TECR

TECR
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	2DZJ
Identifiers
Aliases	TECR , GPSN2, MRT14, SC2, TER, trans-2,3-enoyl-CoA reductase
External IDs	OMIM: 610057 MGI: 1915408 HomoloGene: 36231 GeneCards: TECR
Gene location (Human)
Chr.	Chromosome 19 (human)
End	14,565,980 bp
Gene location (Mouse)
Chr.	Chromosome 8 (mouse)
End	83,607,971 bp
Gene ontology
Molecular function	• oxidoreductase activity, acting on the CH-CH group of donors ; • GO:0001948 protein binding ; • very-long-chain-acyl-CoA dehydrogenase activity ; • oxidoreductase activity ; • very-long-chain enoyl-CoA reductase activity ;
Cellular component	• cytoplasm ; • integral component of membrane ; • integral component of endoplasmic reticulum membrane ; • endoplasmic reticulum membrane ; • endoplasmic reticulum ; • nucleus ; • membrane ;
Biological process	• fatty acid elongation ; • fatty acid biosynthetic process ; • long-chain fatty-acyl-CoA biosynthetic process ; • very long-chain fatty acid biosynthetic process ; • fatty acid metabolic process ; • lipid metabolism ; • sphingolipid metabolic process ;
	Sources:Amigo / QuickGO
Orthologs
	9524
	106529
	ENSG00000099797
	ENSMUSG00000031708
	Q9NZ01
	Q9CY27
	NM_004868 ; NM_138501 ; NM_001321170
	NM_027179 ; NM_134118
	NP_001308099 ; NP_612510
	NP_081455 ; NP_598879
	Wikidata
View/Edit Human	View/Edit Mouse

Last updated September 22, 2021

Trans-2,3-enoyl-CoA reductase is an enzyme that in humans is encoded by the TECR gene.^[5]

This gene encodes a multi-pass membrane protein that resides in the endoplasmic reticulum, and belongs to the steroid 5-alpha reductase family. The elongation of microsomal long and very long chain fatty acid consists of 4 sequential reactions. This protein catalyzes the final step, reducing trans-2,3-enoyl-CoA to saturated acyl-CoA. Alternatively spliced transcript variants have been found for this gene.^[5]

Clinical relevance

Mutations in this gene have been shown to cause non-syndromic mental retardation.^[6]

Related Research Articles

ACADM is a gene that provides instructions for making an enzyme called acyl-coenzyme A dehydrogenase that is important for breaking down (degrading) a certain group of fats called medium-chain fatty acids.

Enoyl-acyl carrier protein reductase, is a key enzyme of the type II fatty acid synthesis (FAS) system. ENR is an attractive target for narrow-spectrum antibacterial drug discovery because of its essential role in metabolism and its sequence conservation across many bacterial species. In addition, the bacterial ENR sequence and structural organization are distinctly different from those of mammalian fatty acid biosynthesis enzymes.

Fatty acid synthase (FAS) is an enzyme that in humans is encoded by the FASN gene.

Trifunctional enzyme subunit beta, mitochondrial (TP-beta) also known as 3-ketoacyl-CoA thiolase, acetyl-CoA acyltransferase, or beta-ketothiolase is an enzyme that in humans is encoded by the HADHB gene.

Peroxisomal acyl-coenzyme A oxidase 1 is an enzyme that in humans is encoded by the ACOX1 gene.

Long-chain-fatty-acid—CoA ligase 4 is an enzyme that in humans is encoded by the ACSL4 gene.

ATP-binding cassette sub-family B member 7, mitochondrial is a protein that in humans is encoded by the ABCB7 gene.

Integral membrane protein 2A is a protein that in humans is encoded by the ITM2A gene.

Mannose-P-dolichol utilization defect 1 protein is a protein that in humans is encoded by the MPDU1 gene.

Very long-chain acyl-CoA synthetase is an enzyme that in humans is encoded by the SLC27A2 gene.

Long-chain fatty acid transport protein 4 is a protein that in humans is encoded by the SLC27A4 gene. This membrane protein is also called FATP4 or ACSVL5. The purified protein shows enzyme activity, esterifying long and very long chain fatty acids with Coenzyme A. It is debated whether it is also a fatty acid transporter at the plasma membrane.

Transmembrane protein 59 is a protein that in humans is encoded by the TMEM59 gene.

Chromodomain-helicase-DNA-binding protein 1-like (ALC1) is an enzyme that in humans is encoded by the CHD1L gene. It has been implicated in chromatin remodeling and DNA relaxation process required for DNA replication, repair and transcription. The ALC1 comprises ATPase domain and macro domain. On the basis of homology within the ATPase domain, ALC1 belongs to Snf2 family.

NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 is an enzyme that in humans is encoded by the NDUFS5 gene.

Trans-2-enoyl-CoA reductase, mitochondrial is an enzyme that in humans is encoded by the MECR gene.

Eukaryotic translation elongation factor 1 epsilon-1 is a protein that in humans is encoded by the EEF1E1 gene.

Bile acyl-CoA synthetase is an enzyme that in humans is encoded by the SLC27A5 gene.

Sperm-associated antigen 7 is a protein that in humans is encoded by the SPAG7 gene.

39S ribosomal protein L33, mitochondrial is a protein that in humans is encoded by the MRPL33 gene.

Very-long-chain enoyl-CoA reductase (EC 1.3.1.93, TSC13 (gene name), CER10 (gene)) is an enzyme with systematic name very-long-chain acyl-CoA:NADP⁺ oxidoreductase. This enzyme catalyses the following chemical reaction

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000099797 - Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000031708 - Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
1 2 "Entrez Gene: Trans-2,3-enoyl-CoA reductase" . Retrieved 2011-12-30.
↑ Çalışkan M, Chong JX, Uricchio L, Anderson R, Chen P, Sougnez C, Garimella K, Gabriel SB, dePristo MA, Shakir K, Matern D, Das S, Waggoner D, Nicolae DL, Ober C (April 2011). "Exome sequencing reveals a novel mutation for autosomal recessive non-syndromic mental retardation in the TECR gene on chromosome 19p13". Hum. Mol. Genet. 20 (7): 1285–9. doi:10.1093/hmg/ddq569. PMC 3115579 . PMID 21212097.

Moon, Y. -A.; Horton, J. D. (2002). "Identification of Two Mammalian Reductases Involved in the Two-carbon Fatty Acyl Elongation Cascade". Journal of Biological Chemistry. 278 (9): 7335–7343. doi: 10.1074/jbc.M211684200 . PMID 12482854.
Hashmi, G.; Shariff, T.; Seul, M.; Vissavajjhala, P.; Hue-Roye, K.; Charles-Pierre, D.; Lomas-Francis, C.; Chaudhuri, A.; Reid, M. E. (2005). "A flexible array format for large-scale, rapid blood group DNA typing". Transfusion. 45 (5): 680–688. doi:10.1111/j.1537-2995.2005.04362.x. PMID 15847654. S2CID 40662473.
Lehner, R.; Kuksis, A. (1996). "Biosynthesis of triacylglycerols". Progress in Lipid Research. 35 (2): 169–201. doi:10.1016/0163-7827(96)00005-7. PMID 8944226.
Mao, M.; Fu, G.; Wu, J. S.; Zhang, Q. H.; Zhou, J.; Kan, L. X.; Huang, Q. H.; He, K. L.; Gu, B. W.; Han, Z. -G.; Shen, Y.; Gu, J.; Yu, Y. -P.; Xu, S. -H.; Wang, Y. -X.; Chen, S. -J.; Chen, Z. (1998). "Identification of genes expressed in human CD34+ hematopoietic stem/progenitor cells by expressed sequence tags and efficient full-length cDNA cloning". Proceedings of the National Academy of Sciences of the United States of America. 95 (14): 8175–8180. Bibcode:1998PNAS...95.8175M. doi: 10.1073/pnas.95.14.8175 . PMC 20949 . PMID 9653160.

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[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000099797 - Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000031708 - Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[entrez-5] 1 2 "Entrez Gene: Trans-2,3-enoyl-CoA reductase" . Retrieved 2011-12-30.

[pmid21212097-6] Çalışkan M, Chong JX, Uricchio L, Anderson R, Chen P, Sougnez C, Garimella K, Gabriel SB, dePristo MA, Shakir K, Matern D, Das S, Waggoner D, Nicolae DL, Ober C (April 2011). "Exome sequencing reveals a novel mutation for autosomal recessive non-syndromic mental retardation in the TECR gene on chromosome 19p13". Hum. Mol. Genet. 20 (7): 1285–9. doi:10.1093/hmg/ddq569. PMC 3115579 . PMID 21212097.

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v t e Oxidoreductases: CH–CH oxidoreductases (EC 1.3)
1.3.1: NAD/NADP acceptor	Enoyl-acyl carrier protein reductase/Enoyl ACP reductase 7-Dehydrocholesterol reductase Biliverdin reductase 2,4 Dienoyl-CoA reductase Dihydroxymethyloxo-tetrahydroquinoline dehydrogenase
1.3.3: Oxygen acceptor	Dihydroorotate dehydrogenase Coproporphyrinogen III oxidase Protoporphyrinogen oxidase Bilirubin oxidase Acyl-CoA oxidase Dihydrouracil oxidase Tetrahydroberberine oxidase Secologanin synthase Tryptophan alpha,beta-oxidase Pyrroloquinoline-quinone synthase L-galactonolactone oxidase
1.3.5: Quinone	Succinate dehydrogenase SDHA SDHB SDHC SDHD
1.3.99: Other acceptors	Fumarate reductase Butyryl-CoA dehydrogenase Acyl CoA dehydrogenase ACADSB ACADS 5α-reductase SRD5A1 SRD5A2 SRD5A3 Glutaryl-CoA dehydrogenase Isovaleryl coenzyme A dehydrogenase 3-oxo-5beta-steroid 4-dehydrogenase

v t e Enzymes
Activity	Active site Binding site Catalytic triad Oxyanion hole Enzyme promiscuity Catalytically perfect enzyme Coenzyme Cofactor Enzyme catalysis
Regulation	Allosteric regulation Cooperativity Enzyme inhibitor Enzyme activator
Classification	EC number Enzyme superfamily Enzyme family List of enzymes
Kinetics	Enzyme kinetics Eadie–Hofstee diagram Hanes–Woolf plot Lineweaver–Burk plot Michaelis–Menten kinetics
Types	EC1 Oxidoreductases (list) EC2 Transferases (list) EC3 Hydrolases (list) EC4 Lyases (list) EC5 Isomerases (list) EC6 Ligases (list) EC7 Translocases (list)

TECR

Contents

Clinical relevance

Related Research Articles

References

Further reading