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- SASDDD3: Human mitochondrial cysteine desulfurase-ISCU-Frataxin (NFS1, ISD... -
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Basic information
Entry | Database: SASBDB / ID: SASDDD3 |
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![]() | Human mitochondrial cysteine desulfurase-ISCU-Frataxin (NFS1, ISD11 and Acp heterodimer complex)
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Function / homology | ![]() regulation of ferrochelatase activity / negative regulation of iron ion import across plasma membrane / molybdopterin cofactor metabolic process / Molybdenum cofactor biosynthesis / proprioception / [4Fe-4S] cluster assembly / iron incorporation into metallo-sulfur cluster / positive regulation of lyase activity / positive regulation of succinate dehydrogenase activity / L-cysteine desulfurase complex ...regulation of ferrochelatase activity / negative regulation of iron ion import across plasma membrane / molybdopterin cofactor metabolic process / Molybdenum cofactor biosynthesis / proprioception / [4Fe-4S] cluster assembly / iron incorporation into metallo-sulfur cluster / positive regulation of lyase activity / positive regulation of succinate dehydrogenase activity / L-cysteine desulfurase complex / Mitochondrial iron-sulfur cluster biogenesis / mitochondrial [2Fe-2S] assembly complex / positive regulation of aconitate hydratase activity / positive regulation of mitochondrial electron transport, NADH to ubiquinone / iron chaperone activity / Maturation of TCA enzymes and regulation of TCA cycle / cysteine desulfurase / cysteine desulfurase activity / negative regulation of organ growth / Mo-molybdopterin cofactor biosynthetic process / iron-sulfur cluster assembly complex / Mitochondrial protein import / [2Fe-2S] cluster assembly / oxidative phosphorylation / adult walking behavior / response to iron ion / embryo development ending in birth or egg hatching / lipid biosynthetic process / iron-sulfur cluster assembly / heme biosynthetic process / negative regulation of multicellular organism growth / lipid A biosynthetic process / organ growth / positive regulation of catalytic activity / muscle cell cellular homeostasis / ferroxidase / negative regulation of release of cytochrome c from mitochondria / iron-sulfur cluster binding / phosphopantetheine binding / protein autoprocessing / acyl binding / ferroxidase activity / acyl carrier activity / ferric iron binding / mitochondrion organization / ferrous iron binding / 2 iron, 2 sulfur cluster binding / fatty acid biosynthetic process / cellular response to hydrogen peroxide / pyridoxal phosphate binding / Maturation of replicase proteins / iron ion transport / positive regulation of cell growth / intracellular iron ion homeostasis / molecular adaptor activity / nuclear body / mitochondrial matrix / response to xenobiotic stimulus / iron ion binding / centrosome / lipid binding / positive regulation of cell population proliferation / negative regulation of apoptotic process / protein homodimerization activity / mitochondrion / zinc ion binding / nucleoplasm / nucleus / metal ion binding / cytosol / cytoplasm Similarity search - Function |
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![]() | ![]() Title: Architectural Features of Human Mitochondrial Cysteine Desulfurase Complexes from Crosslinking Mass Spectrometry and Small-Angle X-Ray Scattering. Authors: Kai Cai / Ronnie O Frederick / Hesam Dashti / John L Markley / ![]() Abstract: Cysteine desulfurase plays a central role in mitochondrial iron-sulfur cluster biogenesis by generating sulfur through the conversion of L-cysteine to L-alanine and by serving as the platform for ...Cysteine desulfurase plays a central role in mitochondrial iron-sulfur cluster biogenesis by generating sulfur through the conversion of L-cysteine to L-alanine and by serving as the platform for assembling other components of the biosynthetic machinery, including ISCU, frataxin, and ferredoxin. The human mitochondrial cysteine desulfurase complex consists of two copies each of NFS1, ISD11, and acyl carrier protein. We describe results from chemical crosslinking coupled with tandem mass spectrometry and small-angle X-ray scattering studies that are consistent with a closed NFS1 dimer rather than an open one for both the cysteine desulfurase-ISCU and cysteine desulfurase-ISCU-frataxin complexes. We present a structural model for the cysteine desulfurase-ISCU-frataxin complex derived from chemical crosslinking restraints in conjunction with the recent crystal structure of the cysteine desulfurase-ISCU-zinc complex and distance constraints from nuclear magnetic resonance. |
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Structure visualization
Structure viewer | Molecule: ![]() ![]() |
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Downloads & links
-Data source
SASBDB page | ![]() |
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-Related structure data
Related structure data | C: citing same article ( |
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Similar structure data |
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External links
Related items in Molecule of the Month |
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-Models
Model #1776 | ![]() Type: atomic / Radius of dummy atoms: 1.90 A / Chi-square value: 4.937284 / P-value: 0.000004 ![]() |
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Sample
![]() | Name: Human mitochondrial cysteine desulfurase-ISCU-Frataxin (NFS1, ISD11 and Acp heterodimer complex) Specimen concentration: 2.00-10.00 / Entity id: 958 / 959 / 960 / 961 / 962 |
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Buffer | Name: 20 mM HEPES, 150 mM NaCl, 5 mM TCEP / pH: 7.5 / Comment: HNT Buffer |
Entity #958 | Name: NFS1 / Type: protein / Description: Cysteine desulfurase, mitochondrial / Formula weight: 44.913 / Num. of mol.: 2 / Source: Homo sapiens / References: UniProt: Q9Y697 Sequence: GPVLRPLYMD VQATTPLDPR VLDAMLPYLI NYYGNPHSRT HAYGWESEAA MERARQQVAS LIGADPREII FTSGATESNN IAIKGVARFY RSRKKHLITT QTEHKCVLDS CRSLEAEGFQ VTYLPVQKSG IIDLKELEAA IQPDTSLVSV MTVNNEIGVK QPIAEIGRIC ...Sequence: GPVLRPLYMD VQATTPLDPR VLDAMLPYLI NYYGNPHSRT HAYGWESEAA MERARQQVAS LIGADPREII FTSGATESNN IAIKGVARFY RSRKKHLITT QTEHKCVLDS CRSLEAEGFQ VTYLPVQKSG IIDLKELEAA IQPDTSLVSV MTVNNEIGVK QPIAEIGRIC SSRKVYFHTD AAQAVGKIPL DVNDMKIDLM SISGHKIYGP KGVGAIYIRR RPRVRVEALQ SGGGQERGMR SGTVPTPLVV GLGAACEVAQ QEMEYDHKRI SKLSERLIQN IMKSLPDVVM NGDPKHHYPG CINLSFAYVE GESLLMALKD VALSSGSACT SASLEPSYVL RAIGTDEDLA HSSIRFGIGR FTTEEEVDYT VEKCIQHVKR LREMSPLWEM VQDGIDLKSI KWTQH |
Entity #959 | Name: ISD11 / Type: protein / Description: LYR motif-containing protein 4 / Formula weight: 11.581 / Num. of mol.: 2 / Source: Homo sapiens / References: UniProt: Q9HD34 Sequence: MAASSRAQVL SLYRAMLRES KRFSAYNYRT YAVRRIRDAF RENKNVKDPV EIQTLVNKAK RDLGVIRRQV HIGQLYSTDK LIIENRDMPR THHHHHH |
Entity #960 | Type: protein / Description: Acyl carrier protein / Formula weight: 11.5 / Num. of mol.: 2 / Source: Escherichia coli (strain K12) / References: UniProt: P0A6A8 Sequence: PspP0A6A8A CPECOLIAcy lcarrierpr oteinOSEsc herichiaco li(strainK12)GNacpPP E1SV2MSTIE ERVKKIIGEQ LGVKQEEVTN NASFVEDLGA DSLDTVELVM ALEEEFDTEI PDEEAEKITT VQAAIDYING HQA |
Entity #961 | Name: ISCU / Type: protein Description: Iron-sulfur cluster assembly enzyme ISCU, mitochondrial Formula weight: 14.655 / Num. of mol.: 2 / Source: Homo sapiens / References: UniProt: Q9H1K1 Sequence: RLYHKKVVDH YENPRNVGSL DKTSKNVGTG LVGAPACGDV MKLQIQVDEK GKIVDARFKT FGCGSAIASS SLATEWVKGK TVEEALTIKN TDIAKELCLP PVKLHCSMLA EDAIKAALAD YKLKQEPKKG EAEKK |
Entity #962 | Name: FXN / Type: protein / Description: Frataxin, mitochondrial / Formula weight: 14.268 / Num. of mol.: 2 / Source: Homo sapiens / References: UniProt: Q16595 Sequence: SGTLGHPGSL DETTYERLAE ETLDSLAEFF EDLADKPYTF EDYDVSFGSG VLTVKLGGDL GTYVINKQTP NKQIWLSSPS SGPKRYDWTG KNWVYSHDGV SLHELLAAEL TKALKTKLDL SSLAYSGKDA |
-Experimental information
Beam | Instrument name: NMRFAM Bruker Nanostar / City: Madison, WI / 国: USA ![]() | ||||||||||||||||||||||||||||||||||||
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Detector | Name: VÅNTEC-2000 / Type: MikroGap / Pixsize x: 200 mm | ||||||||||||||||||||||||||||||||||||
Scan | Measurement date: Apr 18, 2017 / Storage temperature: 25 °C / Cell temperature: 25 °C / Exposure time: 3600 sec. / Number of frames: 4 / Unit: 1/A /
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Distance distribution function P(R) |
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Result | Comments: The solution SAXS study of cysteine desulfurase complex containing ISCU and Frataxin
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