with sexually dimorphic gene expression occurring in different tissue and different stages. Gonadal sex differentiation is just one of the many manifestations of sexual phenotype. As in other birds, the chicken has a ZZ male: ZW female sex chromosome system, in which the male is the homogametic sex. Most evidence favours a Z chromosome dosage mechanism underling chicken sex determination, with little evidence of a role for the W chromosome. Indeed, the W appears to harbour a small number of genes that are un-related to sexual development, but have been retained because they are dosage sensitive factors. As global Z dosage compensation is absent in birds, Z-linked genes may direct sexual development in different tissues (males having on average 1.5 to 2 times the expression level of females). In the embryonic gonads, the Z-linked DMRT1 gene plays a key role in testis development. Beyond the gonads, other combinations of Z-linked genes may govern sexual development, together with a role for sex steroid hormones. Gonadal DMRT1 is thought to activate other players in testis development, namely SOX9 and AMH, and the recently identified HEMGN gene. DMRT1 also represses ovarian pathway genes, such as FOXL2 and CYP19A1. A lower level of DMRT1 expression in the female gonads is compatible with activation of the ovarian pathway. Some outstanding questions include how the key testis and ovary genes, DMRT1 and FOXL2, are regulated. In addition, confirmation of the central role of these genes awaits genome editing approaches.
    Mesh-Begriff(e) Animals ; Aromatase/metabolism ; Cell Differentiation ; Chick Embryo ; Chickens ; Dosage Compensation, Genetic ; Female ; Gene Dosage ; Gene Expression Regulation, Developmental ; Genome ; Gonads/embryology ; Male ; Ovary/embryology ; SOX9 Transcription Factor/metabolism ; Sex Chromosomes ; Sex Determination Processes ; Sex Differentiation/genetics ; Testis/embryology
    Chemische Substanzen SOX9 Transcription Factor ; Aromatase (EC 1.14.14.1)
    Sprache Englisch
    Erscheinungsdatum 2018-04-04
    Erscheinungsland Spain
    Dokumenttyp Journal Article ; Review
    ZDB-ID 1036070-0
    ISSN 1696-3547 ; 0214-6282
    ISSN (online) 1696-3547
    ISSN 0214-6282
    DOI 10.1387/ijdb.170319cs
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  4. Artikel: The avian embryo as a model system for skeletal myogenesis.

    Hirst, Claire E / Marcelle, Christophe

    Results and problems in cell differentiation

    2015  Band 56, Seite(n) 99–122

    Abstract: This review will focus on the use of the chicken and quail as model systems to analyze myogenesis and as such will emphasize the experimental approaches that are strongest in these systems-the amenability of the avian embryo to manipulation and in ovo ... ...

    Abstract This review will focus on the use of the chicken and quail as model systems to analyze myogenesis and as such will emphasize the experimental approaches that are strongest in these systems-the amenability of the avian embryo to manipulation and in ovo observation. During somite differentiation, a wide spectrum of developmental processes occur such as cellular differentiation, migration, and fusion. Cell lineage studies combined with recent advancements in cell imaging allow these biological phenomena to be readily observed and hypotheses tested extremely rapidly-a strength that is restricted to the avian system. A clear weakness of the chicken in the past has been genetic approaches to modulate gene function. Recent advances in the electroporation of expression vectors, siRNA constructs, and use of tissue specific reporters have opened the door to increasingly sophisticated experiments that address questions of interest not only to the somite/muscle field in particular but also fundamental to biology in general. Importantly, an ever-growing body of evidence indicates that somite differentiation in birds is indistinguishable to that of mammals; therefore, these avian studies complement the complex genetic models of the mouse.
    Mesh-Begriff(e) Animals ; Cell Differentiation/genetics ; Cell Lineage/genetics ; Chick Embryo ; Gene Expression Regulation, Developmental ; Mice ; Models, Biological ; Muscle Development/genetics ; Quail/growth & development ; Somites/growth & development
    Sprache Englisch
    Erscheinungsdatum 2015
    Erscheinungsland Germany
    Dokumenttyp Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ISSN 0080-1844
    ISSN 0080-1844
    DOI 10.1007/978-3-662-44608-9_5
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  5. Artikel ; Online: Prion-like domains drive CIZ1 assembly formation at the inactive X chromosome.

    Sofi, Sajad / Williamson, Louisa / Turvey, Gabrielle L / Scoynes, Charlotte / Hirst, Claire / Godwin, Jonathan / Brockdorff, Neil / Ainscough, Justin / Coverley, Dawn

    The Journal of cell biology

    2022  Band 221, Heft 4

    Abstract: CIZ1 forms large assemblies at the inactive X chromosome (Xi) in female fibroblasts in an Xist lncRNA-dependent manner and is required for accurate maintenance of polycomb targets genome-wide. Here we address requirements for assembly formation and show ... ...

    Abstract CIZ1 forms large assemblies at the inactive X chromosome (Xi) in female fibroblasts in an Xist lncRNA-dependent manner and is required for accurate maintenance of polycomb targets genome-wide. Here we address requirements for assembly formation and show that CIZ1 undergoes two direct interactions with Xist, via independent N- and C-terminal domains. Interaction with Xist, assembly at Xi, and complexity of self-assemblies formed in vitro are modulated by two alternatively spliced glutamine-rich prion-like domains (PLD1 and 2). PLD2 is dispensable for accumulation at existing CIZ1-Xi assemblies in wild-type cells but is required in CIZ1-null cells where targeting, assembly, and enrichment for H3K27me3 and H2AK119ub occur de novo. In contrast, PLD1 is required for both de novo assembly and accumulation at preexisting assemblies and, in vitro, drives formation of a stable fibrillar network. Together they impart affinity for RNA and a complex relationship with repeat E of Xist. These data show that alternative splicing of two PLDs modulates CIZ1's ability to build large RNA-protein assemblies.
    Mesh-Begriff(e) Alternative Splicing ; Animals ; Female ; Fibroblasts ; Histones ; Mice ; Nuclear Proteins/genetics ; Prions ; RNA, Long Noncoding/genetics ; X Chromosome/genetics ; X Chromosome Inactivation/genetics
    Chemische Substanzen Ciz1 protein, mouse ; Histones ; Nuclear Proteins ; Prions ; RNA, Long Noncoding ; XIST non-coding RNA
    Sprache Englisch
    Erscheinungsdatum 2022-03-15
    Erscheinungsland United States
    Dokumenttyp Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 218154-x
    ISSN 1540-8140 ; 0021-9525
    ISSN (online) 1540-8140
    ISSN 0021-9525
    DOI 10.1083/jcb.202103185
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  6. Artikel: Iatrogenic Creutzfeldt-Jakob disease presenting 24 years after human growth hormone administration.

    Hirst, Claire

    British journal of hospital medicine (London, England : 2005)

    2005  Band 66, Heft 10, Seite(n) 592–593

    Mesh-Begriff(e) Adult ; Creutzfeldt-Jakob Syndrome/chemically induced ; Female ; Human Growth Hormone/adverse effects ; Humans
    Chemische Substanzen Human Growth Hormone (12629-01-5)
    Sprache Englisch
    Erscheinungsdatum 2005-10
    Erscheinungsland England
    Dokumenttyp Case Reports ; Journal Article ; Review
    ISSN 1750-8460
    ISSN 1750-8460
    DOI 10.12968/hmed.2005.66.10.19901
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  7. Artikel ; Online: There is no such thing as a mild MS relapse. The mild relapse is an Anglo-Saxon delusion - No.

    Hirst, Claire L / Robertson, Neil P

    Multiple sclerosis (Houndmills, Basingstoke, England)

    2012  Band 18, Heft 7, Seite(n) 925–926

    Mesh-Begriff(e) Humans ; Multiple Sclerosis, Relapsing-Remitting ; Recurrence
    Sprache Englisch
    Erscheinungsdatum 2012-07
    Erscheinungsland England
    Dokumenttyp Journal Article
    ZDB-ID 1290669-4
    ISSN 1477-0970 ; 1352-4585
    ISSN (online) 1477-0970
    ISSN 1352-4585
    DOI 10.1177/1352458512450089
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  8. Artikel ; Online: Cytoplasmic NOTCH and membrane-derived β-catenin link cell fate choice to epithelial-mesenchymal transition during myogenesis.

    Sieiro, Daniel / Rios, Anne C / Hirst, Claire E / Marcelle, Christophe

    eLife

    2016  Band 5

    Abstract: How cells in the embryo coordinate epithelial plasticity with cell fate decision in a fast changing cellular environment is largely unknown. In chick embryos, skeletal muscle formation is initiated by migrating Delta1-expressing neural crest cells that ... ...

    Abstract How cells in the embryo coordinate epithelial plasticity with cell fate decision in a fast changing cellular environment is largely unknown. In chick embryos, skeletal muscle formation is initiated by migrating Delta1-expressing neural crest cells that trigger NOTCH signaling and myogenesis in selected epithelial somite progenitor cells, which rapidly translocate into the nascent muscle to differentiate. Here, we uncovered at the heart of this response a signaling module encompassing NOTCH, GSK-3β, SNAI1 and β-catenin. Independent of its transcriptional function, NOTCH profoundly inhibits GSK-3β activity. As a result SNAI1 is stabilized, triggering an epithelial to mesenchymal transition. This allows the recruitment of β-catenin from the membrane, which acts as a transcriptional co-factor to activate myogenesis, independently of WNT ligand. Our results intimately associate the initiation of myogenesis to a change in cell adhesion and may reveal a general principle for coupling cell fate changes to EMT in many developmental and pathological processes.
    Sprache Englisch
    Erscheinungsdatum 2016-05-24
    Erscheinungsland England
    Dokumenttyp Journal Article
    ZDB-ID 2687154-3
    ISSN 2050-084X ; 2050-084X
    ISSN (online) 2050-084X
    ISSN 2050-084X
    DOI 10.7554/eLife.14847
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  9. Artikel ; Online: Acute myeloid leucaemia presenting as a rapidly progressive polyradiculoneuropathy.

    Hirst, Claire L / Willis, Mark / Hussain, Hussain / Powell, Rob

    BMJ case reports

    2015  Band 2015

    Abstract: Neurological involvement at onset in acute myeloid leucaemia (AML) is rare, with only a few isolated case reports. We present the case of a 46-year-old man with rapidly progressive polyradiculoneuropathy as the presenting feature of AML. The proposed ... ...

    Abstract Neurological involvement at onset in acute myeloid leucaemia (AML) is rare, with only a few isolated case reports. We present the case of a 46-year-old man with rapidly progressive polyradiculoneuropathy as the presenting feature of AML. The proposed mechanism for this is postulated to be direct intraneural infiltration, although a paraneoplastic, autoimmune-related phenomenon could be possible. Despite chemotherapeutic intervention, the patient died 1 month after initial presentation. Although rare, neurological manifestations of AML do occur and it is important to include haematological malignancies in the differential diagnosis in patients presenting with neurological symptoms.
    Mesh-Begriff(e) Fatal Outcome ; Humans ; Leukemia, Myeloid, Acute/complications ; Leukemia, Myeloid, Acute/diagnosis ; Leukemia, Myeloid, Acute/drug therapy ; Male ; Middle Aged ; Polyradiculoneuropathy/etiology
    Sprache Englisch
    Erscheinungsdatum 2015-11-18
    Erscheinungsland England
    Dokumenttyp Case Reports ; Journal Article
    ISSN 1757-790X
    ISSN (online) 1757-790X
    DOI 10.1136/bcr-2015-209556
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  10. Artikel ; Online: Genetic Manipulation of the Avian Urogenital System Using In Ovo Electroporation.

    Hirst, Claire E / Serralbo, Olivier / Ayers, Katie L / Roeszler, Kelly N / Smith, Craig A

    Methods in molecular biology (Clifton, N.J.)

    2017  Band 1650, Seite(n) 177–190

    Abstract: One of the advantages of the avian embryo as an experimental model is its in ovo development and hence accessibility for genetic manipulation. Electroporation has been used extensively in the past to study gene function in chicken and quail embryos . ... ...

    Abstract One of the advantages of the avian embryo as an experimental model is its in ovo development and hence accessibility for genetic manipulation. Electroporation has been used extensively in the past to study gene function in chicken and quail embryos . Readily accessible tissues such as the neural tube, somites, and limb bud, in particular, have been targeted. However, more inaccessible tissues, such as the embryonic urogenital system , have proven more challenging to study. Here, we describe the use of in ovo electroporation of TOL2 vectors or RCASBP avian viral vectors for the rapid functional analysis of genes involved in avian sex determination and urogenital development . In the context of the developing urogenital system , these vectors have inherent advantages and disadvantages, which will be considered here. Either vector can both be used for mis-expressing a gene and for targeting endogenous gene knockdown via expression of short hairpin RNAs (shRNAs). Both of these vectors integrate into the genome and are hence spread throughout developing tissues. Going forward, electroporation could be combined with CRISPR/Cas9 technology for targeted genome editing in the avian urogenital system .
    Mesh-Begriff(e) Animals ; Chick Embryo ; Chickens/genetics ; DNA Transposable Elements ; Electroporation/methods ; Gene Expression Regulation, Developmental ; Gene Knockdown Techniques ; Gene Transfer Techniques ; Genetic Vectors ; Urogenital System/physiology
    Chemische Substanzen DNA Transposable Elements
    Sprache Englisch
    Erscheinungsdatum 2017
    Erscheinungsland United States
    Dokumenttyp Journal Article
    ISSN 1940-6029
    ISSN (online) 1940-6029
    DOI 10.1007/978-1-4939-7216-6_11
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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