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FLASH GENE
Symbol LYRM4 contributors: mct/npt/pgu - updated : 09-05-2014
HGNC name LYR motif containing 4
HGNC id 21365
Corresponding disease
COXPD19 combined oxidative phosphorylation deficiency 19
Location 6p25.1      Physical location : 5.108.653 - 5.261.172
Synonym name
  • chromosome 6 open reading frame 149
  • mitochondrial matrix Nfs1 interacting protein
  • homolog of yeast Isd11
    • Synonym symbol(s) CGI-203, C6orf149, ISD11
    DNA
    TYPE functioning gene
    STRUCTURE 229.20 kb     3 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    3 - 1501 - 91 - 2006 16341090
    3 - 815 - 130 - 2006 16341090
    4 - 1639 - 96 - 2006 16341090
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrineneuroendocrinepituitary  highly
    Lymphoid/Immunetonsils   highly
    Nervousnerve   highly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • conserved tripeptide &
    • 65533;LYR&
    65533; near the N-terminus
    HOMOLOGY
    intraspecies homolog to HSPA9B
    Homologene
    FAMILY
  • complex I LYR family
    • CATEGORY chaperone/stress
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,nucleus
    text
  • colocalizes with frataxin and is targeted to the mitochondria
  • localizes to the mitochondrial compartment, as expected, but also to the nucleus
    • basic FUNCTION
  • acting as an adaptor between frataxin and NFS1/ISCU
  • required for the assembly of Fe/S proteins, and acting together with NFS1 in an early step in the biogenesis of Fe/S proteins
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • component of the eukaryotic complex NFS1/ISCU, an essential component of iron-sulfur cluster biogenesis
  • form a tight complex with NFS1, thus stabilizing NFS1 and preventing its aggregation
  • complex SDU and SDUF comprised of NFS1, LYRM4, and ISCU and NFS1, LYRM4, ISCU, and FXN, respectively (SDUF is capable of synthesizing Fe/S cluster)
    • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacting with FXN (binds the iron
    • sulfur biogenesis NFS1/ISCU complex through LYRM4, this interaction is nickel-dependent, and multiple consequences of frataxin deficiency are duplicated by LYRM4 deficiency)
  • interacting with NFS1 (acts as an adaptor of NFS1 by forming a tight complex that helps ISCS to fulfill the cysteine desulfurase function in the Fe/S cluster biosynthesis either in mitochondria or in the nuclear/cytosolic compartment of cells)
  • LYRM4 has a profound effect on the oligomeric state of NFS1
    • cell & other
    REGULATION
    activated by ISCU and FXN that stimulate NFS1 and LYRM4 cysteine desulfurase activity
    ASSOCIATED DISORDERS
    corresponding disease(s) COXPD19
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    in FRDA patient cells
    constitutional       loss of function
    disrupts normal mitochondrial and cytosolic iron homeostasis
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Lyrm4 was down-regulated in the prefrontal cortex of mice with microdeletions in the locus syntenic to 22q11.2 patients affected by schizophrenia