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Identification
HMDB Protein ID HMDBP08677
Secondary Accession Numbers
  • 14397
Name Iron-sulfur cluster assembly enzyme ISCU, mitochondrial
Synonyms
  1. NifU-like N-terminal domain-containing protein
  2. NifU-like protein
Gene Name ISCU
Protein Type Unknown
Biological Properties
General Function Involved in iron ion binding
Specific Function Involved in the assembly or repair of the [Fe-S] clusters present in iron-sulfur proteins. Binds iron
Pathways Not Available
Reactions Not Available
GO Classification
Function
ion binding
cation binding
metal ion binding
binding
transition metal ion binding
metal cluster binding
iron-sulfur cluster binding
iron ion binding
protein binding
Process
metabolic process
biosynthetic process
cellular biosynthetic process
cofactor biosynthetic process
metallo-sulfur cluster assembly
iron-sulfur cluster assembly
Cellular Location
  1. Nucleus
  2. Isoform 2:Cytoplasm
Gene Properties
Chromosome Location Chromosome:1
Locus 12q24.1
SNPs ISCU
Gene Sequence
>504 bp
ATGGCGGCGGCTGGGGCTTTCCGTCTGAGGCGGGCGGCATCGGCTCTGCTGCTGCGGAGC
CCCCGCCTGCCCGCCCGGGAGCTGTCGGCCCCGGCCCGACTCTATCACAAGAAGGTTGTT
GATCATTATGAAAATCCTAGAAACGTGGGGTCCCTTGACAAGACATCTAAAAATGTTGGA
ACTGGACTGGTGGGGGCTCCAGCATGTGGTGACGTAATGAAATTACAGATTCAAGTGGAT
GAAAAGGGGAAGATTGTGGATGCTAGGTTTAAAACATTTGGCTGTGGTTCCGCAATTGCC
TCCAGCTCATTAGCCACTGAATGGGTGAAAGGAAAGACGGTGGAGGAAGCCTTGACTATC
AAAAACACAGATATCGCCAAGGAGCTCTGCCTTCCTCCCGTGAAACTGCACTGCTCCATG
CTGGCTGAAGATGCAATCAAGGCCGCCCTGGCTGATTACAAATTGAAACAAGAACCCAAA
AAAGGAGAGGCAGAGAAGAAATGA
Protein Properties
Number of Residues 167
Molecular Weight 17936.7
Theoretical pI 10.13
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • None
Protein Sequence
>Iron-sulfur cluster assembly enzyme ISCU, mitochondrial
MAAAGAGRLRRVASALLLRSPRLPARELSAPARLYHKKVVDHYENPRNVGSLDKTSKNVG
TGLVGAPACGDVMKLQIQVDEKGKIVDARFKTFGCGSAIASSSLATEWVKGKTVEEALTI
KNTDIAKELCLPPVKLHCSMLAEDAIKAALADYKLKQEPKKGEAEKK
GenBank ID Protein 56699456
UniProtKB/Swiss-Prot ID Q9H1K1
UniProtKB/Swiss-Prot Entry Name ISCU_HUMAN
PDB IDs
GenBank Gene ID NM_213595.2
GeneCard ID ISCU
GenAtlas ID ISCU
HGNC ID HGNC:29882
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Dephoure N, Zhou C, Villen J, Beausoleil SA, Bakalarski CE, Elledge SJ, Gygi SP: A quantitative atlas of mitotic phosphorylation. Proc Natl Acad Sci U S A. 2008 Aug 5;105(31):10762-7. doi: 10.1073/pnas.0805139105. Epub 2008 Jul 31. [PubMed:18669648 ]
  3. Mayya V, Lundgren DH, Hwang SI, Rezaul K, Wu L, Eng JK, Rodionov V, Han DK: Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions. Sci Signal. 2009 Aug 18;2(84):ra46. doi: 10.1126/scisignal.2000007. [PubMed:19690332 ]
  4. Gauci S, Helbig AO, Slijper M, Krijgsveld J, Heck AJ, Mohammed S: Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach. Anal Chem. 2009 Jun 1;81(11):4493-501. doi: 10.1021/ac9004309. [PubMed:19413330 ]
  5. Tong WH, Rouault T: Distinct iron-sulfur cluster assembly complexes exist in the cytosol and mitochondria of human cells. EMBO J. 2000 Nov 1;19(21):5692-700. [PubMed:11060020 ]
  6. Hwang DM, Dempsey A, Tan KT, Liew CC: A modular domain of NifU, a nitrogen fixation cluster protein, is highly conserved in evolution. J Mol Evol. 1996 Nov;43(5):536-40. [PubMed:8875867 ]
  7. Mochel F, Knight MA, Tong WH, Hernandez D, Ayyad K, Taivassalo T, Andersen PM, Singleton A, Rouault TA, Fischbeck KH, Haller RG: Splice mutation in the iron-sulfur cluster scaffold protein ISCU causes myopathy with exercise intolerance. Am J Hum Genet. 2008 Mar;82(3):652-60. doi: 10.1016/j.ajhg.2007.12.012. Epub 2008 Feb 14. [PubMed:18304497 ]