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FLASH GENE
Symbol PIBF1 contributors: mct - updated : 12-03-2018
HGNC name progesterone immunomodulatory binding factor 1
HGNC id 23352
Corresponding disease
JBTS33 Joubert syndrome 33
Location 13q22.1      Physical location : 73.356.229 - 73.590.589
Synonym name
  • progesterone-induced blocking factor
  • chromosome 13 open reading frame 24
  • centrosomal protein of 90 kDa
    • Synonym symbol(s) C13orf24, RP11-505F3.1, PIBF, KIAA1008, CEP90, JBTS33
    DNA
    TYPE functioning gene
    STRUCTURE 234.40 kb     18 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    text shorter 34kDa and 34 kDa active form
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    18 splicing 3114 89 757 - 2004 15305375
    - splicing 750 - 103 - 2004 15305375
    - splicing 1098 25 216 - 2004 15305375
    19 - 3285 - 786 - 2017 27918564
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularvessel   lowly
    Digestiveintestinesmall intestine  highly
     liver   predominantly
    Reproductivemale systemtestis  moderately
    Respiratoryrespiratory tractlarynx  lowly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES Hydrophilic
    STRUCTURE
    motifs/domains
  • N-terminal signal sequence
  • a nuclear localization signal
  • a leucine zipper motif
  • a basic zipper sequence
  • a PEST sequence
  • an endoplasmic reticulum membrane retention signal
  • several potential N-glycosylation and phosphorylation sites
    • secondary structure alpha-helical protein
    isoforms Precursor
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY regulatory , signaling
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    intracellular,cytoplasm,cytoskeleton,microtubule,centrosome
    intracellular,nucleus
    text
  • component of pericentriolar satellites, and is present both in the centrosome and in the cytoplasm, but is transiently concentrated at the centrosome once cells enter mitosis
    • basic FUNCTION
  • mediator of progesterone that by acting on the phospholipase A2 enzyme interferes with arachidonic acid metabolism, induces a Th2 biased immune response, and by controlling NK activity exerts an anti-abortive effect
  • is a molecule with inhibitory effects on cell-mediated immune reactions
  • is crucial for maintaining the integrity of spindle poles during mitosis
  • likely PIBF1 affects the expression of leptin and its receptor, and PIBF1 expression is inversely related to trophoblast invasiveness
  • is a centriolar satellite protein that is critical for spindle pole integrity in mitotic cells
  • KISS1, KISS1R play a major role in trophoblast invasion, and progesterone-induced blocking factor (PIBF1) is needed for maintaining pregnancy
  • is an immunomodulatory protein with known role in the regulation of the immune response in the reproductive system
  • vital regulatory cascade involving IL33, decidual B cells and PIBF1 in safeguarding term pregnancy
  • promotes likely proliferation, migration, and invasion of human glioblastoma cells
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • PIBF1 acts on lymphocytes in pregnancy to induce a type 1 to type 2 cytokine shift by upregulating the production of type 2 cytokines
  • physically interacts with PCM1 at centriolar satellites, and this interaction is essential for centrosomal accumulation of the centriolar satellites and eventually for primary cilia formation
  • PIBF1 is also required for BBS4 loading on centriolar satellites and its localization in primary cilia
  • PIBF1 expression by B cells is induced by the mucosal alarmin IL33
    • cell & other
    REGULATION
    induced by progesterone
    ASSOCIATED DISORDERS
    corresponding disease(s) JBTS33
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    decreased KISS1 and PIBF1 expressions in trophoblasts and deciduas are associated with recurrent spontaneous abortion (RSA)
    constitutional     --low  
    spindle pole fragmentation was the most characteristic phenotype in PIBF1-depleted cells
    Susceptibility
    Variant & Polymorphism
    Candidate gene
  • for treatment of reccurent aborting (failing in women with recurrent abortion)
    • Marker
  • pregnancy termination can be predicted by lower than normal pregnancy PIBF1 values within 5 days before labor and can contribute to the diagnosis of preterm birth
  • preterm birth may be predictable at 24-28 gestational week by lower than normal pregnancy PIBF1 values and measurement of PIBF1 concentration in biological fluids at that time may be of importance in clinical practice
    • Therapy target
    ANIMAL & CELL MODELS
  • B cell deficiency in mice resulted in a lower uterine level of active progesterone-induced blocking factor 1 (Pibf1), and therapeutic administration of Pibf1 mitigated spontaneous preterm labor (PTL) and uterine inflammation in B cell-deficient mice