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MT001181

UniProt Annotations

Entry Information

Gene Name	CDGSH iron sulfur domain 1
Protein Entry	CISD1_HUMAN
UniProt ID	Q9NZ45
Species	Human

Comments

Comment type	Description
Biophysicochemical Properties	Redox potential: E is 0 +/- 10 mV for 2Fe-2S at pH 7.5. {ECO:0000269\|PubMed:19580816};
Cofactor	Name=[2Fe-2S] cluster; Xref=ChEBI:CHEBI:49601; Evidence={ECO:0000269\|PubMed:17376863, ECO:0000269\|PubMed:17584744, ECO:0000269\|PubMed:17766439, ECO:0000269\|PubMed:17766440, ECO:0000269\|PubMed:17905743}; Note=Binds 1 [2Fe-2S] cluster per subunit. The [2Fe-2S] cluster is redox-active and pH labile and is significantly less stable at pH 4.5 as compared with pH 7.0. {ECO:0000269\|PubMed:17376863, ECO:0000269\|PubMed:17584744, ECO:0000269\|PubMed:17766439, ECO:0000269\|PubMed:17766440, ECO:0000269\|PubMed:17905743};
Function	Plays a key role in regulating maximal capacity for electron transport and oxidative phosphorylation (By similarity). May be involved in Fe-S cluster shuttling and/or in redox reactions. {ECO:0000250, ECO:0000269\|PubMed:17584744, ECO:0000269\|PubMed:17766440}.
Induction	Expression is down-regulated by glibenclamide and 5- [(4-carboxyphenyl)methylene]-2-thioxo-3-(3-trifluoromethyl)phenyl- 4-thiazolidinone (CFTR(inh)172), and up-regulated by cAMP/isoproterenol/IBMX, components that inhibit and stimulate chloride transport activity respectively. {ECO:0000269\|PubMed:18047834}.
Miscellaneous	Binds pioglitazone, an anti-diabetes drug. Binding increases the stability of the 2Fe-2S cluster.
Similarity	Belongs to the CISD protein family. {ECO:0000305}.
Subcellular Location	Mitochondrion outer membrane {ECO:0000269\|PubMed:17376863, ECO:0000269\|PubMed:17766439, ECO:0000269\|PubMed:17905743, ECO:0000269\|PubMed:18047834}; Single- pass type III membrane protein {ECO:0000269\|PubMed:17376863, ECO:0000269\|PubMed:17766439, ECO:0000269\|PubMed:17905743, ECO:0000269\|PubMed:18047834}.
Subunit	Homodimer. {ECO:0000269\|PubMed:17766439, ECO:0000269\|PubMed:17766440, ECO:0000269\|PubMed:17905743}.
Tissue Specificity	Expression is reduced in cells derived from cystic fibrosis patients. {ECO:0000269\|PubMed:18047834}.