AlphaFold predicted structure
LYRM4 · Q9HD34
Mean pLDDT
93.1/ 100
Very high
91 residues
Confidence breakdown
- Very high(≥ 90)87%
- Confident(70–90)4%
- Low(50–70)9%
- Very low(< 50)0%
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AlphaFold (Jumper et al., 2021) · CC BY 4.0
LYRM4
Chr 6p25.1LYR motif containing 4
- Aliases:
- CGI-203, ISD11
- MANE:
- ENST00000330636.9
Annotations refreshed 9 hours ago.
Predicted protein structure
Clinical relevance (Genomics England PanelApp)
Diagnostic Grade (Green)
Likely inborn error of metabolism
BIALLELIC, autosomal or pseudoautosomalMitochondrial disorders
BIALLELIC, autosomal or pseudoautosomalPossible mitochondrial disorder - nuclear genes
BIALLELIC, autosomal or pseudoautosomalUndiagnosed metabolic disorders
BIALLELIC, autosomal or pseudoautosomal
Disease associations (Open Targets)
combined oxidative phosphorylation deficiency 19
0.68severe neonatal lactic acidosis due to NFS1-ISD11 complex deficiency
0.62medical procedure
0.24trauma complication
0.24complication
0.24liver disorder
0.21rectosigmoid junction neoplasm
0.21cardiomyopathy
0.21narcolepsy
0.19hypotensive disorder
0.19
Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.
Protein function (UniProt)
LYR motif-containing protein 4
Stabilizing factor, of the core iron-sulfur cluster (ISC) assembly complex, that regulates, in association with NDUFAB1, the stability and the cysteine desulfurase activity of NFS1 and participates in the [2Fe-2S] clusters assembly on the scaffolding protein ISCU (PubMed:17331979, PubMed:31664822). The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner. Then this complex is stabilized by FDX2 which provides reducing equivalents to accomplish the [2Fe-2S] cluster assembly. Finally, the [2Fe-2S] cluster is transferred from ISCU to chaperone proteins, including HSCB, HSPA9 and GLRX5 (By similarity). May also participates in the iron-sulfur protein biogenesis in the cytoplasm through its interaction with the cytoplasmic form of NFS1 (PubMed:19454487)