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Protein

CDGSH iron-sulfur domain-containing protein 1

Gene

CISD1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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.By similarity2 Publications

Miscellaneous

Binds pioglitazone, an anti-diabetes drug. Binding increases the stability of the 2Fe-2S cluster.

Cofactori

[2Fe-2S] cluster5 PublicationsNote: 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 7.0.5 Publications

Redox potential

E is 0 +/- 10 mV for 2Fe-2S at pH 7.5.1 Publication

Manual assertion based on experiment ini

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi72Iron-sulfur (2Fe-2S)1
Metal bindingi74Iron-sulfur (2Fe-2S)1
Metal bindingi83Iron-sulfur (2Fe-2S)1
Metal bindingi87Iron-sulfur (2Fe-2S); via pros nitrogen1

GO - Molecular functioni

  • 2 iron, 2 sulfur cluster binding Source: UniProtKB-KW
  • identical protein binding Source: IntAct
  • metal ion binding Source: UniProtKB-KW

GO - Biological processi

Keywordsi

Ligand2Fe-2S, Iron, Iron-sulfur, Metal-binding

Names & Taxonomyi

Protein namesi
Recommended name:
CDGSH iron-sulfur domain-containing protein 1
Alternative name(s):
MitoNEET
Gene namesi
Name:CISD1
Synonyms:C10orf70, ZCD1
ORF Names:MDS029
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 10

Organism-specific databases

EuPathDBiHostDB:ENSG00000122873.11
HGNCiHGNC:30880 CISD1
MIMi611932 gene
neXtProtiNX_Q9NZ45

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei14 – 31Helical; Signal-anchor for type III membrane proteinSequence analysisAdd BLAST18
Topological domaini32 – 108CytoplasmicSequence analysisAdd BLAST77

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion outer membrane

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi72C → S: Abolishes absorption in the 300-500 nm range. 1 Publication1
Mutagenesisi74C → S: Abolishes absorption in the 300-500 nm range. 1 Publication1
Mutagenesisi83C → S: Abolishes absorption in the 300-500 nm range. 1 Publication1
Mutagenesisi84D → N: Does not affect absorption in the 300-500 nm range. 1 Publication1
Mutagenesisi87H → C: Affects absorption in the 300-500 nm range but it is not reduced. Increased stability of the 2Fe-2S cluster at low pH. 1 Publication1
Mutagenesisi87H → Q: Abolishes absorption in the 300-500 nm range. 1 Publication1

Organism-specific databases

DisGeNETi55847
OpenTargetsiENSG00000122873
PharmGKBiPA162382289

Chemistry databases

ChEMBLiCHEMBL1795168

Polymorphism and mutation databases

BioMutaiCISD1
DMDMi25453105

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00000898032 – 108CDGSH iron-sulfur domain-containing protein 1Add BLAST107

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylserineCombined sources1
Cross-linki42Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Modified residuei55N6-acetyllysine; alternateBy similarity1
Cross-linki55Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin); alternate1 Publication
Modified residuei68N6-acetyllysine; alternateBy similarity1
Cross-linki68Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin); alternate1 Publication
Cross-linki78Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki79Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki89Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Modified residuei104N6-acetyllysine; alternateBy similarity1
Cross-linki104Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin); alternate1 Publication
Cross-linki105Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki106Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication

Post-translational modificationi

Ubiquitinated by PRKN during mitophagy, leading to its degradation and enhancement of mitophagy. Deubiquitinated by USP30.1 Publication

Keywords - PTMi

Acetylation, Isopeptide bond, Ubl conjugation

Proteomic databases

EPDiQ9NZ45
MaxQBiQ9NZ45
PaxDbiQ9NZ45
PeptideAtlasiQ9NZ45
PRIDEiQ9NZ45
TopDownProteomicsiQ9NZ45

PTM databases

iPTMnetiQ9NZ45
PhosphoSitePlusiQ9NZ45
SwissPalmiQ9NZ45

Expressioni

Tissue specificityi

Expression is reduced in cells derived from cystic fibrosis patients.1 Publication

Inductioni

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.1 Publication

Gene expression databases

BgeeiENSG00000122873
CleanExiHS_CISD1
ExpressionAtlasiQ9NZ45 baseline and differential
GenevisibleiQ9NZ45 HS

Organism-specific databases

HPAiCAB045965
HPA074383

Interactioni

Subunit structurei

Homodimer.3 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
itself4EBI-1050885,EBI-1050885

GO - Molecular functioni

  • identical protein binding Source: IntAct

Protein-protein interaction databases

BioGridi120949, 30 interactors
DIPiDIP-46446N
IntActiQ9NZ45, 5 interactors
STRINGi9606.ENSP00000363041

Chemistry databases

BindingDBiQ9NZ45

Structurei

Secondary structure

1108
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi47 – 49Combined sources3
Beta strandi53 – 66Combined sources14
Beta strandi68 – 71Combined sources4
Beta strandi73 – 75Combined sources3
Turni78 – 81Combined sources4
Helixi86 – 94Combined sources9
Beta strandi98 – 104Combined sources7

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2QD0X-ray1.81A/B32-108[»]
2QH7X-ray1.50A/B33-108[»]
2R13X-ray1.80A33-108[»]
3EW0X-ray1.40A/B33-108[»]
3LPQX-ray1.70A/B33-107[»]
3REEX-ray1.76A32-108[»]
4EZFX-ray1.19A/B33-108[»]
4F1EX-ray2.40A/B/C/D/E/F/G/H/I/J/K/L/M/N/O/P/Q/R33-108[»]
4F28X-ray1.55A/B33-108[»]
4F2CX-ray1.35A/B33-108[»]
ProteinModelPortaliQ9NZ45
SMRiQ9NZ45
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ9NZ45

Family & Domainsi

Sequence similaritiesi

Belongs to the CISD protein family.Curated

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3461 Eukaryota
ENOG41122II LUCA
GeneTreeiENSGT00390000001233
HOGENOMiHOG000242301
HOVERGENiHBG052444
InParanoidiQ9NZ45
OMAiKDHRNKS
OrthoDBiEOG091G0XB4
PhylomeDBiQ9NZ45
TreeFamiTF324661

Family and domain databases

InterProiView protein in InterPro
IPR018967 FeS-contain_CDGSH-typ
IPR019610 FeS-contain_mitoNEET_N
PfamiView protein in Pfam
PF10660 MitoNEET_N, 1 hit
PF09360 zf-CDGSH, 1 hit
SMARTiView protein in SMART
SM00704 ZnF_CDGSH, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q9NZ45-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSLTSSSSVR VEWIAAVTIA AGTAAIGYLA YKRFYVKDHR NKAMINLHIQ
60 70 80 90 100
KDNPKIVHAF DMEDLGDKAV YCRCWRSKKF PFCDGAHTKH NEETGDNVGP

LIIKKKET
Length:108
Mass (Da):12,199
Last modified:October 1, 2000 - v1
Checksum:i2E47000F0635CBB7
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY960578 mRNA Translation: AAY32336.1
AF220049 mRNA Translation: AAF67642.1
AK312017 mRNA Translation: BAG34955.1
CH471083 Genomic DNA Translation: EAW54163.1
BC005962 mRNA Translation: AAH05962.1
BC007043 mRNA Translation: AAH07043.1
BC008474 mRNA Translation: AAH08474.1
BC059168 mRNA Translation: AAH59168.1
CCDSiCCDS7251.1
RefSeqiNP_060934.1, NM_018464.4
UniGeneiHs.370102

Genome annotation databases

EnsembliENST00000333926; ENSP00000363041; ENSG00000122873
GeneIDi55847
KEGGihsa:55847
UCSCiuc001jkc.6 human

Similar proteinsi

Entry informationi

Entry nameiCISD1_HUMAN
AccessioniPrimary (citable) accession number: Q9NZ45
Secondary accession number(s): Q1X902
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 25, 2002
Last sequence update: October 1, 2000
Last modified: March 28, 2018
This is version 148 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome
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Main funding by: National Institutes of Health