Affinage

PROP1

Homeobox protein prophet of Pit-1 · UniProt O75360

Length
226 aa
Mass
25.0 kDa
Annotated
2026-06-10
100 papers in source corpus 24 papers cited in narrative 24 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/7 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

PROP1 is a pituitary-specific paired-like homeodomain transcription factor that governs the migration and differentiation of multipotent anterior-pituitary progenitors, marking a common progenitor that gives rise to all hormone-secreting cell types of both anterior and intermediate lobes (PMID:27351100, PMID:26812162). It functions through sequence-specific DNA binding: PROP1 binds preferentially as a dimer to palindromic (inverted) TAAT motifs separated by three nucleotides, and only this dimeric engagement drives transcriptional activation, whereas monomeric binding does not (PMID:19524124). Mutations across the homeodomain (e.g. F88S, R73C) abolish or sharply reduce DNA binding and transactivation, and even C-terminal truncation (W194X) impairs both transactivation and DNA contact, defining the homeodomain and C-terminus as essential for function (PMID:10946881, PMID:15531542, PMID:15941866). Genetically, PROP1 is required for and epistatic to Pit1 (POU1F1) expression (PMID:9462743), and in its absence progenitors are retained in the perilumenal region of Rathke's pouch, fail to migrate to the anterior lobe, and the gland later shows excess apoptosis and reduced proliferation (PMID:12183375, PMID:15591534). Mechanistically PROP1 drives an EMT-like program required for this migration—it is needed for N-cadherin downregulation and Slug expression, and genome-wide it binds epithelial and EMT-inducer genes including Zeb2, Notch2, and Gli2, with Zeb2 activation a key downstream step (PMID:18996108, PMID:27351100); Notch2 expression and retinoic-acid signaling (via Aldh1a2) act downstream, while RA/RARα also feeds back to activate Prop1 transcription (PMID:14732396, PMID:31913463, PMID:29356182). Timely silencing of PROP1 is equally essential: HESX1 and Groucho-related corepressors TLE1/TLE3 directly bind and repress PROP1, and sustained PROP1 expression delays terminal differentiation of gonadotropes and thyrotropes (PMID:11371507, PMID:20181723). Recessive PROP1 loss-of-function mutations cause combined pituitary hormone deficiency in humans, with the cellular basis being failure to seed the anterior lobe with progenitors (PMID:9462743, PMID:15591534).

Mechanistic history

Synthesis pass · year-by-year structured walk · 20 steps
  1. 1998 High

    Established PROP1 as a DNA-binding transcriptional activator acting genetically upstream of Pit1, explaining a distinct dwarfism syndrome.

    Evidence In vitro DNA-binding/transactivation assays of mutant vs wild-type plus genetic epistasis in dwarf mice

    PMID:9462743

    Open questions at the time
    • Did not define the DNA recognition motif
    • Direct downstream target genes not identified
  2. 2000 High

    Mapped a structural requirement for DNA binding by showing helix 1 of the homeodomain is essential, linking patient mutations to molecular loss-of-function.

    Evidence EMSA and luciferase reporter assays with the F88S mutant in TSA-201 cells

    PMID:10946881

    Open questions at the time
    • Single mutation; full homeodomain structure-function not mapped
  3. 2001 High

    Showed that PROP1 must be silenced on schedule, since constitutive overexpression delays differentiation and promotes pituitary lesions.

    Evidence Transgenic mouse gain-of-function with histological and hormonal phenotyping

    PMID:11371507

    Open questions at the time
    • Mechanism of differentiation delay not resolved
    • Repressors mediating silencing not yet identified
  4. 2002 High

    Provided the cellular basis for hypopituitarism by showing Prop1-null progenitors are trapped in Rathke's pouch and fail to migrate, distinct from Lhx4 function.

    Evidence Double-mutant epistasis, pulse-label migration, apoptosis/proliferation assays in mouse embryos

    PMID:12183375

    Open questions at the time
    • Molecular driver of migration failure not yet defined
  5. 2004 High

    Identified Notch2 as a PROP1-dependent pathway component, placing Notch signaling downstream of or parallel to PROP1 in progenitor control.

    Evidence In situ hybridization/immunostaining in Prop1 loss- and gain-of-function mice

    PMID:14732396

    Open questions at the time
    • PROP1 not sufficient for Notch2, so co-factors unknown
    • Direct vs indirect regulation unresolved at this stage
  6. 2004 High

    Quantified the postnatal consequences of failed progenitor seeding (apoptosis, reduced proliferation), mechanistically connecting mouse data to human pituitary hypoplasia.

    Evidence Longitudinal volumetric, BrdU migration, apoptosis and proliferation analyses

    PMID:15591534

    Open questions at the time
    • Transcriptional targets driving the phenotype not identified
  7. 2005 Medium

    Refined the structure-function map by showing the C-terminal transactivation domain also contributes to DNA binding.

    Evidence Transfection transactivation and DNA-binding assays of the W194X truncation

    PMID:15941866

    Open questions at the time
    • Single mutation, single study
    • Structural basis of C-terminal DNA contribution unknown
  8. 2005 Medium

    Showed PROP1 controls gonadotrope timing through a mechanism independent of known gonadotrope transcription factors, nominating new candidate targets.

    Evidence Transgenic overexpression with microarray and hormonal phenotyping

    PMID:16384867

    Open questions at the time
    • Candidate genes Klrg1/Prss28 not validated beyond microarray
    • Direct targets vs secondary effects unresolved
  9. 2007 Medium

    Defined cis-regulatory architecture of Prop1 and confirmed functional sufficiency via complete BAC rescue.

    Evidence Comparative genomics, BAC transgene rescue, enhancer assays, transgenic intronic-element reporter

    PMID:17557180

    Open questions at the time
    • Trans-acting factors at each element not identified here
    • Single lab
  10. 2008 High

    Identified PROP1 as a driver of EMT and migration, showing it is required for N-cadherin downregulation and Slug expression for exit from Rathke's pouch.

    Evidence Prop1/Hes1 double-mutant epistasis with N-cadherin, Slug and hormone marker staining

    PMID:18996108

    Open questions at the time
    • Direct EMT target genes not yet mapped genome-wide
    • Migrational guidance cues unresolved
  11. 2009 High

    Defined the precise DNA recognition mechanism: dimeric binding to inverted TAAT motifs spaced by 3 nt is required for activation.

    Evidence SELEX, EMSA, and reporter assays

    PMID:19524124

    Open questions at the time
    • No co-crystal structure of the PROP1 dimer on DNA
  12. 2009 Medium

    Showed PROP1 can convert HESX1 from a repressive monomer into a heterodimer with altered DNA specificity, linking the two factors biochemically.

    Evidence EMSA with random oligonucleotide selection and HESX1/PROP1 co-incubation

    PMID:19879326

    Open questions at the time
    • Heterodimer function not validated in vivo
    • Single in vitro study
  13. 2009 Medium

    Localized PROP1 to SOX2+ stem/progenitor cells and a stem-cell niche, supporting a role in converting progenitors to the PIT1 lineage.

    Evidence Immunohistochemistry and co-localization across rat developmental stages; in vitro spheroid differentiation of GPS cells

    PMID:19283075 PMID:19442651

    Open questions at the time
    • No functional manipulation in these descriptive studies
    • Single-lab observations
  14. 2010 High

    Identified the corepressor machinery silencing PROP1, showing TLE1/TLE3 and HESX1 directly repress its activity and constitutive HESX1 blocks terminal differentiation.

    Evidence Cell-culture repression and interaction assays plus transgenic HESX1/TLE3 mice

    PMID:20181723

    Open questions at the time
    • Endogenous timing of repressor recruitment not mapped
    • Genomic loci of repression unknown
  15. 2011 Medium

    Confirmed conserved PROP1 function in an orthologous vertebrate and revealed compensatory pathways during late development.

    Evidence Morpholino splice-blocking in zebrafish with in situ hybridization for pit1, prl, gh, lim3

    PMID:21362424

    Open questions at the time
    • Nature of compensation unknown
    • Knockdown not genetic null
  16. 2013 Medium

    Documented the postnatal lineage shift from PROP1+ stem/progenitors to committed PIT1+ progenitors.

    Evidence Immunohistochemistry for NESTIN/PROP1/PIT1 and BrdU across developmental stages

    PMID:23855824

    Open questions at the time
    • Descriptive co-localization without functional perturbation
  17. 2016 High

    Defined the genome-wide PROP1 regulatory network, mechanistically establishing EMT control via direct binding to epithelial and EMT-inducer genes with Zeb2 as a key step.

    Evidence ChIP-seq and RNA-seq in mutant mice, isolated stem cells, and engineered cell lines

    PMID:27351100

    Open questions at the time
    • Direct vs indirect status of individual targets not all dissected
    • Co-factor occupancy not fully mapped
  18. 2016 High

    Lineage tracing established PROP1 as marking a common multipotent progenitor for all anterior and intermediate lobe lineages, not just the PIT1 lineage, and confirmed human PROP1 can substitute functionally.

    Evidence Prop1-Cre lineage tracing with Cre-dependent reporter and human PROP1 rescue

    PMID:26812162

    Open questions at the time
    • Timing of fate restriction within the progenitor pool not resolved
  19. 2018 Medium

    Placed RA/RARα signaling upstream of Prop1 transcription via a defined cis-regulatory element.

    Evidence Ex vivo Rathke's pouch organ culture with RA and luciferase reporter truncation assays

    PMID:29356182

    Open questions at the time
    • In vivo requirement of the RARα element not tested
    • Single lab
  20. 2020 High

    Showed RA signaling also acts downstream of PROP1, with Aldh1a2-dependent RA production required for normal pituitary morphogenesis and hormone expression.

    Evidence Conditional Aldh1a2 knockout and dominant-negative RA-signaling transgenic mice, partially phenocopying Prop1 mutants

    PMID:31913463

    Open questions at the time
    • Direct PROP1 regulation of Aldh1a2 not demonstrated
    • Feed-forward vs feedback architecture of RA-Prop1 loop not fully resolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • How PROP1 dimer occupancy is dynamically switched between activation (with target genes) and repression (with HESX1/TLE) at specific loci during the developmental window remains unresolved.
  • No structural model of PROP1 dimer on inverted TAAT DNA
  • Endogenous timing/loci of HESX1-TLE recruitment vs activation not mapped
  • Full set of direct EMT targets driving migration not defined

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140110 transcription regulator activity 5 GO:0003677 DNA binding 4
Localization
GO:0005634 nucleus 3
Pathway
R-HSA-1266738 Developmental Biology 4 R-HSA-74160 Gene expression (Transcription) 3
Partners
HESX1TLE1TLE3

Evidence

Reading pass · 24 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1998 PROP1 mutations (including frameshift and missense) result in a gene product with reduced DNA-binding and transcriptional activation ability, as demonstrated by in vitro functional assays comparing mutant versus wild-type PROP1 activity; PROP1 is required for Pit1 (POU1F1) expression and is epistatic to Pit1 in pituitary development. In vitro DNA-binding and transcriptional activation assays; genetic epistasis in mouse models (Ames dwarf epistatic to Snell/Jackson dwarfism) Nature genetics High 9462743
2000 The F88S missense mutation in the first helix of the PROP1 homeodomain abolishes DNA binding to a PRDQ9 Prop1 response element in gel shift assays and reduces transcriptional activation of a luciferase reporter to ~34% of wild-type, demonstrating that the hydrophobic core of helix 1 is required for DNA binding. Gel shift (EMSA) assay and luciferase reporter transcriptional activation assay in transiently transfected TSA-201 cells The Journal of clinical endocrinology and metabolism High 10946881
2001 Constitutive (transgenic) overexpression of PROP1 in pituitary thyrotropes and gonadotropes delays terminal differentiation of gonadotropes, causes transient hypogonadotropic hypogonadism, impairs thyrotrope function, and increases susceptibility to pituitary adenomas and Rathke's cleft cysts, demonstrating that timely silencing of PROP1 is required for normal pituitary cell differentiation. Transgenic mouse overexpression; histological and hormonal phenotyping Human molecular genetics High 11371507
2002 In Prop1-deficient mice, anterior pituitary progenitors fail to differentiate and are retained in the perilumenal area of Rathke's pouch, failing to migrate to the anterior lobe; after birth these pituitaries show enhanced apoptosis and reduced proliferation because the anterior lobe is not seeded with progenitors. In contrast, Lhx4 mutants show increased cell death and temporal shift in Lhx3 activation. Double Lhx4/Prop1 mutants exhibit complete failure of all anterior pituitary cell types to differentiate, indicating overlapping but mechanistically distinct roles. Genetic epistasis (double mutants); longitudinal histological analysis; pulse-label cell migration assay; apoptosis and proliferation quantification in mouse embryos Development (Cambridge, England) High 12183375
2004 Longitudinal studies in Prop1-deficient dwarf mice show that mutant pituitary cells are retained in the perilumenal region and fail to migrate and differentiate; after birth the mutant pituitary exhibits enhanced apoptosis and reduced proliferation due to failure to seed the anterior lobe with progenitors, providing a cellular mechanism for the pituitary hypoplasia seen in human PROP1 patients. Longitudinal volumetric analysis; BrdU pulse labeling for cell migration; apoptosis assay; proliferation cell counting in Prop1-deficient and wild-type mice Molecular endocrinology (Baltimore, Md.) High 15591534
2004 Prop1 deficiency in mice results in near-complete absence of Notch2 expression in the developing pituitary, while other panhypopituitary mutants do not show this effect; transgenic overexpression of Prop1 is not sufficient to enhance Notch2 expression, indicating that Prop1 is necessary but not sufficient for Notch2 expression, and that Notch signaling is downstream of or parallel to Prop1 in controlling pituitary progenitor differentiation. In situ hybridization and immunostaining in Prop1 mutant and transgenic mice; analysis of Notch pathway gene expression by spatial/temporal profiling Developmental biology High 14732396
2004 The R73C mutation of PROP1 reduces transactivation capacity to 11.5% of wild-type and abolishes binding to a high-affinity DNA sequence in vitro, confirming R73 as a functionally critical residue in the homeodomain. In vitro transactivation assay and DNA binding (EMSA/gel shift) in transfected cells The Journal of clinical endocrinology and metabolism High 15531542
2005 PROP1 is required for normal gonadotrope terminal differentiation; transgenic overexpression of PROP1 in gonadotropes and thyrotropes causes delayed LH/FSH synthesis without affecting GnRH receptor expression or transcription factors GATA2, NR5A1, TBX19, NR0B1, or TGFβ pathway members, suggesting PROP1 influences gonadotrope development by a distinct mechanism; microarray identified candidate genes Klrg1 and Prss28. Transgenic mouse overexpression; microarray gene expression analysis; immunostaining and hormonal phenotyping Endocrinology Medium 16384867
2008 In Prop1/Hes1 double mutants, cells prematurely differentiate into ACTH and alphaGSU-producing cells within Rathke's pouch; N-cadherin downregulation (required for cell movement out of Rathke's pouch) does not occur in Prop1 single mutants (where N-cadherin remains high), but does occur in double mutants, allowing cells to exit but without correct migrational cues; Slug (EMT marker) is absent in the Prop1 mutant dorsal anterior lobe, indicating Prop1 is required for EMT and cell migration during pituitary organogenesis. Genetic epistasis (Prop1/Hes1 double mutants); immunostaining for N-cadherin, Slug, and pituitary hormones; in situ hybridization Developmental biology High 18996108
2009 HESX1 and PROP1 form a heterodimer on an inverted TAAT motif; HESX1 binds as a monomer to TAATT but not TAAT, and in the presence of PROP1 it develops binding to an inverted TAAT motif via heterodimer formation, suggesting that as PROP1 appears in pituitary development it alters HESX1 from a repressive monomer to a heterodimeric complex with altered DNA-binding specificity. EMSA with random oligonucleotide selection; co-incubation of HESX1 and PROP1 proteins Molecular and cellular endocrinology Medium 19879326
2009 PROP1 binds preferentially as a dimer to palindromic TAAT sequences separated by 3 nucleotides (inverted TAAT motifs); dimeric binding to inverted TAAT motifs drives transcriptional activation, whereas monomeric binding to a single TAAT or inverted ATTA motif does not; SELEX identified the 11-nucleotide consensus binding motif with a required T at position 5. SELEX (Systematic Evolution of Ligands by EXponential enrichment); EMSA; transient transfection reporter assay Molecular and cellular endocrinology High 19524124
2009 PROP1 is expressed in SOX2-expressing stem/progenitor cells in the rat pituitary from embryonic through postnatal periods; at E13.5 PROP1 is expressed in all Rathke's pouch cells; subsequently PROP1-positive cells localize to the marginal cell layer (stem cell niche); PROP1 transiently co-localizes with PIT1 but not with hormones in the embryonic period, indicating a role in converting SOX2+ progenitors to PIT1-lineage committed cells. Immunohistochemistry on rat embryonic and postnatal pituitary sections; co-localization analysis with SOX2, PIT1, and hormone markers Biochemical and biophysical research communications Medium 19442651
2009 GPS (GFRa2/Prop1/Stem) cells in the adult pituitary niche express PROP1 along with stem cell markers (Sox2, Sox9, Oct4) and form non-endocrine spheroids in culture that can differentiate into hormone-producing cells or neurons, indicating PROP1 marks a pituitary stem cell population with neuroectoderm potential. Immunostaining; in vitro spheroid formation and differentiation assay; BrdU label retention; telomere length analysis PloS one Medium 19283075
2010 TLE1 and TLE3 (Groucho-related corepressors) interact with PROP1 and repress PROP1 transcriptional activity independently of HESX1 via direct protein-protein interaction; HESX1 represses PROP1 and this repression is augmented by TLE1 and TLE3; constitutive expression of HESX1 alone (but not TLE3 alone) suppresses terminal differentiation of thyrotropes and gonadotropes in transgenic mice. Cell culture transcriptional repression assay; transgenic mouse model with constitutive HESX1 and/or TLE3 expression; protein-protein interaction assay Molecular endocrinology (Baltimore, Md.) High 20181723
2016 Genome-wide analysis of PROP1 DNA binding and gene expression effects in mutant mice, isolated stem cells, and engineered mouse cell lines revealed that PROP1 stimulates pituitary stem cells to undergo an epithelial-to-mesenchymal transition (EMT)-like process required for cell migration and differentiation; PROP1 binds to epithelial genes (e.g., Claudin 23) and EMT inducer genes (Zeb2, Notch2, Gli2); Zeb2 activation appears to be a key downstream step. ChIP-seq (genome-wide PROP1 DNA binding); RNA-seq in Prop1 mutant mice; functional assays in isolated pituitary stem cells and engineered mouse cell lines eLife High 27351100
2016 Lineage tracing with a Prop1-Cre mouse line demonstrated that all hormone-secreting cell types of both the anterior and intermediate pituitary lobes are descended from Prop1-expressing progenitors, establishing PROP1 as marking a common multipotent progenitor for all pituitary cell lineages including gonadotropes, corticotropes, and melanotropes (not just the PIT1 lineage). Transgenic Prop1-Cre lineage tracing in mice; Cre-dependent reporter expression Endocrinology High 26812162
2005 A novel PROP1 mutation (W194X) truncating the protein in its transactivation domain reduces transactivation capacity to 34.4% of wild-type; unexpectedly, DNA-binding properties are also altered, indicating that the C-terminal end of PROP1 contributes to protein-DNA interaction. Transfection-based transactivation assay; DNA binding assay The Journal of clinical endocrinology and metabolism Medium 15941866
2020 PROP1-dependent retinoic acid (RA) signaling regulates pituitary development: Prop1-mutant mice show reduced Aldh1a2 (the enzyme converting retinaldehyde to RA) expression; conditional deletion of Aldh1a2 or dominant-negative inhibition of RA signaling during pituitary organogenesis partially phenocopies Prop1-mutant mice, including embryonic pituitary dysmorphology and reduced hormone (especially thyrotropin) expression. Conditional mouse knockout (Aldh1a2); dominant-negative transgenic mouse model; gene expression analysis Endocrinology High 31913463
2018 Retinoic acid (RA) signaling through RARα increases Prop1 mRNA expression in Rathke's pouch; ex vivo organ culture and in vitro reporter assays identified a cis-regulatory RARα-responsive element in the 5'-upstream region of the Prop1 gene, establishing RA/RARα as an upstream regulator of Prop1 transcription. Ex vivo Rathke's pouch organ culture with RA treatment; in vitro luciferase reporter assay with serial truncations of Prop1 5' upstream region; in situ hybridization Journal of neuroendocrinology Medium 29356182
2007 Comparative genomics and transgenic functional analysis identified three conserved regulatory elements of Prop1: a promoter-proximal element, an intronic element (first intron), and a downstream element; the intronic element is sufficient to confer dorsal expansion of pituitary expression in transgenic mice, and a BAC transgene containing Prop1 completely rescues the Prop1 mutant phenotype. Comparative genomics; BAC transgene rescue; enhancer assay in cell culture; transgenic mice with intronic element driving reporter Mammalian genome Medium 17557180
2015 Reporter assays showed that SOX2 has cell type-dependent inhibitory and activating functions on Prop1 expression through the proximal and distal upstream regions respectively; RBP-J (Notch mediator) has small regulatory activity; 8 transcription factors (MSX2, PAX6, PIT1, PITX1, PITX2, RPF1, SOX8, SOX11) regulate Prop1 expression from its 3kb upstream region; 10 additional factors (including HES1, HEY1, FOXJ1) show synergy with SOX2. Luciferase reporter assay with 3kb upstream region and 1st intron of Prop1 in CHO and pituitary-related cell lines; systematic screening of 39 transcription factors The Journal of reproduction and development Medium 26640231
2013 NESTIN-expressing rapidly dividing progenitor cells in the pituitary transition from PROP1-positive to PIT1-positive during neonatal development, indicating that the cell supply system shifts from PROP1+ stem/progenitor cells to committed PIT1+ progenitors postnatally. Immunohistochemistry for NESTIN, PROP1, and PIT1 co-expression across developmental stages; BrdU incorporation analysis Journal of neuroendocrinology Medium 23855824
2014 miR-593 and miR-511 directly target the 3'-UTR of the PROP1 gene and attenuate PROP1 protein expression, as validated by western blot and 3'-UTR reporter assay in HEK293T cells. 3'-UTR luciferase reporter assay; western blot after miRNA transfection in HEK293T cells International journal of molecular medicine Medium 25434367
2011 In zebrafish, morpholino-mediated knockdown of prop1 reduces expression of pit1, prolactin (prl), and growth hormone (gh), and also unexpectedly downregulates lim3 (a gene not previously known to be Prop1-dependent); pituitary morphology and cellular organization are abnormal, but the effects are gradually reversed during late development despite persistent splice-blocking, suggesting additional compensatory pathways. Morpholino splice-blocking in zebrafish; in situ hybridization for pit1, prl, gh, tshβ, pitx3, lim3 General and comparative endocrinology Medium 21362424

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
1998 Mutations in PROP1 cause familial combined pituitary hormone deficiency. Nature genetics 382 9462743
1998 The PROP1 2-base pair deletion is a common cause of combined pituitary hormone deficiency. The Journal of clinical endocrinology and metabolism 151 9745452
1998 Phenotypic variability in familial combined pituitary hormone deficiency caused by a PROP1 gene mutation resulting in the substitution of Arg-->Cys at codon 120 (R120C). The Journal of clinical endocrinology and metabolism 143 9768691
2004 Role of PROP1 in pituitary gland growth. Molecular endocrinology (Baltimore, Md.) 141 15591534
1999 "Hot spot" in the PROP1 gene responsible for combined pituitary hormone deficiency. The Journal of clinical endocrinology and metabolism 141 10323394
2009 A GRFa2/Prop1/stem (GPS) cell niche in the pituitary. PloS one 137 19283075
2002 Lhx4 and Prop1 are required for cell survival and expansion of the pituitary primordia. Development (Cambridge, England) 122 12183375
1999 Longitudinal hormonal and pituitary imaging changes in two females with combined pituitary hormone deficiency due to deletion of A301,G302 in the PROP1 gene. The Journal of clinical endocrinology and metabolism 118 10084575
2002 Pituitary magnetic resonance imaging and function in patients with growth hormone deficiency with and without mutations in GHRH-R, GH-1, or PROP-1 genes. The Journal of clinical endocrinology and metabolism 101 12414875
2004 Developmental regulation of Notch signaling genes in the embryonic pituitary: Prop1 deficiency affects Notch2 expression. Developmental biology 97 14732396
1998 Human Prop-1: cloning, mapping, genomic structure. Mutations in familial combined pituitary hormone deficiency. FEBS letters 96 9824293
2001 Persistent Prop1 expression delays gonadotrope differentiation and enhances pituitary tumor susceptibility. Human molecular genetics 95 11371507
2004 PROP1 mutations cause progressive deterioration of anterior pituitary function including adrenal insufficiency: a longitudinal analysis. The Journal of clinical endocrinology and metabolism 93 15472232
1998 Compound heterozygous deletion of the PROP-1 gene in children with combined pituitary hormone deficiency. The Journal of clinical endocrinology and metabolism 92 9661653
2001 PROP1 gene screening in patients with multiple pituitary hormone deficiency reveals two sites of hypermutability and a high incidence of corticotroph deficiency. The Journal of clinical endocrinology and metabolism 89 11549703
1999 Clinical and biochemical phenotype of familial anterior hypopituitarism from mutation of the PROP1 gene. The Journal of clinical endocrinology and metabolism 80 9920061
2000 Adrenocorticotropin deficiency in combined pituitary hormone deficiency patients homozygous for a novel PROP1 deletion. The Journal of clinical endocrinology and metabolism 78 11134108
2009 PROP1 coexists with SOX2 and induces PIT1-commitment cells. Biochemical and biophysical research communications 75 19442651
2001 Longitudinal imaging reveals pituitary enlargement preceding hypoplasia in two brothers with combined pituitary hormone deficiency attributable to PROP1 mutation. The Journal of clinical endocrinology and metabolism 71 11549674
2005 Mutations within the transcription factor PROP1 are rare in a cohort of patients with sporadic combined pituitary hormone deficiency (CPHD). Clinical endocrinology 70 15963055
2016 PROP1 triggers epithelial-mesenchymal transition-like process in pituitary stem cells. eLife 63 27351100
2005 Mutation analysis of POUF-1, PROP-1 and HESX-1 show low frequency of mutations in children with sporadic forms of combined pituitary hormone deficiency and septo-optic dysplasia. Clinical endocrinology 63 15670191
2004 Pituitary magnetic resonance imaging in 15 patients with Prop1 gene mutations: pituitary enlargement may originate from the intermediate lobe. The Journal of clinical endocrinology and metabolism 61 15126542
2002 A unique case of combined pituitary hormone deficiency caused by a PROP1 gene mutation (R120C) associated with normal height and absent puberty. Clinical endocrinology 53 12153609
2016 All Hormone-Producing Cell Types of the Pituitary Intermediate and Anterior Lobes Derive From Prop1-Expressing Progenitors. Endocrinology 52 26812162
2000 Combined pituitary hormone deficiency caused by a novel mutation of a highly conserved residue (F88S) in the homeodomain of PROP-1. The Journal of clinical endocrinology and metabolism 50 10946881
2002 The spectrum of hypopituitarism caused by PROP1 mutations. Best practice & research. Clinical endocrinology & metabolism 48 12464226
2008 Premature differentiation and aberrant movement of pituitary cells lacking both Hes1 and Prop1. Developmental biology 47 18996108
2005 Auxological and endocrine phenotype in a population-based cohort of patients with PROP1 gene defects. European journal of endocrinology 46 16131601
2000 MR imaging of the pituitary gland in children and young adults with congenital combined pituitary hormone deficiency associated with PROP1 mutations. AJR. American journal of roentgenology 46 10658742
2005 An uncommon phenotype with familial central hypogonadism caused by a novel PROP1 gene mutant truncated in the transactivation domain. The Journal of clinical endocrinology and metabolism 43 15941866
1999 Combined pituitary hormone deficiency: role of Pit-1 and Prop-1. Acta paediatrica (Oslo, Norway : 1992). Supplement 43 10626543
1998 A mutational hot spot in the Prop-1 gene in Russian children with combined pituitary hormone deficiency. Pituitary 41 11081182
2000 Molecular analysis of LHX3 and PROP-1 in pituitary hormone deficiency patients with posterior pituitary ectopia. The Journal of clinical endocrinology and metabolism 40 10946868
2000 Central hypocortisolism as part of combined pituitary hormone deficiency due to mutations of PROP-1 gene. European journal of endocrinology 38 11022176
2006 PROP1 gene analysis in Portuguese patients with combined pituitary hormone deficiency. Clinical endocrinology 34 16984240
2003 Clinical characteristics and molecular analysis of PIT1, PROP1,LHX3, and HESX1 in combined pituitary hormone deficiency patients with abnormal pituitary MR imaging. Hormone research 34 14646405
2021 The Prop1-like homeobox gene unc-42 specifies the identity of synaptically connected neurons. eLife 32 34165428
2010 PROP1, HESX1, POU1F1, LHX3 and LHX4 mutation and deletion screening and GH1 P89L and IVS3+1/+2 mutation screening in a Dutch nationwide cohort of patients with combined pituitary hormone deficiency. Hormone research in paediatrics 32 20389107
2008 Molecular analysis of novel PROP1 mutations associated with combined pituitary hormone deficiency (CPHD). Clinical endocrinology 32 19128366
2007 Molecular analysis of PROP1, PIT1, HESX1, LHX3, and LHX4 shows high frequency of PROP1 mutations in patients with familial forms of combined pituitary hormone deficiency. Arquivos brasileiros de endocrinologia e metabologia 32 18157385
2005 Role of prophet of Pit1 (PROP1) in gonadotrope differentiation and puberty. Endocrinology 31 16384867
2012 Short communication: A missense mutation in the PROP1 (prophet of Pit 1) gene affects male fertility and milk production traits in the US Holstein population. Journal of dairy science 30 23245960
2004 A familial form of congenital hypopituitarism due to a PROP1 mutation in a large kindred: phenotypic and in vitro functional studies. The Journal of clinical endocrinology and metabolism 30 15531542
2013 A critical functional missense mutation (H173R) in the bovine PROP1 gene significantly affects growth traits in cattle. Gene 27 24029076
2006 Combined pituitary hormone deficiency (CPHD) due to a complete PROP1 deletion. Clinical endocrinology 27 16918947
2013 High prevalence of PROP1 defects in Lithuania: phenotypic findings in an ethnically homogenous cohort of patients with multiple pituitary hormone deficiency. The Journal of clinical endocrinology and metabolism 26 24178788
2015 Genesis of two most prevalent PROP1 gene variants causing combined pituitary hormone deficiency in 21 populations. European journal of human genetics : EJHG 25 26059845
2010 Corepressors TLE1 and TLE3 interact with HESX1 and PROP1. Molecular endocrinology (Baltimore, Md.) 25 20181723
2020 PROP1-Dependent Retinoic Acid Signaling Regulates Developmental Pituitary Morphogenesis and Hormone Expression. Endocrinology 23 31913463
2011 PROP1 and CTNNB1 expression in adamantinomatous craniopharyngiomas with or without β-catenin mutations. Clinics (Sao Paulo, Brazil) 23 22086512
1999 Combined pituitary hormone deficiency in an inbred Brazilian kindred associated with a mutation in the PROP-1 gene. Molecular genetics and metabolism 23 10329025
2014 Molecular analysis of PROP1, POU1F1, LHX3, and HESX1 in Turkish patients with combined pituitary hormone deficiency: a multicenter study. Endocrine 22 25500790
2006 [Standardization of DNA extraction with NaCl from oral mucosa cells: application in PROP1 gene study]. Arquivos brasileiros de endocrinologia e metabologia 22 16544023
2004 Prolonged jaundice and hypothyroidism as the presenting symptoms in a neonate with a novel Prop1 gene mutation (Q83X). European journal of endocrinology 22 15012608
2013 Rapid transition of NESTIN-expressing dividing cells from PROP1-positive to PIT1-positive advances prenatal pituitary development. Journal of neuroendocrinology 21 23855824
2006 High prevalence of PROP1 gene mutations in Hungarian patients with childhood-onset combined anterior pituitary hormone deficiency. Endocrine 20 17526936
2016 Identification of Novel PROP1 and POU1F1 Mutations in Patients with Combined Pituitary Hormone Deficiency. Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme 18 27756091
2011 Mutations and pituitary morphology in a series of 82 patients with PROP1 gene defects. Hormone research in paediatrics 18 22024773
2002 Pseudotumor of the pituitary due to PROP-1 deletion. Journal of pediatric endocrinology & metabolism : JPEM 18 11822586
2007 Comparative genomics reveals functional transcriptional control sequences in the Prop1 gene. Mammalian genome : official journal of the International Mammalian Genome Society 17 17557180
2010 Identification of SNPs within the sheep PROP1 gene and their effects on wool traits. Molecular biology reports 16 21104025
2008 Pituitary changes in Prop1 transgenic mice: hormone producing tumors and signet-ring type gonadotropes. Acta histochemica et cytochemica 16 18636109
2004 A novel PROP1 gene mutation (157delA) in Japanese siblings with combined anterior pituitary hormone deficiency. Clinical endocrinology 16 15521968
2003 Two new PROP1 gene mutations responsible for compound pituitary hormone deficiency. Clinical genetics 16 12859410
2003 PROP-1 gene mutation (R120C) causing combined pituitary hormone deficiencies with variable clinical course in eight siblings of one Jewish Moroccan family. Hormone research 16 14614227
2002 Familial combined pituitary hormone deficiency caused by PROP-1 gene mutation. Growth patterns and MRI studies in untreated subjects. Hormone research 16 12006708
2013 Three-dimensional studies of Prop1-expressing cells in the rat pituitary just before birth. Cell and tissue research 15 24026438
2018 Retinoic acid signalling is a candidate regulator of the expression of pituitary-specific transcription factor Prop1 in the developing rodent pituitary. Journal of neuroendocrinology 14 29356182
2005 Short-term caloric restriction does not modify the in vivo insulin signaling pathway leading to Akt activation in skeletal muscle of Ames dwarf (Prop1(df)/Prop1(df)) mice. Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme 14 16308835
2000 Expression of the pituitary transcription factor Ptx-1, but not that of the trans-activating factor prop-1, is reduced in human corticotroph adenomas and is associated with decreased alpha-subunit secretion. The Journal of clinical endocrinology and metabolism 14 10902805
2015 Novel Mutations in HESX1 and PROP1 Genes in Combined Pituitary Hormone Deficiency. Hormone research in paediatrics 13 26111865
2012 Frequency of mutations in PROP-1 gene in Turkish children with combined pituitary hormone deficiency. The Turkish journal of pediatrics 13 23692781
2011 Three-dimensional studies of Prop1-expressing cells in the rat pituitary primordium of Rathke's pouch. Cell and tissue research 13 22113335
2011 Genetic background of Prop1(df) mutants provides remarkable protection against hypothyroidism-induced hearing impairment. Journal of the Association for Research in Otolaryngology : JARO 13 22143287
2004 Ovulation induction and successful pregnancy outcome in two patients with Prop1 gene mutations. Fertility and sterility 13 15302300
2017 Molecular analysis of brazilian patients with combined pituitary hormone deficiency and orthotopic posterior pituitary lobe reveals eight different PROP1 alterations with three novel mutations. Clinical endocrinology 12 28734020
2011 Involvement of Prop1 homeobox gene in the early development of fish pituitary gland. General and comparative endocrinology 12 21362424
2008 Basic science and clinical research advances in the pituitary transcription factors: Pit-1 and Prop-1. Current opinion in endocrinology, diabetes, and obesity 12 18594277
2008 A novel missense (A79V) mutation of goat PROP1 gene and its association with production traits. Molecular biology reports 12 19031010
2001 Insufficient adrenarche in patients with combined pituitary hormone deficiency caused by a PROP-1 gene defect. Journal of pediatric endocrinology & metabolism : JPEM 12 11592567
2005 High level expression of Prop-1 gene in gonadotropic cell lines. The Journal of reproduction and development 11 16394624
2002 Adrenocorticotrope deficiency with clinical evidence for late onset in combined pituitary hormone deficiency caused by a homozygous 301-302delAG mutation of the PROP1 gene. Pituitary 11 12812307
2000 Cloning, characterization, and physical mapping of the canine Prop-1 gene (PROP1): exclusion as a candidate for combined pituitary hormone deficiency in German shepherd dogs. Cytogenetics and cell genetics 11 10773688
2003 Long-term follow-up of childhood-onset hypopituitarism in patients with the PROP-1 gene mutation. Hormone research 10 14530604
2018 Gene polymorphisms in PROP1 associated with growth traits in sheep. Gene 9 30316920
2014 Circulating microRNA profiles and the identification of miR-593 and miR-511 which directly target the PROP1 gene in children with combined pituitary hormone deficiency. International journal of molecular medicine 9 25434367
2013 Suprasellar mass mimicking a hypothalamic glioma in a patient with a complete PROP1 deletion. European journal of medical genetics 9 23831233
2010 A large deletion of PROP1 gene in patients with combined pituitary hormone deficiency from two unrelated Chinese pedigrees. Hormone research in paediatrics 9 20395664
2009 Pituitary enlargement in patients with PROP1 gene inactivating mutation represents cystic hyperplasia of the intermediate pituitary lobe. Histopathology and over 10 years follow-up of two patients. Journal of pediatric endocrinology & metabolism : JPEM 9 19774847
2006 Pituitary size fluctuation in long-term MR studies of PROP1 deficient patients: A persistent pathophysiological mechanism? Journal of endocrinological investigation 9 16794371
2015 Two coexisting heterozygous frameshift mutations in PROP1 are responsible for a different phenotype of combined pituitary hormone deficiency. Journal of applied genetics 8 26608600
2013 Isolated central hypothyroidism in young siblings as a manifestation of PROP1 deficiency: clinical impact of whole exome sequencing. Hormone research in paediatrics 8 23652424
2009 Pituitary homeodomain transcription factors HESX1 and PROP1 form a heterodimer on the inverted TAAT motif. Molecular and cellular endocrinology 8 19879326
2009 Ames dwarf (Prop1(df)/Prop1(df)) mice display increased sensitivity of the major GH-signaling pathways in liver and skeletal muscle. Growth hormone & IGF research : official journal of the Growth Hormone Research Society and the International IGF Research Society 8 20022531
2006 Waxing and waning of a pituitary mass in a young woman with combined pituitary hormone deficiency (CPHD) due to a PROP-1 mutation. Pituitary 8 16703408
2006 Congenital combined pituitary hormone deficiency attributable to a novel PROP1 mutation (467insT). Journal of pediatric endocrinology & metabolism : JPEM 8 16759034
2019 Identification of a Novel PROP1 Mutation in a Patient with Combined Pituitary Hormone Deficiency and Enlarged Pituitary. International journal of molecular sciences 7 30988269
2015 Search for regulatory factors of the pituitary-specific transcription factor PROP1 gene. The Journal of reproduction and development 7 26640231
2009 Dimeric PROP1 binding to diverse palindromic TAAT sequences promotes its transcriptional activity. Molecular and cellular endocrinology 7 19524124

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