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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

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

DNA

TYPE	functioning gene
STRUCTURE	229.20 kb     3 Exon(s)

MAPPING

cloned

Y

linked

N

status

provisional

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_020408 3 - 1501 NP_065141 - 91 - 2006 16341090 NM_001164840 3 - 815 NP_001158312 - 130 - 2006 16341090 NM_001164841 4 - 1639 NP_001158313 - 96 - 2006 16341090

EXPRESSION

Type	ubiquitous

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Endocrine neuroendocrine pituitary     highly Lymphoid/Immune tonsils       highly Nervous nerve       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)

Type Gene Modification Chromosome rearrangement Protein expression Protein 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