Lipoyl synthase, mitochondrial

Details

Name
Lipoyl synthase, mitochondrial
Synonyms
  • 2.8.1.8
  • LAS
  • Lip-syn
  • Lipoate synthase
  • Lipoic acid synthase
  • LS
Gene Name
LIAS
Organism
Humans
Amino acid sequence
>lcl|BSEQ0037190|Lipoyl synthase, mitochondrial
MSLRCGDAARTLGPRVFGRYFCSPVRPLSSLPDKKKELLQNGPDLQDFVSGDLADRSTWD
EYKGNLKRQKGERLRLPPWLKTEIPMGKNYNKLKNTLRNLNLHTVCEEARCPNIGECWGG
GEYATATATIMLMGDTCTRGCRFCSVKTARNPPPLDASEPYNTAKAIAEWGLDYVVLTSV
DRDDMPDGGAEHIAKTVSYLKERNPKILVECLTPDFRGDLKAIEKVALSGLDVYAHNVET
VPELQSKVRDPRANFDQSLRVLKHAKKVQPDVISKTSIMLGLGENDEQVYATMKALREAD
VDCLTLGQYMQPTRRHLKVEEYITPEKFKYWEKVGNELGFHYTASGPLVRSSYKAGEFFL
KNLVAKRKTKDL
Number of residues
372
Molecular Weight
41910.695
Theoretical pI
8.77
GO Classification
Functions
4 iron, 4 sulfur cluster binding / lipoate synthase activity / metal ion binding
Processes
inflammatory response / lipoate biosynthetic process / neural tube closure / protein lipoylation / response to lipopolysaccharide / response to oxidative stress
Components
mitochondrion
General Function
Metal ion binding
Specific Function
Catalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Mitochondrion
Gene sequence
>lcl|BSEQ0020609|Lipoyl synthase, mitochondrial (LIAS)
ATGTCTCTACGCTGCGGGGATGCAGCCCGCACCCTGGGGCCCCGGGTATTTGGGAGATAT
TTTTGCAGCCCAGTCAGACCGTTAAGCTCCTTGCCAGATAAAAAAAAGGAACTCCTACAG
AATGGACCAGACCTTCAAGATTTTGTATCTGGTGATCTTGCAGACAGGAGCACCTGGGAT
GAATATAAAGGAAACCTAAAACGCCAGAAAGGAGAAAGGTTAAGACTACCTCCATGGCTA
AAGACAGAGATTCCCATGGGGAAAAATTACAATAAACTGAAAAATACTTTGCGGAATTTA
AATCTCCATACAGTATGTGAGGAAGCTCGATGTCCCAATATTGGAGAGTGTTGGGGAGGT
GGAGAATATGCCACCGCCACAGCCACGATCATGTTGATGGGTGACACATGTACAAGAGGT
TGCAGATTTTGTTCTGTTAAGACTGCAAGAAATCCTCCTCCACTGGATGCCAGTGAGCCC
TACAATACTGCAAAGGCAATTGCAGAATGGGGTCTGGATTATGTTGTCCTGACATCTGTG
GATCGAGATGATATGCCTGATGGGGGAGCTGAACACATTGCAAAGACCGTATCATATTTA
AAGGAAAGTAAGGTTCGTGATCCTCGGGCCAATTTTGATCAGTCCCTACGTGTACTGAAA
CATGCCAAGAAGGTTCAGCCTGATGTTATTTCTAAAACATCTATAATGTTGGGTTTAGGC
GAGAATGATGAGCAAGTATATGCAACAATGAAAGCACTTCGTGAGGCAGATGTAGACTGC
TTGACTTTAGGACAATATATGCAGCCAACAAGGCGTCACCTTAAGGTTGAAGAATATATT
ACTCCTGAAAAATTCAAATACTGGGAAAAAGTAGGAAATGAACTTGGATTTCATTATACT
GCAAGTGGCCCTTTGGTGCGTTCTTCATATAAAGCAGGTGAATTTTTCCTGAAAAATCTA
GTGGCTAAAAGAAAAACAAAAGACCTCTAA
Chromosome Location
4
Locus
4p14
External Identifiers
ResourceLink
UniProtKB IDO43766
UniProtKB Entry NameLIAS_HUMAN
GenBank Protein ID23958222
GenBank Gene IDBC023635
GenAtlas IDLIAS
HGNC IDHGNC:16429
General References
  1. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [Article]
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  3. 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. [Article]
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  5. Mayr JA, Zimmermann FA, Fauth C, Bergheim C, Meierhofer D, Radmayr D, Zschocke J, Koch J, Sperl W: Lipoic acid synthetase deficiency causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation. Am J Hum Genet. 2011 Dec 9;89(6):792-7. doi: 10.1016/j.ajhg.2011.11.011. [Article]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00166Lipoic acidapproved, investigational, nutraceuticalunknownDetails