DHTKD1 (dehydrogenase E1 and transketolase domain containing 1)

2014-11-01  

Identity

HGNC
LOCATION
10p14
LOCUSID
ALIAS
AAKAD,AMOXAD,CMT2Q
FUSION GENES

Other Information

Locus ID:

NCBI: 55526
MIM: 614984
HGNC: 23537
Ensembl: ENSG00000181192

Variants:

dbSNP: 55526
ClinVar: 55526
TCGA: ENSG00000181192
COSMIC: DHTKD1

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000181192ENST00000263035Q96HY7
ENSG00000181192ENST00000415935H7C1J3
ENSG00000181192ENST00000437298C9JWN1
ENSG00000181192ENST00000448829H7C149

Expression (GTEx)

0
10
20
30
40
50
60

Pathways

PathwaySourceExternal ID
MetabolismREACTOMER-HSA-1430728
Metabolism of amino acids and derivativesREACTOMER-HSA-71291
Glyoxylate metabolism and glycine degradationREACTOMER-HSA-389661

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
344841232021Knock-Out of DHTKD1 Alters Mitochondrial Respiration and Function, and May Represent a Novel Pathway in Cardiometabolic Disease Risk.12
350524242021Heterozygous DHTKD1 Variants in Two European Cohorts of Amyotrophic Lateral Sclerosis Patients.5
344841232021Knock-Out of DHTKD1 Alters Mitochondrial Respiration and Function, and May Represent a Novel Pathway in Cardiometabolic Disease Risk.12
350524242021Heterozygous DHTKD1 Variants in Two European Cohorts of Amyotrophic Lateral Sclerosis Patients.5
320248852020Synthetic analogues of 2-oxo acids discriminate metabolic contribution of the 2-oxoglutarate and 2-oxoadipate dehydrogenases in mammalian cells and tissues.17
321602762020DHTKD1 and OGDH display substrate overlap in cultured cells and form a hybrid 2-oxo acid dehydrogenase complex in vivo.16
323036402020Structure-function analyses of the G729R 2-oxoadipate dehydrogenase genetic variant associated with a disorder of l-lysine metabolism.5
326334842020Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex.9
320248852020Synthetic analogues of 2-oxo acids discriminate metabolic contribution of the 2-oxoglutarate and 2-oxoadipate dehydrogenases in mammalian cells and tissues.17
321602762020DHTKD1 and OGDH display substrate overlap in cultured cells and form a hybrid 2-oxo acid dehydrogenase complex in vivo.16
323036402020Structure-function analyses of the G729R 2-oxoadipate dehydrogenase genetic variant associated with a disorder of l-lysine metabolism.5
326334842020Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex.9
258608182015Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria.20
258608182015Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria.20
240764692013DHTKD1 is essential for mitochondrial biogenesis and function maintenance.32

Citation

Dessen P

DHTKD1 (dehydrogenase E1 and transketolase domain containing 1)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/62476/dhtkd1-(dehydrogenase-e1-and-transketolase-domain-containing-1)