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FLASH GENE
Symbol FNIP1 contributors: mct - updated : 17-05-2021
HGNC name folliculin interacting protein 1
HGNC id 29418
DNA
TYPE functioning gene
STRUCTURE 155.35 kb     17 Exon(s)
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
18 - 6655 - 1166 - 2020 32181500
17 - 6571 - 1138 - 2020 32181500
17 - 6433 - 1121 - 2020 32181500
13 - 1728 - 508 - 2020 32181500
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart   highly
Digestiveliver   highly
Reproductivefemale systemplacenta  highly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / hematopoieticbone marrow  highly
Connectiveadipose  highly
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
HOMOLOGY
Homologene
FAMILY
CATEGORY adaptor
SUBCELLULAR LOCALIZATION     plasma membrane
    intracellular
intracellular,cytoplasm,organelle,membrane
intracellular,cytoplasm,organelle,lysosome
intracellular,cytoplasm,cytosolic
intracellular,nucleus
basic FUNCTION
  • having an important function in modulating energy/nutrient-sensing signaling pathways
  • is vital for B cell development and metabolic homeostasis
  • is an adaptor protein that physically interacts with AMPK, an energy-sensing kinase that stimulates mitochondrial biogenesis and autophagy in response to low ATP
  • FNIP1 is vital for invariant natural killer T (iNKT) cell development by maintaining metabolic homeostasis in response to metabolic stress
  • FNIP1 controls skeletal muscle fiber type specification
  • roles for FNIP1 in regulating kidney development and function
  • intrinsic role of FNIP1 as a crucial factor in the concerted regulation of mitochondrial function and muscle fiber type that determine muscle fitness
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    energy/nutrient-sensing signaling pathways
    a component
  • part of of FLCN-AMPK-FNIP complex which is regulated by Ser62 phosphorylation
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • with FLCN, through the C-terminus of FLCN, and colocalize in the cytoplasm
  • FLCN-GABARAP association is modulated by the presence of either folliculin-interacting protein FNIP1 or FNIP2 and further regulated by ULK1
  • role for FNIP1 as a negative regulator of AMP
  • FNIP1 modulates autophagy and energy response pathways in part through the regulation of AMPK, MTOR, and TFE3 in B cell progenitors
  • FNIP1 exerts control upon muscle mitochondrial oxidative program through AMPK signaling
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional germinal mutation      
    is associated with B-cell deficiency, cardiomyopathy, and elevated AMPK activity
    constitutional       loss of function
    FNIP1 disruption resulted in many cellular and molecular changes previously implicated in the development of PKD
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Fnip1(-/-) mice develop normally, are not susceptible to kidney neoplasia, but display a striking pro-B cell block that is entirely independent of mTOR activity
  • loss of Fnip1 profoundly mitigated muscle damage in a murine model of Duchenne muscular dystrophy