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FLASH GENE
Symbol FNIP1 contributors: mct - updated : 17-05-2021
HGNC name folliculin interacting protein 1
HGNC id 29418
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