Details for: DHTKD1

Gene ID: 55526

Symbol: DHTKD1

Ensembl ID: ENSG00000181192

Description: dehydrogenase E1 and transketolase domain containing 1

Associated with

Other Information

Genular Protein ID: 1270775878

Symbol: DHTK1_HUMAN

Name: 2-oxoadipate dehydrogenase complex component E1

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 15164054

Title: The DNA sequence and comparative analysis of human chromosome 10.

PubMed ID: 15164054

DOI: 10.1038/nature02462

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 10997877

Title: 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.

PubMed ID: 10997877

DOI: 10.1093/dnares/7.4.271

PubMed ID: 17974005

Title: The full-ORF clone resource of the German cDNA consortium.

PubMed ID: 17974005

DOI: 10.1186/1471-2164-8-399

PubMed ID: 23141294

Title: A nonsense mutation in DHTKD1 causes Charcot-Marie-Tooth disease type 2 in a large Chinese pedigree.

PubMed ID: 23141294

DOI: 10.1016/j.ajhg.2012.09.018

PubMed ID: 29191460

Title: The mitochondrial 2-oxoadipate and 2-oxoglutarate dehydrogenase complexes share their E2 and E3 components for their function and both generate reactive oxygen species.

PubMed ID: 29191460

DOI: 10.1016/j.freeradbiomed.2017.11.018

PubMed ID: 29752936

Title: 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.

PubMed ID: 29752936

DOI: 10.1016/j.bbabio.2018.05.001

PubMed ID: 32303640

Title: Structure-function analyses of the G729R 2-oxoadipate dehydrogenase genetic variant associated with a disorder of l-lysine metabolism.

PubMed ID: 32303640

DOI: 10.1074/jbc.ra120.012761

PubMed ID: 32633484

Title: Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex.

PubMed ID: 32633484

DOI: 10.1021/acschembio.0c00114

PubMed ID: 32695416

Title: Crystal structure and interaction studies of human DHTKD1 provide insight into a mitochondrial megacomplex in lysine catabolism.

PubMed ID: 32695416

DOI: 10.1107/s205225252000696x

PubMed ID: 24275569

Title: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.

PubMed ID: 24275569

DOI: 10.1016/j.jprot.2013.11.014

PubMed ID: 25944712

Title: N-terminome analysis of the human mitochondrial proteome.

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

PubMed ID: 23141293

Title: DHTKD1 mutations cause 2-aminoadipic and 2-oxoadipic aciduria.

PubMed ID: 23141293

DOI: 10.1016/j.ajhg.2012.10.006

PubMed ID: 25860818

Title: Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria.

PubMed ID: 25860818

DOI: 10.1007/s10545-015-9841-9

PubMed ID: 26141459

Title: New Cases of DHTKD1 Mutations in Patients with 2-Ketoadipic Aciduria.

PubMed ID: 26141459

DOI: 10.1007/8904_2015_462

Sequence Information:

  • Length: 919
  • Mass: 103077
  • Checksum: CD1E437F919324B6
  • Sequence:
  • MASATAAAAR RGLGRALPLF WRGYQTERGV YGYRPRKPES REPQGALERP PVDHGLARLV 
    TVYCEHGHKA AKINPLFTGQ ALLENVPEIQ ALVQTLQGPF HTAGLLNMGK EEASLEEVLV 
    YLNQIYCGQI SIETSQLQSQ DEKDWFAKRF EELQKETFTT EERKHLSKLM LESQEFDHFL 
    ATKFSTVKRY GGEGAESMMG FFHELLKMSA YSGITDVIIG MPHRGRLNLL TGLLQFPPEL 
    MFRKMRGLSE FPENFSATGD VLSHLTSSVD LYFGAHHPLH VTMLPNPSHL EAVNPVAVGK 
    TRGRQQSRQD GDYSPDNSAQ PGDRVICLQV HGDASFCGQG IVPETFTLSN LPHFRIGGSV 
    HLIVNNQLGY TTPAERGRSS LYCSDIGKLV GCAIIHVNGD SPEEVVRATR LAFEYQRQFR 
    KDVIIDLLCY RQWGHNELDE PFYTNPIMYK IIRARKSIPD TYAEHLIAGG LMTQEEVSEI 
    KSSYYAKLND HLNNMAHYRP PALNLQAHWQ GLAQPEAQIT TWSTGVPLDL LRFVGMKSVE 
    VPRELQMHSH LLKTHVQSRM EKMMDGIKLD WATAEALALG SLLAQGFNVR LSGQDVGRGT 
    FSQRHAIVVC QETDDTYIPL NHMDPNQKGF LEVSNSPLSE EAVLGFEYGM SIESPKLLPL 
    WEAQFGDFFN GAQIIFDTFI SGGEAKWLLQ SGIVILLPHG YDGAGPDHSS CRIERFLQMC 
    DSAEEGVDGD TVNMFVVHPT TPAQYFHLLR RQMVRNFRKP LIVASPKMLL RLPAAVSTLQ 
    EMAPGTTFNP VIGDSSVDPK KVKTLVFCSG KHFYSLVKQR ESLGAKKHDF AIIRVEELCP 
    FPLDSLQQEM SKYKHVKDHI WSQEEPQNMG PWSFVSPRFE KQLACKLRLV GRPPLPVPAV 
    GIGTVHLHQH EDILAKTFA

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.