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LYR Motif Containing Protein 4 (LYRM4)

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CGI-203; ISD11; C6orf149

LYR Motif Containing Protein 4 (LYRM4)
CCC N958
HGNC 21365
MGI 2683538
PubMed 23069351
UniProt Q9HD34
The deduced 91-amino acid human protein has a highly conserved LYR motif near its N terminus. Human ISD11 formed a stable complex in vivo with the human cysteine desulfurase (NFS1), which generates the inorganic sulfur needed for Fe/S protein biogenesis. Similar to NFS1, ISD11 localized to the mitochondrial compartment, but also to the nucleus of mammalian cells. Suppression of human ISD11 by siRNA inactivated mitochondrial and cytosolic aconitases. In addition, ISD11 suppression activated iron-responsive element-binding activity of iron regulatory protein-1 (ACO1), increased protein levels of iron regulatory protein-2 (IREB2), and resulted in abnormal punctate ferric iron accumulations in cells. ISD11 is important in the biogenesis of Fe/S clusters in mammalian cells, and its loss disrupts normal mitochondrial and cytosolic iron homeostasis.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant LYR Motif Containing Protein 4 (LYRM4) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to LYR Motif Containing Protein 4 (LYRM4) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to LYR Motif Containing Protein 4 (LYRM4) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for LYR Motif Containing Protein 4 (LYRM4) CLIA Kit Customized Service Offer
n/a ELISA Kit for LYR Motif Containing Protein 4 (LYRM4) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant LYR Motif Containing Protein 4 (LYRM4) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to LYR Motif Containing Protein 4 (LYRM4) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to LYR Motif Containing Protein 4 (LYRM4) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for LYR Motif Containing Protein 4 (LYRM4) CLIA Kit Customized Service Offer
n/a ELISA Kit for LYR Motif Containing Protein 4 (LYRM4) ELISA Kit Customized Service Offer
  1. "Identification of the human crooked neck gene by comparative gene identification."Biochim. Biophys. Acta 1517:449-454(2001) [PubMed] [Europe PMC] [Abstract]
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  3. "The DNA sequence and analysis of human chromosome 6." Nature 425:805-811(2003) [PubMed] [Europe PMC] [Abstract]
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  5. "Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones."Hum. Mol. Genet. 16:929-941(2007) [PubMed] [Europe PMC] [Abstract]
  6. "A novel role for human Nfs1 in the cytoplasm: Nfs1 acts as a sulfur donor for MOCS3, a protein involved in molybdenum cofactor biosynthesis."J. Biol. Chem. 283:25178-25185(2008) [PubMed] [Europe PMC] [Abstract]
  7. "Human ISD11 is essential for both iron-sulfur cluster assembly and maintenance of normal cellular iron homeostasis."Hum. Mol. Genet. 18:3014-3025(2009) [PubMed] [Europe PMC] [Abstract]
  8. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  9. "Mutations in LYRM4, encoding iron-sulfur cluster biogenesis factor ISD11, cause deficiency of multiple respiratory chain complexes."Hum. Mol. Genet. 22:4460-4473(2013) [PubMed] [Europe PMC] [Abstract]