LYRM4 - LYR motif containing 4 Gene

Also Known as ISD11; CGI-203; COXPD19; C6orf149

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 57128

About LYRM4

Cytogenetic location: 6p25.1 Genomic coordinates (GRCh38): 6:5,031,753-5,260,950 (from NCBI)

This gene has 9 transcripts (splice variants), 143 orthologues, 1 paralogue and is associated with 2 phenotypes. Ubiquitous expression in ovary (RPKM 2.3), thyroid (RPKM 2.1) and 25 other tissues.

Summary

The protein encoded by this gene is found in both mitochondria and the nucleus, where it binds cysteine desulfurase and helps free inorganic sulfur for Fe/S clusters. Disruption of this gene negatively impacts mitochondrial and cytosolic iron homeostasis. [provided by RefSeq, Sep 2016]

LYRM4 Products (5)

mRNA Protein Name
NM_001164840.3 NP_001158312.1 LYR motif-containing protein 4 isoform 2
NM_001164841.3 NP_001158313.1 LYR motif-containing protein 4 isoform 3
NM_001318782.1 NP_001305711.1 LYR motif-containing protein 4 isoform 4
NM_001318783.1 NP_001305712.1 LYR motif-containing protein 4 isoform 5
NM_020408.6 NP_065141.3 LYR motif-containing protein 4 isoform 1
Molecular Function GO Annotation Evidence References Source
enables protein binding IPI
IPI: Inferred from physical interaction
18650437 GOA
enables protein homodimerization activity IDA
IDA: Inferred from direct assay
29097656 GOA
Biological Process GO Annotation Evidence References Source
involved in [2Fe-2S] cluster assembly IDA
IDA: Inferred from direct assay
17331979 GOA
Cellular Component GO Annotation Evidence References Source
part of mitochondrial [2Fe-2S] assembly complex IDA
IDA: Inferred from direct assay
21298097 GOA
located in mitochondrion IDA
IDA: Inferred from direct assay
17331979 GOA
located in nucleus IDA
IDA: Inferred from direct assay
23593335 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

LYRM4 Protein Structure

Complex1_LYR_1

Complex1_LYR_1: Complex1_LYR-like (8 - 66)

  • 0
  • 91 a.a.
Protein Preferred Names Protein Names

LYR motif-containing protein 4

  • homolog of yeast Isd11

LYRM4 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method References
Intra
LYRM4 Q9HD34 NFS1 Homo sapiens Q9Y697 26342079
Intra
LYRM4 Q9HD34 NFS1 Homo sapiens Q9Y697 27499296
Intra
LYRM4 Q9HD34 NFS1 Homo sapiens Q9Y697 33961781
Intra
LYRM4 Q9HD34 NFS1 Homo sapiens Q9Y697 26342079
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases Alias
Combined Oxidative Phosphorylation Deficiency 19
  • COXPD19

  • Severe Neonatal Lactic Acidosis Due To Nfs1-Isd11 Complex Deficiency

  • Combined Oxidative Phosphorylation Deficiency, Type 19

Chromosome 6pter-P24 Deletion Syndrome
  • 6p Subtelomeric Deletion Syndrome

  • 6p25 Microdeletion Syndrome

  • Distal Monosomy 6p

  • Distal Deletion 6p

  • Monosomy 6p25

Friedreich Ataxia
  • Friedreich Ataxia 1

  • FRDA

  • Friedreich Ataxia With Retained Reflexes

  • Frda1

  • Fa

  • Friedreich'S Ataxia

  • Hereditary Spinal Ataxia

  • Fa1

  • Friedreich'S Tabes

  • Hereditary Spinal Sclerosis

  • Spinocerebellar Ataxia, Friedreich

  • Friedreich Spinocerebellar Ataxia

  • Friedrich'S Ataxia

Combined Oxidative Phosphorylation Deficiency
Multiple Mitochondrial Dysfunctions Syndrome 3
  • MMDS3

  • Iba57 Deficiency

  • Multiple Mitochondrial Dysfunctions Syndrome Type 3

  • Mitochondrial Dysfunctions, Multiple, Syndrome, Type 3

Lactic Acidosis
  • Acidosis, Lactic

  • Acidosis Lactic

Autosomal Recessive Cerebellar Ataxia
  • Arca

Hereditary Ataxia
  • Sca

  • Spinocerebellar Ataxia

  • Ataxias Hereditary

  • Ataxias, Hereditary

Cerebellar Disease
  • Cerebellar Diseases

  • Cerebellar Dysfunction

  • Cerebellar Abnormality

  • Cerebellar Disorders

Gastroesophageal Reflux
  • Gastroesophageal Reflux Disease

  • Gerd

  • GER

  • Gastroesophageal Reflux, Pediatric

  • Acid Reflux

  • Gastresophageal Reflux

  • Gastro-Esophageal Reflux

  • Gerd - Gastro-Esophageal Reflux Disease

3-Methylglutaconic Aciduria, Type Iii
  • Optic Atrophy

  • 3-Methylglutaconic Aciduria Type 3

  • Costeff Syndrome

  • Mga3

  • Costeff Optic Atrophy Syndrome

  • Optic Atrophy Plus Syndrome

  • Infantile Optic Atrophy With Chorea And Spastic Paraplegia

  • 3-Methylglutaconic Aciduria Type Iii

  • Autosomal Recessive Optic Atrophy Plus Syndrome

  • Autosomal Recessive Optic Atrophy Type 3

  • Opa3 Defect

  • MGCA3

  • Mga, Type Iii

  • Iraqi Jewish Optic Atrophy Plus

  • Mga Type Iii

  • Optic Atrophy, Infantile, With Chorea And Spastic Paraplegia

  • Iraqi-Jewish 'Optic Atrophy Plus'

  • Optic Atrophy 3, Autosomal Recessive

  • Opa3, Autosomal Recessive

  • Opa3-Related 3-Methylglutaconic Aciduria

  • Iraqi-Jewish Optic Atrophy Plus

  • Atrophy Of Optic Disc

  • 3-Alpha Methylglutaconic Aciduria Type Iii

  • Optic Atrophy 3

  • Optic Atrophy Infantile With Chorea And Spastic Paraplegia

  • Autosomal Recessive Opa3

  • Autosomal Recessive Optic Atrophy 3

  • 3-Methylglutaconic Aciduria 3

  • 3-Alpha-Methylglutaconic Aciduria Type 3

  • Optic Atrophy 3 Autosomal Recessive

  • Atrophy, Optic

  • Atrophy, Optic, Plus Syndrome

  • Optic Nerve Atrophy

  • Primary Optic Atrophy

  • Oa - [Optic Atrophy]

  • Second Cranial Nerve Atrophy

  • Second Cranium Nerve Atrophy

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Bos taurus LYRM4 VGNC VGNC:31110
Rattus norvegicus LYRM4 RGD RGD:7672286
Mus musculus LYRM4 MGD MGI:2683538
Others LYRM4 NCBI