======= AUH =======
== Gene Information ==
* **Official Symbol**: AUH
* **Official Name**: AU RNA binding methylglutaconyl-CoA hydratase
* **Aliases and Previous Symbols**: N/A
* **Entrez ID**: [[https://www.ncbi.nlm.nih.gov/gene/?term=549|549]]
* **UniProt**: [[https://www.uniprot.org/uniprot/Q13825|Q13825]]
* **Interactions**: [[https://thebiogrid.org/search.php?search=AUH&organism=9606|BioGRID]]
* **PubMed articles**: [[https://www.ncbi.nlm.nih.gov/pubmed/?term=gene%20AUH|Open PubMed]]
* **OMIM**: [[https://omim.org/entry/600529|Open OMIM]]
== Function Summary ==
* **Entrez Summary**: This gene encodes bifunctional mitochondrial protein that has both RNA-binding and hydratase activities. The encoded protein is a methylglutaconyl-CoA hydratase that catalyzes the hydration of 3-methylglutaconyl-CoA to 3-hydroxy-3-methyl-glutaryl-CoA, a critical step in the leucine degradation pathway. This protein also binds AU-rich elements (AREs) found in the 3' UTRs of rapidly decaying mRNAs including c-fos, c-myc and granulocyte/ macrophage colony stimulating factor. ARE elements are involved in directing RNA to rapid degradation and deadenylation. This protein is localizes to the mitochondrial matrix and the inner mitochondrial membrane and may be involved in mitochondrial protein synthesis. Mutations in this gene are the cause of 3-methylglutaconic aciduria, type I. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2015].
* **UniProt Summary**: Catalyzes the conversion of 3-methylglutaconyl-CoA to 3- hydroxy-3-methylglutaryl-CoA (PubMed:11738050, PubMed:12434311, PubMed:12655555). Also has itaconyl-CoA hydratase activity by converting itaconyl-CoA into citramalyl-CoA in the C5- dicarboxylate catabolism pathway (PubMed:29056341). The C5- dicarboxylate catabolism pathway is required to detoxify itaconate, a vitamin B12-poisoning metabolite (PubMed:29056341). Has very low enoyl-CoA hydratase activity (PubMed:7892223). Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs (PubMed:7892223). {ECO:0000269|PubMed:11738050, ECO:0000269|PubMed:12434311, ECO:0000269|PubMed:12655555, ECO:0000269|PubMed:7892223, ECO:0000303|PubMed:29056341}.
|ECH|
|methylglutaconyl-CoA hydratase activity|
|itaconyl-CoA hydratase activity|
|leucine catabolic process|
|leucine metabolic process|
|enoyl-CoA hydratase activity|
|branched-chain amino acid catabolic process|
|branched-chain amino acid metabolic process|
|fatty acid beta-oxidation|
|mRNA 3-UTR binding|
|fatty acid oxidation|
|lipid oxidation|
|fatty acid catabolic process|
|alpha-amino acid catabolic process|
|monocarboxylic acid catabolic process|
|cellular amino acid catabolic process|
|cellular lipid catabolic process|
|lipid modification|
|alpha-amino acid metabolic process|
|organic acid catabolic process|
|carboxylic acid catabolic process|
|lipid catabolic process|
|cellular amino acid metabolic process|
|fatty acid metabolic process|
|mitochondrial matrix|
|small molecule catabolic process|
|monocarboxylic acid metabolic process|
|carboxylic acid metabolic process|
|cellular lipid metabolic process|
|oxidation-reduction process|
|oxoacid metabolic process|
|organic acid metabolic process|
|organonitrogen compound catabolic process|
|lipid metabolic process|
|mitochondrion|
|small molecule metabolic process|
|organic substance catabolic process|
|cellular catabolic process|
\\
=== CRISPR Data ===
^Screen^Score^
|[[:results:exp102|Nifuroxazide 5μM R03 exp102]]|-2.41|
|[[:results:exp449|Arsenic trioxide 60μM R08 exp449]]|1.81|
No correlation found to any other genes in chemogenomics.
Global Fraction of Cell Lines Where Essential: 0/739
^Tissue^Fraction Of Cell Lines Where Essential^
|1290807.0|0/1|
|909776.0|0/1|
|bile duct|0/28|
|blood|0/28|
|bone|0/26|
|breast|0/33|
|central nervous system|0/56|
|cervix|0/4|
|colorectal|0/17|
|esophagus|0/13|
|fibroblast|0/1|
|gastric|0/16|
|kidney|0/21|
|liver|0/20|
|lung|0/75|
|lymphocyte|0/16|
|ovary|0/26|
|pancreas|0/24|
|peripheral nervous system|0/16|
|plasma cell|0/15|
|prostate|0/1|
|skin|0/24|
|soft tissue|0/9|
|thyroid|0/2|
|upper aerodigestive|0/22|
|urinary tract|0/29|
|uterus|0/5|
== Essentiality in NALM6 ==
* **Essentiality Rank**: 13536
* **Expression level (log2 read counts)**: 3.42
{{:chemogenomics:nalm6 dist.png?nolink |}}