Peroxisomal bifunctional enzyme

EHHADH
Identifiers
Aliases	EHHADH, ECHD, FRTS3, L-PBE, LBFP, LBP, PBFE, enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase, enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase, MFE1
External IDs	OMIM: 607037; MGI: 1277964; GeneCards: EHHADH
Enzyme activity
Gene location (Human)
Chr.	Chromosome 3 (human)
End	185,281,990 bp
Gene location (Mouse)
Chr.	Chromosome 16 (mouse)
End	21,606,557 bp
RNA expression pattern
	Top expressed in
	right lobe of liver; ; kidney tubule; ; jejunal mucosa; ; glomerulus; ; metanephric glomerulus; ; renal medulla; ; human kidney; ; Epithelium of choroid plexus; ; mucosa of transverse colon; ; gonad;
	Top expressed in
	right kidney; ; human kidney; ; left lobe of liver; ; parotid gland; ; proximal tubule; ; duodenum; ; jejunum; ; brown adipose tissue; ; yolk sac; ; epithelium of stomach;
	More reference expression data
	n/a
Gene ontology
Molecular function	dodecenoyl-CoA delta-isomerase activity; signaling receptor binding; catalytic activity; lyase activity; isomerase activity; enoyl-CoA hydratase activity; oxidoreductase activity; protein binding; enzyme binding; long-chain-enoyl-CoA hydratase activity; 3-hydroxyacyl-CoA dehydrogenase activity; nucleotide binding; GTP binding; structural constituent of cytoskeleton; GTPase activity;
Cellular component	peroxisome; peroxisomal matrix; cytosol; cytoplasm; microtubule; cytoskeleton; microtubule cytoskeleton;
Biological process	fatty acid metabolic process; internal protein amino acid acetylation; metabolism; lipid metabolism; fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA oxidase; protein targeting to peroxisome; microtubule-based process; cytoskeleton organization; microtubule cytoskeleton organization; mitotic cell cycle;
	Sources:Amigo / QuickGO
Orthologs
Databases	NCBI: entry; OMA: entry
	1962
	74147
	ENSG00000113790
	ENSMUSG00000022853
	Q08426; Q9NY65
	Q9DBM2
	NM_001166415; NM_001966
	NM_023737
	NP_001159887; NP_001957; NP_001180343; NP_061816
	NP_076226
	Wikidata
View/Edit Human	View/Edit Mouse

Peroxisomal bifunctional enzyme is an enzyme that in humans is encoded by the gene EHHADH. This is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. Mutations of the gene are a cause of peroxisomal disorders such as Zellweger syndrome.

3-Hydroxyacyl ACP dehydratase activity

Peroxisomal bifunctional enzyme is able to act as a Enoyl-CoA hydratase which is involved in the breakdown of fatty acids in the peroxisomes.

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000113790 – Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000022853 – 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.
↑ "EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. 6 September 2017. Retrieved 12 September 2017.
↑ Hoefler G, Forstner M, McGuinness MC, Hulla W, Hiden M, Krisper P, Kenner L, Ried T, Lengauer C, Zechner R (January 1994). "cDNA cloning of the human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase bifunctional enzyme and localization to chromosome 3q26.3-3q28: a free left Alu Arm is inserted in the 3' noncoding region". Genomics. 19 (1): 60–67. doi:10.1006/geno.1994.1013. PMID 8188243.

This article on a gene on human chromosome 3 is a stub. You can help Wikipedia by adding missing information.

[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000113790 – Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000022853 – 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.

[5] "EHHADH enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [ Homo sapiens (human) ]". NCBI. 6 September 2017. Retrieved 12 September 2017.

[Hoefler_1994-6] Hoefler G, Forstner M, McGuinness MC, Hulla W, Hiden M, Krisper P, Kenner L, Ried T, Lengauer C, Zechner R (January 1994). "cDNA cloning of the human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase bifunctional enzyme and localization to chromosome 3q26.3-3q28: a free left Alu Arm is inserted in the 3' noncoding region". Genomics. 19 (1): 60–67. doi:10.1006/geno.1994.1013. PMID 8188243.

Peroxisomal bifunctional enzyme

3-Hydroxyacyl ACP dehydratase activity

See also

References