Sequence of DHTK1_HUMAN
EC Number:1.2.1.105
EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
Reaction
2-oxoglutarate + CoA + NAD+ = succinyl-CoA + CO2 + NADH
Other sequences found for EC No. 1.2.1.105
EC Number:1.2.4.2
EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
Reaction
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine = [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
Other sequences found for EC No. 1.2.4.2
General information:
Sequence
0 MASATAAAAR RGLGRALPLF WRGYQTERGV YGYRPRKPES REPQGALERP PVDHGLARLV
60 TVYCEHGHKA AKINPLFTGQ ALLENVPEIQ ALVQTLQGPF HTAGLLNMGK EEASLEEVLV
120 YLNQIYCGQI SIETSQLQSQ DEKDWFAKRF EELQKETFTT EERKHLSKLM LESQEFDHFL
180 ATKFSTVKRY GGEGAESMMG FFHELLKMSA YSGITDVIIG MPHRGRLNLL TGLLQFPPEL
240 MFRKMRGLSE FPENFSATGD VLSHLTSSVD LYFGAHHPLH VTMLPNPSHL EAVNPVAVGK
300 TRGRQQSRQD GDYSPDNSAQ PGDRVICLQV HGDASFCGQG IVPETFTLSN LPHFRIGGSV
360 HLIVNNQLGY TTPAERGRSS LYCSDIGKLV GCAIIHVNGD SPEEVVRATR LAFEYQRQFR
420 KDVIIDLLCY RQWGHNELDE PFYTNPIMYK IIRARKSIPD TYAEHLIAGG LMTQEEVSEI
480 KSSYYAKLND HLNNMAHYRP PALNLQAHWQ GLAQPEAQIT TWSTGVPLDL LRFVGMKSVE
540 VPRELQMHSH LLKTHVQSRM EKMMDGIKLD WATAEALALG SLLAQGFNVR LSGQDVGRGT
600 FSQRHAIVVC QETDDTYIPL NHMDPNQKGF LEVSNSPLSE EAVLGFEYGM SIESPKLLPL
660 WEAQFGDFFN GAQIIFDTFI SGGEAKWLLQ SGIVILLPHG YDGAGPDHSS CRIERFLQMC
720 DSAEEGVDGD TVNMFVVHPT TPAQYFHLLR RQMVRNFRKP LIVASPKMLL RLPAAVSTLQ
780 EMAPGTTFNP VIGDSSVDPK KVKTLVFCSG KHFYSLVKQR ESLGAKKHDF AIIRVEELCP
840 FPLDSLQQEM SKYKHVKDHI WSQEEPQNMG PWSFVSPRFE KQLACKLRLV GRPPLPVPAV
900 GIGTVHLHQH EDILAKTFA
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Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
1177971
Deloukas P.,Earthrowl M.E.,Grafham D.V.,Rubenfield M.,French L.,Steward C.A.,Sims S.K.,Jones M.C.,Searle S.,Scott C.,Howe K.,Hunt S.E.,Andrews T.D.,Gilbert J.G.R.,Swarbreck D.,Ashurst J.L.,Taylor A.,Battles J.,Bird C.P.,Ainscough R.,Almeida J.P.,Ashwell R.I.S.,Ambrose K.D.,Babbage A.K.,Bagguley C.L.,Bailey J.,Banerjee R.,Bates K.,Beasley H.,Bray-Allen S.,Brown A.J.,Brown J.Y.,Burford D.C.,Burrill W.,Burton J.,Cahill P.,Camire D.,Carter N.P.,Chapman J.C.,Clark S.Y.,Clarke G.,Clee C.M.,Clegg S.,Corby N.,Coulson A.,Dhami P.,Dutta I.,Dunn M.,Faulkner L.,Frankish A.,Frankland J.A.,Garner P.,Garnett J.,Gribble S.,Griffiths C.,Grocock R.,Gustafson E.,Hammond S.,Harley J.L.,Hart E.,Heath P.D.,Ho T.P.,Hopkins B.,Horne J.,Howden P.J.,Huckle E.,Hynds C.,Johnson C.,Johnson D.,Kana A.,Kay M.,Kimberley A.M.,Kershaw J.K.,Kokkinaki M.,Laird G.K.,Lawlor S.,Lee H.M.,Leongamornlert D.A.,Laird G.,Lloyd C.,Lloyd D.M.,Loveland J.,Lovell J.,McLaren S.,McLay K.E.,McMurray A.,Mashreghi-Mohammadi M.,Matthews L.,Milne S.,Nickerson T.,Nguyen M.,Overton-Larty E.,Palmer S.A.,Pearce A.V.,Peck A.I.,Pelan S.,Phillimore B.,Porter K.,Rice C.M.,Rogosin A.,Ross M.T.,Sarafidou T.,Sehra H.K.,Shownkeen R.,Skuce C.D.,Smith M.,Standring L.,Sycamore N.,Tester J.,Thorpe A.,Torcasso W.,Tracey A.,Tromans A.,Tsolas J.,Wall M.,Walsh J.,Wang H.,Weinstock K.,West A.P.,Willey D.L.,Whitehead S.L.,Wilming L.,Wray P.W.,Young L.,Chen Y.,Lovering R.C.,Moschonas N.K.,Siebert R.,Fechtel K.,Bentley D.,Durbin R.M.,Hubbard T.,Doucette-Stamm L.,Beck S.,Smith D.R.,Rogers J.
The DNA sequence and comparative analysis of human chromosome 10.
Nature
429
375-381
2004
1177972
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
1177973
Nagase T.,Kikuno R.,Nakayama M.,Hirosawa M.,Ohara O.
Prediction of the coding sequences of unidentified human genes. XVIII. The complete sequences of 100 new cDNA clones from brain which code for large proteins in vitro.
DNA Res.
7
273-281
2000
1177974
Bechtel S.,Rosenfelder H.,Duda A.,Schmidt C.P.,Ernst U.,Wellenreuther R.,Mehrle A.,Schuster C.,Bahr A.,Bloecker H.,Heubner D.,Hoerlein A.,Michel G.,Wedler H.,Koehrer K.,Ottenwaelder B.,Poustka A.,Wiemann S.,Schupp I.
The full-ORF clone resource of the German cDNA consortium.
BMC Genomics
8
399-399
2007
1177975
Xu W.Y.,Gu M.M.,Sun L.H.,Guo W.T.,Zhu H.B.,Ma J.F.,Yuan W.T.,Kuang Y.,Ji B.J.,Wu X.L.,Chen Y.,Zhang H.X.,Sun F.T.,Huang W.,Huang L.,Chen S.D.,Wang Z.G.
A nonsense mutation in DHTKD1 causes Charcot-Marie-Tooth disease type 2 in a large Chinese pedigree.
Am. J. Hum. Genet.
91
1088-1094
2012
1177976
Nemeria N.S.,Gerfen G.,Nareddy P.R.,Yang L.,Zhang X.,Szostak M.,Jordan F.
The mitochondrial 2-oxoadipate and 2-oxoglutarate dehydrogenase complexes share their E2 and E3 components for their function and both generate reactive oxygen species.
Free Radic. Biol. Med.
115
136-145
2018
1177977
Nemeria N.S.,Gerfen G.,Yang L.,Zhang X.,Jordan F.
Evidence for functional and regulatory cross-talk between the tricarboxylic acid cycle 2-oxoglutarate dehydrogenase complex and 2-oxoadipate dehydrogenase on the l-lysine, l-hydroxylysine and l-tryptophan degradation pathways from studies in vitro.
Biochim. Biophys. Acta
1859
932-939
2018
1177978
Zhang X.,Nemeria N.S.,Leandro J.,Houten S.,Lazarus M.,Gerfen G.,Ozohanics O.,Ambrus A.,Nagy B.,Brukh R.,Jordan F.
Structure-function analyses of the G729R 2-oxoadipate dehydrogenase genetic variant associated with a disorder of l-lysine metabolism.
J. Biol. Chem.
295
8078-8095
2020
1177979
Leandro J.,Khamrui S.,Wang H.,Suebsuwong C.,Nemeria N.S.,Huynh K.,Moustakim M.,Secor C.,Wang M.,Dodatko T.,Stauffer B.,Wilson C.G.,Yu C.,Arkin M.R.,Jordan F.,Sanchez R.,DeVita R.J.,Lazarus M.B.,Houten S.M.
Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex.
ACS Chem. Biol.
15
2041-2047
2020
1177980
Bezerra G.A.,Foster W.R.,Bailey H.J.,Hicks K.G.,Sauer S.W.,Dimitrov B.,McCorvie T.J.,Okun J.G.,Rutter J.,Koelker S.,Yue W.W.
Crystal structure and interaction studies of human DHTKD1 provide insight into a mitochondrial megacomplex in lysine catabolism.
IUCrJ
7
693-706
2020
1177981
Bian Y.,Song C.,Cheng K.,Dong M.,Wang F.,Huang J.,Sun D.,Wang L.,Ye M.,Zou H.
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
J. Proteomics
96
253-262
2014
1177982
Vaca Jacome A.S.,Rabilloud T.,Schaeffer-Reiss C.,Rompais M.,Ayoub D.,Lane L.,Bairoch A.,Van Dorsselaer A.,Carapito C.
N-terminome analysis of the human mitochondrial proteome.
Proteomics
15
2519-2524
2015
1177983
Danhauser K.,Sauer S.W.,Haack T.B.,Wieland T.,Staufner C.,Graf E.,Zschocke J.,Strom T.M.,Traub T.,Okun J.G.,Meitinger T.,Hoffmann G.F.,Prokisch H.,Koelker S.
DHTKD1 mutations cause 2-aminoadipic and 2-oxoadipic aciduria.
Am. J. Hum. Genet.
91
1082-1087
2012
1177984
Hagen J.,te Brinke H.,Wanders R.J.,Knegt A.C.,Oussoren E.,Hoogeboom A.J.,Ruijter G.J.,Becker D.,Schwab K.O.,Franke I.,Duran M.,Waterham H.R.,Sass J.O.,Houten S.M.
Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria.
J. Inherit. Metab. Dis.
38
873-879
2015
1177985
Stiles A.R.,Venturoni L.,Mucci G.,Elbalalesy N.,Woontner M.,Goodman S.,Abdenur J.E.
New Cases of DHTKD1 Mutations in Patients with 2-Ketoadipic Aciduria.
JIMD Rep.
25
15-19
2016
